Nefropatia iga (choroba bergera)
Leczenie
IgA nefropatia, będąca najczęstszą postacią pierwotnego kłębuszkowego zapalenia nerek, nie posiada obecnie terapii całkowicie eliminującej chorobę, jednak leczenie zindywidualizowane, oparte na ocenie białkomoczu, eGFR, ciśnienia tętniczego i badania histopatologicznego, pozwala spowolnić progresję i opóźnić rozwój schyłkowej niewydolności nerek. Podstawą terapii jest optymalizacja leczenia wspomagającego, obejmująca kontrolę ciśnienia tętniczego (docelowo 130/80 mmHg, a przy białkomoczu >1 g/dobę ciśnienie skurczowe <125 mmHg), redukcję białkomoczu (>0,5 g/dobę wskazuje na konieczność stosowania ACE-I lub ARB), modyfikację stylu życia oraz unikanie nefrotoksycznych leków. Nowoczesne terapie farmakologiczne obejmują inhibitory SGLT2 (dapagliflozyna, empagliflozyna), które wykazały skuteczność w spowolnieniu progresji CKD, oraz nowo zatwierdzone leki takie jak sparsentan (podwójny antagonista receptora endoteliny i angiotensyny) i budezonid o przedłużonym uwalnianiu (Tarpeyo), które znacząco redukują białkomocz i chronią funkcję nerek.
- Podstawowe podejście terapeutyczne w IgA nefropatii (chorobie Bergera)
- Farmakoterapia w IgA nefropatii
- Leczenie immunosupresyjne w IgA nefropatii
- Glikokortykosteroidy
- Budezonid o przedłużonym uwalnianiu
- Inne leki immunosupresyjne
- Inhibitory komplementu
- Dodatkowe opcje terapeutyczne
- Leczenie w schyłkowej niewydolności nerek
- Modyfikacje stylu życia i diety
- Aktualne i nowe kierunki leczenia IgA nefropatii
Podstawowe podejście terapeutyczne w IgA nefropatii (chorobie Bergera)
IgA nefropatia (choroba Bergera) jest najczęstszą postacią pierwotnego kłębuszkowego zapalenia nerek na świecie. Obecnie nie istnieje metoda leczenia, która całkowicie wyleczyłaby tę chorobę, ale dostępne są opcje terapeutyczne, które mogą spowolnić jej progresję i zapobiec lub opóźnić rozwój schyłkowej niewydolności nerek.12 Głównym celem leczenia jest złagodzenie objawów, zmniejszenie białkomoczu, spowolnienie progresji choroby oraz zapobieganie powikłaniom.34
Leczenie IgA nefropatii powinno być zindywidualizowane ze względu na różnorodność przebiegu klinicznego i rokowania. Przy wyborze odpowiedniej strategii terapeutycznej należy wziąć pod uwagę takie czynniki jak: poziom białkomoczu, szacunkowy współczynnik filtracji kłębuszkowej (eGFR), ciśnienie tętnicze krwi oraz wyniki badania histopatologicznego.56
Leczenie wspomagające
Podstawą terapii dla wszystkich pacjentów z IgA nefropatią jest optymalizacja leczenia wspomagającego, niezależnie od ryzyka progresji choroby. Obejmuje ono kontrolę ciśnienia tętniczego, zmniejszenie białkomoczu oraz modyfikację stylu życia.78
Kluczowe elementy leczenia wspomagającego obejmują:910
- Zwiększenie regularnej aktywności fizycznej, zwłaszcza ćwiczeń wytrzymałościowych
- Kontrolę masy ciała w celu zapobiegania lub zmniejszania otyłości
- Zdrową dietę i korektę ewentualnych zaburzeń lipidowych
- Zaprzestanie palenia tytoniu (palenie zwiększa ryzyko dializoterapii 5-10-krotnie!)
- Zmniejszenie spożycia soli
- Unikanie stosowania niesteroidowych leków przeciwzapalnych jako środków przeciwbólowych
- Odpowiednie nawodnienie (1,5 litra płynów dziennie jest wystarczające, chyba że u pacjenta występują dodatkowo kamienie nerkowe lub nawracające infekcje pęcherza moczowego)
Farmakoterapia w IgA nefropatii
Leki hamujące układ renina-angiotensyna
Inhibitory konwertazy angiotensyny (ACE-I) i antagoniści receptora angiotensyny II (ARB) stanowią podstawę farmakoterapii u pacjentów z IgA nefropatią. Leki te są zalecane u wszystkich pacjentów z białkomoczem >0,5 g/dobę, niezależnie od obecności nadciśnienia tętniczego.1112
ACE-I i ARB działają poprzez:1314
- Zmniejszenie ciśnienia tętniczego krwi
- Redukcję białkomoczu
- Ochronę funkcji nerek
- Spowolnienie postępu choroby nerek
Docelowe wartości ciśnienia tętniczego to zazwyczaj 130/80 mmHg. Jeśli białkomocz przekracza 1 g/dobę, zaleca się obniżenie ciśnienia skurczowego poniżej 125 mmHg.15
Badanie STOP-IgAN wykazało, że około jedna trzecia pacjentów osiągnęła zadowalające wyniki bez konieczności stosowania leków immunosupresyjnych po 6-miesięcznej próbie optymalizacji leczenia zachowawczego, obejmującego agresywne miareczkowanie inhibitorów ACE lub ARB.16
Inhibitory SGLT2
Inhibitory kotransportera sodowo-glukozowego 2 (SGLT2) wykazały istotną skuteczność w spowolnieniu progresji przewlekłej choroby nerek. Leki te obniżają poziom białka w moczu i pomagają spowolnić proces chorobowy.1718
Badania DAPA-CKD i EMPA-KIDNEY zostały przedwcześnie zakończone ze względu na widoczne korzyści ze stosowania inhibitorów SGLT2. Chociaż badania te głównie dotyczyły pacjentów z przewlekłą chorobą nerek o różnej etiologii, znaczna liczba uczestników miała zdiagnozowaną IgA nefropatię.1920
Dapagliflozyna i empagliflozyna blokują wychwyt glukozy w nefronie, co powoduje, że mocz zawiera dużo cukru, a to odciążenie pracy pozornie chroni funkcję nerek.21
Antagoniści receptora endoteliny
Sparsentan jest nowym, nieimmunosupresyjnym, podwójnym antagonistą receptora endoteliny i angiotensyny, który otrzymał przyspieszoną zgodę FDA na leczenie IgA nefropatii w lutym 2023 roku.2223
Badanie PROTECT wykazało, że sparsentan znacząco zmniejsza białkomocz w porównaniu do standardowego leczenia irbesartanem. Grupa otrzymująca sparsentan wykazała znacznie mniejszy białkomocz i tendencję do zachowania GFR w porównaniu do grupy otrzymującej irbesartan.2425
Atrasentan, antagonista receptora endoteliny typu A, został również zatwierdzony przez FDA do zmniejszania białkomoczu u dorosłych pacjentów z IgA nefropatią zagrożonych szybką progresją choroby.2627
Leczenie immunosupresyjne w IgA nefropatii
Glikokortykosteroidy
Kortykosteroidy są kluczowe w zmniejszaniu białkomoczu w IgA nefropatii, szczególnie gdy jego poziom przekracza 1g/dobę. Zwykle przepisuje się stopniowo zmniejszającą się dawkę prednizonu przez 2 do 4 miesięcy.2829
Badanie TESTING, które koncentrowało się na pacjentach z IgA nefropatią i białkomoczem przekraczającym 1 g/dobę, wykazało, że leczenie metyloprednizolonem przez 6 do 9 miesięcy znacząco spowalniało progresję choroby w porównaniu z placebo. Jednak ze względu na nadmierną liczbę infekcji i działań niepożądanych, początkową dawkę metyloprednizolonu musiano zmniejszyć i dodać profilaktykę kotrimoksazolem przeciwko zakażeniom oportunistycznym.3031
Należy jednak zauważyć, że stosowanie kortykosteroidów w IgA nefropatii stało się kontrowersyjne. Jeśli są stosowane, powinny być podawane tylko starannie wyselekcjonowanym pacjentom i najlepiej nie dłużej niż przez 6 miesięcy.3233
Budezonid o przedłużonym uwalnianiu
Budezonid, w postaci doustnej formuły o przedłużonym uwalnianiu (Tarpeyo/Nefecon), jest nową terapią dla IgA nefropatii. Ta formulacja składa się z budezonidu zapakowanego w kapsułkę wrażliwą na pH, która preferencyjnie uwalnia aktywny lek w końcowym odcinku jelita krętego w pobliżu kępek Peyera – miejsca znacznej produkcji limfoidalnej tkanki wytwarzającej IgA.3435
System dostarczania leku bezpośrednio do miejsca docelowego umożliwia ekstensywny metabolizm pierwszego przejścia, minimalizując efekty ogólnoustrojowe. Wyniki badań NEFIGAN i NeflgArd wykazały znaczące zmniejszenie białkomoczu i zachowanie GFR przy stosowaniu Nefecon.3637
FDA zatwierdziła budezonid o przedłużonym uwalnianiu (Tarpeyo) do zmniejszania białkomoczu u dorosłych z pierwotną IgA nefropatią, którzy są zagrożeni szybką progresją choroby. Może być stosowany tylko jako uzupełnienie zoptymalizowanej standardowej opieki, w tym najwyższej tolerowanej dawki inhibitorów ACE lub ARB.3839
Inne leki immunosupresyjne
Cyklofosfamid powinien być zarezerwowany dla szybko postępującego kłębuszkowego zapalenia nerek z półksiężycami.4041 Może powodować poważne działania niepożądane, w tym długoterminowe ryzyko nowotworzenia i bezpłodności.42
Wytyczne KDIGO sugerują, aby nie leczyć kombinacją kortykosteroidów z cyklofosfamidem lub azatiopryną, chyba że pacjent ma IgA nefropatię z półksiężycami z szybko pogarszającą się funkcją nerek.43
Jedno badanie wykazało, że pacjenci z wysokim ryzykiem IgA nefropatii, leczeni kombinacją prednizonu z cyklofosfamidem przez początkowe 3 miesiące, a następnie azatiopryną przez co najmniej 2 lata, osiągnęli znaczącą ochronę funkcji nerek.44
Inhibitory komplementu
Iptacopan (Fabhalta), inhibitor komplementu, został zatwierdzony przez FDA do zmniejszania białkomoczu u dorosłych z IgA nefropatią zagrożonych szybką progresją choroby.4546 Działa on na specyficzny proces w nerkach, który, gdy zostanie zakłócony, może uwalniać więcej białka do moczu.47
Dodatkowe opcje terapeutyczne
Leki obniżające poziom cholesterolu
Statyny są zalecane pacjentom z IgA nefropatią, którzy mają podwyższony poziom cholesterolu. Te leki pomagają:4849
- Kontrolować poziom cholesterolu
- Spowalniać uszkodzenie nerek
- Zmniejszać ryzyko chorób sercowo-naczyniowych, które są częstsze u pacjentów z chorobą nerek
Diuretyki
Diuretyki (leki moczopędne) pomagają usuwać nadmiar płynów z organizmu, co może pomóc w kontroli obrzęków rąk i stóp (obrzęk).5051 Mogą również przyczyniać się do lepszej kontroli ciśnienia tętniczego.52
Kwasy omega-3
Kwasy tłuszczowe omega-3, dostępne w suplementach oleju rybiego, mogą pomóc zmniejszyć stan zapalny w kłębuszkach nerkowych bez szkodliwych skutków ubocznych.5354
Badanie z Mayo Clinic z 1994 roku wykazało, że długoterminowe leczenie olejem rybim bogatym w kwasy tłuszczowe omega-3 było związane z niewielkim zmniejszeniem progresji do niewydolności nerek, jednak bez zmniejszenia białkomoczu u części pacjentów z wysokim ryzykiem pogorszenia funkcji nerek.55
Obecne dowody nie potwierdzają stosowania oleju rybiego jako monoterapii, ale niektórzy lekarze łączą olej rybi z innymi terapiami.56 Wytyczne KDIGO sugerują, że olej rybi może być podawany pacjentom, którzy mają utrzymujący się białkomocz ≥1 g/dobę mimo 3-6 miesięcy zoptymalizowanej opieki wspomagającej (w tym leczenia inhibitorami ACE lub ARB i kontroli ciśnienia tętniczego).57
Tonsillektomia
Tonsillektomia (usunięcie migdałków) jest kontrowersyjnym leczeniem IgA nefropatii.58 Migdałki stanowią znaczące miejsce przechowywania tkanki limfatycznej związanej z błoną śluzową (MALT), a poziomy IgA zmniejszają się po tonsillektomii.59
Istnieje korelacja między stężeniem komórek dendrytycznych w migdałkach a tworzeniem półksiężyców u pacjentów z IgA nefropatią. Liczne badania retrospektywne wykazały korzyści z tonsillektomii w indukowaniu remisji lub spowolnieniu progresji IgA nefropatii, szczególnie w populacjach azjatyckich, zwłaszcza w Japonii.60
Jednak tonsillektomia powinna być odpowiednia tylko dla pacjentów z nawracającymi infekcjami migdałków, a obecnie nie jest zalecana w wytycznych, chyba że jest wskazana z powodu nawracających infekcji.6162
Leczenie w schyłkowej niewydolności nerek
U osób, u których IgA nefropatia postępuje do schyłkowej niewydolności nerek (ESRD), dostępne są następujące opcje leczenia:6364
Dializa
Dializoterapia jest metodą leczenia, która zastępuje funkcję nerek poprzez usuwanie toksyn i nadmiaru płynów z krwi, gdy nerki nie są w stanie efektywnie wykonywać tej funkcji.65 Dializa może być wykonywana na dwa sposoby: hemodializa lub dializa otrzewnowa.66
Transplantacja nerki
Transplantacja nerki jest skuteczną opcją dla pacjentów z IgA nefropatią, którzy rozwinęli schyłkową chorobę nerek. Jednak choroba często nawraca po transplantacji (20-60%).6768
Jedno znaczące badanie wykazało wskaźnik nawrotów 23% w przeszczepionych nerkach po 15 latach, co korelowało z 3,7-krotnym wzrostem odrzucenia przeszczepu. Mimo nawrotu złogów IgA w przeszczepionej nerce, progresja do nawracającej ESRD z przeszczepioną nerką jest rzadka.69
Stosowanie globuliny antytymocytowej i utrzymywanie immunosupresji kortykosteroidami może złagodzić nawrót IgA nefropatii. Ponadto leczenie inhibitorami ACE lub ARB może pomóc opóźnić progresję nawracającej choroby w przeszczepach.70
Modyfikacje stylu życia i diety
Oprócz farmakoterapii, modyfikacje stylu życia i diety odgrywają istotną rolę w leczeniu IgA nefropatii:7172
Zalecane modyfikacje diety obejmują:7374
- Ograniczenie ilości sodu w diecie (dieta niskosodowa)
- Stosowanie diety ubogiej w tłuszcze nasycone i cholesterol
- Umiarkowane spożycie białka
- Przejście na dietę opartą głównie na roślinach
Inne zalecane zmiany stylu życia to:7576
- Podejmowanie regularnej aktywności fizycznej
- Utrzymywanie zdrowej masy ciała
- Zaprzestanie palenia tytoniu
- Kontrola ciśnienia tętniczego
- Kontrola poziomu cukru we krwi, szczególnie u osób z cukrzycą
Aktualne i nowe kierunki leczenia IgA nefropatii
W ostatnich latach zatwierdzono kilka nowych leków do leczenia IgA nefropatii:7778
- Budezonid o przedłużonym uwalnianiu (Tarpeyo) – kortykosteroid
- Sparsentan (Filspari) – podwójny antagonista receptora angiotensyny II i endoteliny-1
- Atrasentan (Vanrafia) – antagonista receptora endoteliny typu A
- Iptacopan (Fabhalta) – inhibitor komplementu
Trwają lub zostały niedawno zakończone badania kliniczne fazy 3 nad bezpieczeństwem i skutecznością co najmniej 11 leków do leczenia IgA nefropatii.79 Badane strategie obejmują stosowanie kortykosteroidów o ukierunkowanym uwalnianiu, terapie przeciwko komplementom, modulatory aktywności limfocytów oraz antagonistów anty-endoteliny lub anty-aldosteronu.80
Wśród obiecujących leków w fazie badań klinicznych znajdują się:8182
- Narsoplimab – przeciwciało monoklonalne
- Felzartamab – w pełni ludzkie przeciwciało monoklonalne IgG1 zaprojektowane do usuwania komórek plazmatycznych CD38+
- Sibeprenlimab – inhibitor APRIL
Podejście do leczenia IgA nefropatii szybko się rozwija, a badania nad nowymi terapiami skupiają się na celowaniu w określone szlaki chorobowe. Dotyczy to szczególnie leków immunosupresyjnych działających na specyficzne elementy odpowiedzi immunologicznej, które bezpośrednio przyczyniają się do patogenezy IgA nefropatii.8384
Dzięki lepszemu zrozumieniu patomechanizmów IgA nefropatii i coraz większej liczby opcji terapeutycznych, perspektywy dla pacjentów z tą chorobą stają się coraz bardziej obiecujące. Wczesne rozpoznanie i odpowiednie leczenie mogą znacząco opóźnić progresję choroby i poprawić jakość życia pacjentów.8586
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Materiały źródłowe
- #1 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The management of IgAN begins with confirming the diagnosis, typically through a renal biopsy, while ruling out secondary causes. Key considerations for formulating the management plan include assessing proteinuria, eGFR, blood pressure, and histological findings. The primary goals of treatment are to induce remission and avert the onset of complications. […] First-line agents for managing proteinuria and lowering blood pressure in IgAN patients typically involve angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). The target blood pressure is typically set at 130/80 mm Hg. If proteinuria is more than 1 g/d, the systolic blood pressure target is less than 125 mm Hg. […] Conservative therapy plays a crucial role in managing IgAN by reducing proteinuria and slowing the rate of renal function decline in IgAN, which cannot be overemphasized. In the Supportive Versus Immunosuppressive Therapy of Progressive IgA Nephropathy (STOP-IgAN) study, around one-third of patients achieved satisfactory outcomes without requiring immunosuppressive therapy following a 6-month trial of optimizing conservative measures, including aggressive ACE inhibitors or ARB titration (optimal medical management). In this study, the comparison between immunosuppressive therapy and optimal medical management alone revealed no significant improvement in primary outcomesâdeath, ESRD, or a decline in GFR exceeding 40%âamong those receiving immunosuppressive treatment during the 3-year and 10-year follow-up periods.
- #2 IgA nephropathy – Wikipediahttps://en.wikipedia.org/wiki/IgA_nephropathy
The ideal treatment for IgAN would remove IgA from the glomerulus and prevent further IgA deposition. This goal still remains a remote prospect. […] There are a few additional caveats that have to be considered while treating IgA nephropathy. IgA nephropathy has a very variable course, ranging from a benign recurrent hematuria up to a rapid progression to chronic kidney failure and failure of other major organs. Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. […] In cases where tonsillitis is the precipitating factor for episodic hematuria, a tonsillectomy has been claimed to reduce the frequency of those episodes. However, it does not reduce the incidence of progressive kidney failure. […] A subset of IgA nephropathy patients, who have minimal change disease on light microscopy and clinically have nephrotic syndrome, show an exquisite response to steroids, behaving more or less like minimal change disease. In other patients, the evidence for steroids is not compelling.
- #3 IgA nephropathy (Berger disease) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/iga-nephropathy/diagnosis-treatment/drc-20352274
There’s no cure for IgA nephropathy. There’s also no sure way to predict how much the disease will affect your health over time. Some people need only medical tests to track whether the disease is getting worse. […] For others, medicines can slow the disease from becoming worse and help manage symptoms. […] Medicines to treat IgA nephropathy include: […] High blood pressure drugs. Medicines called angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) can lower blood pressure and reduce how much protein the body loses. […] Medicines that calm the immune system. These also are called immunosuppressants. They include corticosteroids and other strong medicines that can lessen the body’s defenses. They might keep the immune system from making the proteins that attack the glomeruli. These medicines can cause serious side effects, such as high blood pressure, high blood sugar and a greater risk of infection. Your doctor helps you choose an immunosuppressant medicine. Many new treatments have been approved or are being tested in clinical trials. Talk with your doctor about the latest advances in the treatment of IgA nephropathy.
- #4 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
Treatment can slow the progress of the disease and prevent ESRD. If you have IgA nephropathy, your healthcare provider will probably refer you to a nephrologist, a kidney disease specialist. […] Treatment for IgA nephropathy includes medication to: […] Regulate blood pressure with angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), or other medicines […] Remove extra fluid with a diuretic […] Control your immune system to lower kidney inflammation with prescribed steroids such as prednisone or cyclophosphamide, a different medicine […] Lower your cholesterol levels with medications such as statins. […] Some research shows that a tonsillectomy (tonsil removal) can benefit a small portion of people who have IgA nephropathy. Talk to your healthcare provider to see if this may be an option for you.
- #5 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
Because the clinical presentation and prognosis in IgA nephropathy varies widely, treatment must be individualized. All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Control of proteinuria is prudent, since there is a mostly linear association between the severity of proteinuria and decline in estimated glomerular filtration rate (GFR). Use of immunosuppression should be determined by considering the rate of progression, comorbidities, and whether alarming features are present on biopsy. However, immunosuppression has become controversial. Currently, corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. Sodiumglucose transporter 2 (SGLT2) inhibitors, which have an established role in chronic kidney disease generally, have also demonstrated benefit in IgA nephropathy.
- #6 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The management of IgAN begins with confirming the diagnosis, typically through a renal biopsy, while ruling out secondary causes. Key considerations for formulating the management plan include assessing proteinuria, eGFR, blood pressure, and histological findings. The primary goals of treatment are to induce remission and avert the onset of complications. […] First-line agents for managing proteinuria and lowering blood pressure in IgAN patients typically involve angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). The target blood pressure is typically set at 130/80 mm Hg. If proteinuria is more than 1 g/d, the systolic blood pressure target is less than 125 mm Hg. […] Conservative therapy plays a crucial role in managing IgAN by reducing proteinuria and slowing the rate of renal function decline in IgAN, which cannot be overemphasized. In the Supportive Versus Immunosuppressive Therapy of Progressive IgA Nephropathy (STOP-IgAN) study, around one-third of patients achieved satisfactory outcomes without requiring immunosuppressive therapy following a 6-month trial of optimizing conservative measures, including aggressive ACE inhibitors or ARB titration (optimal medical management). In this study, the comparison between immunosuppressive therapy and optimal medical management alone revealed no significant improvement in primary outcomesâdeath, ESRD, or a decline in GFR exceeding 40%âamong those receiving immunosuppressive treatment during the 3-year and 10-year follow-up periods.
- #7 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
Because the clinical presentation and prognosis in IgA nephropathy varies widely, treatment must be individualized. All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Control of proteinuria is prudent, since there is a mostly linear association between the severity of proteinuria and decline in estimated glomerular filtration rate (GFR). Use of immunosuppression should be determined by considering the rate of progression, comorbidities, and whether alarming features are present on biopsy. However, immunosuppression has become controversial. Currently, corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. Sodiumglucose transporter 2 (SGLT2) inhibitors, which have an established role in chronic kidney disease generally, have also demonstrated benefit in IgA nephropathy.
- #8 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. […] Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Immunosuppression has become controversial. Corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. […] The US Food and Drug Administration (FDA) has approved the following agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo), Sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist, Atrasentan, an endothelin type A receptor antagonist, Iptacopan (Fabhalta), a complement inhibitor. […] Kidney transplantation is effective in patients with IgA nephropathy that has progressed to end-stage kidney disease. However, the disorder frequently recurs after transplantation.
- #9 IgA Nephropathy (Berger’s Disease): ERKNet for Patientshttps://www.erknet.org/patients/your-kidney-disease/iga-nephropathy/disease-information
The choice of the therapeutic approach varies according to the individuals condition and in particular the estimated risk of progression to end-stage kidney disease (ESKD). A proper early diagnosis may give the opportunity for early treatment and for the improvement of the prognosis. It is still unknown why 30-60% of children and adults with IgAN will never experience a decrease in kidney function during life, while about 10% of them will develop symptoms of chronic kidney failure within 10 years from the diagnostic kidney biopsy, or within 20 years additional 20-30% of patients. […] Essential at any stage of the disease are changes in lifestyle, consisting of: Increasing regular physical activity focusing on endurance sports (avoiding high intensity sports (e.g. heavy weight lifting) Weight control for prevention/reduction of obesity Healthy diet and correction of possible lipid disorders No smoking (smoking increases the risk of dialysis 5-10-fold!) Low salt intake Avoidance of using so-called non-steroid antiphlogistic drugs as pain killers, e.g. diclofenac, ibuprofen, indomethacine (aspirin, paracetamol and opiates are safe as far as the kidneys are concerned) 1.5 liters of daily fluid is enough unless patients have either kidney stones or repeated bladder infections in addition to the IgAN. All these measures greatly improve the prognosis and decrease the risk of progression to kidney failure in IgAN.
- #10 IgA Nephropathy (Berger’s Disease) Treatment Optionshttps://www.healthline.com/health/treatment-options-for-iga-nephropathy
A healthcare professional may recommend a variety of lifestyle changes to help you manage IgA nephropathy. […] These dietary modifications may include: Limiting the amount of sodium in your diet, following a diet that is low in saturated fats and cholesterol, shifting toward a plant-based diet. […] A healthcare professional may also advise you to eat protein in moderation. […] Fish oil supplements that contain omega-3 fatty acids may help slow kidney damage by lowering blood pressure. […] High blood pressure and heart or cardiovascular problems may be treated with medication. […] Kidney failure may require dialysis or a kidney transplant. […] Participating in a clinical trial may be a treatment option for some people with IgA nephropathy. […] Treatment options include non-immunosuppressive and immunosuppressive medications and cholesterol-lowering medications. Your doctor might also recommend lifestyle and dietary changes to manage IgA nephropathy. […] Further treatments, such as dialysis and kidney transplant, may be required for complications from IgA nephropathy.
- #11 IgA Nephropathy (IgAN) | National Kidney Foundationhttps://www.kidney.org/kidney-topics/iga-nephropathy
There is no cure for IgAN. So, treatment is focused on managing symptoms, managing your blood pressure, and slowing the kidney disease process. Newer medications that directly target the IgAN disease process are also available, with more being studied in clinical trials. […] ACE inhibitors or ARBs: these medicines lower the amount of protein in your urine, lower your blood pressure, and help slow down the CKD disease process. Even if you don’t have high blood pressure, they can help slow the kidney damage caused by IgAN. These medicines have been used the longest for treating IgAN and are considered first line. […] SGLT2 inhibitors: these medicines also lower the amount of protein in your urine and help slow down the CKD disease process. They can also help you manage your blood sugar levels if you have diabetes. SGLT2 inhibitors can be used together with an ACE inhibitor or ARB.
- #12 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The management of IgAN begins with confirming the diagnosis, typically through a renal biopsy, while ruling out secondary causes. Key considerations for formulating the management plan include assessing proteinuria, eGFR, blood pressure, and histological findings. The primary goals of treatment are to induce remission and avert the onset of complications. […] First-line agents for managing proteinuria and lowering blood pressure in IgAN patients typically involve angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). The target blood pressure is typically set at 130/80 mm Hg. If proteinuria is more than 1 g/d, the systolic blood pressure target is less than 125 mm Hg. […] Conservative therapy plays a crucial role in managing IgAN by reducing proteinuria and slowing the rate of renal function decline in IgAN, which cannot be overemphasized. In the Supportive Versus Immunosuppressive Therapy of Progressive IgA Nephropathy (STOP-IgAN) study, around one-third of patients achieved satisfactory outcomes without requiring immunosuppressive therapy following a 6-month trial of optimizing conservative measures, including aggressive ACE inhibitors or ARB titration (optimal medical management). In this study, the comparison between immunosuppressive therapy and optimal medical management alone revealed no significant improvement in primary outcomesâdeath, ESRD, or a decline in GFR exceeding 40%âamong those receiving immunosuppressive treatment during the 3-year and 10-year follow-up periods.
- #13 IgA nephropathy (Berger disease) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/iga-nephropathy/diagnosis-treatment/drc-20352274
There’s no cure for IgA nephropathy. There’s also no sure way to predict how much the disease will affect your health over time. Some people need only medical tests to track whether the disease is getting worse. […] For others, medicines can slow the disease from becoming worse and help manage symptoms. […] Medicines to treat IgA nephropathy include: […] High blood pressure drugs. Medicines called angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) can lower blood pressure and reduce how much protein the body loses. […] Medicines that calm the immune system. These also are called immunosuppressants. They include corticosteroids and other strong medicines that can lessen the body’s defenses. They might keep the immune system from making the proteins that attack the glomeruli. These medicines can cause serious side effects, such as high blood pressure, high blood sugar and a greater risk of infection. Your doctor helps you choose an immunosuppressant medicine. Many new treatments have been approved or are being tested in clinical trials. Talk with your doctor about the latest advances in the treatment of IgA nephropathy.
- #14 IgA Nephropathy (Berger’s Disease) | Doctorhttps://patient.info/doctor/iga-nephropathy-bergers-disease-pro
No specific therapy is available for IgAN so the aim is supportive management. Lowering blood pressure and renin-angiotensin system inhibition remain the cornerstone of management. Patients should be advised to follow a low-salt diet. […] Angiotensin-converting enzyme (ACE) inhibitors/angiotensin-II receptor antagonists (AIIRAs): Hypertension needs early and aggressive treatment, titrating up to maximum tolerated dose. ACE inhibitors or AIIRAs are the drugs of choice. They protect kidney function and are also beneficial when blood pressure is normal. […] Steroid therapy is associated with a decrease of proteinuria and with a statistically significant reduction of the risk of ESKD. Corticosteroids should be given for six months to patients with preserved kidney function, nephrotic syndrome and few histological changes on light microscopy. A typical regime is 1 g of intravenous methylprednisolone for three consecutive days at the beginning of months one, three and five, with low-dose oral steroids every other day for six months. There may be benefit in extending beyond the six-month period.
- #15 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The management of IgAN begins with confirming the diagnosis, typically through a renal biopsy, while ruling out secondary causes. Key considerations for formulating the management plan include assessing proteinuria, eGFR, blood pressure, and histological findings. The primary goals of treatment are to induce remission and avert the onset of complications. […] First-line agents for managing proteinuria and lowering blood pressure in IgAN patients typically involve angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). The target blood pressure is typically set at 130/80 mm Hg. If proteinuria is more than 1 g/d, the systolic blood pressure target is less than 125 mm Hg. […] Conservative therapy plays a crucial role in managing IgAN by reducing proteinuria and slowing the rate of renal function decline in IgAN, which cannot be overemphasized. In the Supportive Versus Immunosuppressive Therapy of Progressive IgA Nephropathy (STOP-IgAN) study, around one-third of patients achieved satisfactory outcomes without requiring immunosuppressive therapy following a 6-month trial of optimizing conservative measures, including aggressive ACE inhibitors or ARB titration (optimal medical management). In this study, the comparison between immunosuppressive therapy and optimal medical management alone revealed no significant improvement in primary outcomesâdeath, ESRD, or a decline in GFR exceeding 40%âamong those receiving immunosuppressive treatment during the 3-year and 10-year follow-up periods.
- #16 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The management of IgAN begins with confirming the diagnosis, typically through a renal biopsy, while ruling out secondary causes. Key considerations for formulating the management plan include assessing proteinuria, eGFR, blood pressure, and histological findings. The primary goals of treatment are to induce remission and avert the onset of complications. […] First-line agents for managing proteinuria and lowering blood pressure in IgAN patients typically involve angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). The target blood pressure is typically set at 130/80 mm Hg. If proteinuria is more than 1 g/d, the systolic blood pressure target is less than 125 mm Hg. […] Conservative therapy plays a crucial role in managing IgAN by reducing proteinuria and slowing the rate of renal function decline in IgAN, which cannot be overemphasized. In the Supportive Versus Immunosuppressive Therapy of Progressive IgA Nephropathy (STOP-IgAN) study, around one-third of patients achieved satisfactory outcomes without requiring immunosuppressive therapy following a 6-month trial of optimizing conservative measures, including aggressive ACE inhibitors or ARB titration (optimal medical management). In this study, the comparison between immunosuppressive therapy and optimal medical management alone revealed no significant improvement in primary outcomesâdeath, ESRD, or a decline in GFR exceeding 40%âamong those receiving immunosuppressive treatment during the 3-year and 10-year follow-up periods.
- #17 IgA Nephropathy (IgAN) | National Kidney Foundationhttps://www.kidney.org/kidney-topics/iga-nephropathy
There is no cure for IgAN. So, treatment is focused on managing symptoms, managing your blood pressure, and slowing the kidney disease process. Newer medications that directly target the IgAN disease process are also available, with more being studied in clinical trials. […] ACE inhibitors or ARBs: these medicines lower the amount of protein in your urine, lower your blood pressure, and help slow down the CKD disease process. Even if you don’t have high blood pressure, they can help slow the kidney damage caused by IgAN. These medicines have been used the longest for treating IgAN and are considered first line. […] SGLT2 inhibitors: these medicines also lower the amount of protein in your urine and help slow down the CKD disease process. They can also help you manage your blood sugar levels if you have diabetes. SGLT2 inhibitors can be used together with an ACE inhibitor or ARB.
- #18 IgA nephropathy | Kidney Care UKhttps://kidneycareuk.org/kidney-disease-information/kidney-conditions/iga-nephropathy/
Some people with IgAN do not need any treatment and the condition remain stable over time. […] If treatment is needed, the initial aim is to protect kidney function by lowering blood pressure via lifestyle measures such as losing weight, stopping smoking and reducing salt in the diet. […] Blood pressure medication may also be prescribed, such as angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). […] If you have protein in the urine, you may be prescribed blood pressure medication, even if you do not have high blood pressure. […] SGLT2 (sodium-glucose co-transporter 2) inhibitors, are also often prescribed to slow the progression of IgAN. […] If such supportive treatment methods do not help, immunosuppressants such as steroids may be prescribed, to dampen the immune system.
- #19 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
SGLT inhibitors demonstrated significant efficacy in slowing the progression of CKD. The DAPA-CKD and EMPA-KIDNEY trials were terminated early due to the evident benefits of SGLT2 inhibitors. Although these trials primarily included patients with all-cause CKD, a significant number of patients from both trials were diagnosed with IgAN. Further studies are needed to evaluate the effects of SGLT2 inhibitors on patients with IgAN. […] Combination therapy involving corticosteroids along with another agent is typically considered for cases of progressive IgAN. A randomized prospective trial focusing on high-risk IgAN patients showed improved renal survival in those receiving prednisone along with cyclophosphamide or azathioprine compared to those without immunotherapy. However, results reported in another multicenter trial found no significant difference between patients treated with corticosteroids alone and those receiving combination therapy.
- #20 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The FDA has approved targeted-release budesonide (Tarpeyo) for proteinuria reduction in adults with primary IgA nephropathy who are at risk for rapid disease progression; the FDA issued accelerated approval for this indication in 2021 and converted it to full approval in December 2023. […] The Dapagliflozin and Prevention of Adverse outcomes in Chronic Kidney Disease (DAPA-CKD) and the Study of Heart and Kidney Protection With Empagliflozin trials confirmed that the SGLT2 inhibitors dapagliflozin and empagliflozin have nephroprotective effects in nondiabetic patients with albuminuric chronic kidney disease, and that the benefit extended to patients with eGFR 30 mL/min/1.73 m2. […] Current evidence does not support the use of fish oil as monotherapy, but some physicians combine fish oil with other therapies. KDIGO guidelines suggest that fish oil may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D). […] Tonsillectomy is a controversial treatment for IgA nephropathy. […] Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%).
- #21 IgA Nephropathy (Berger’s Disease): ERKNet for Patientshttps://www.erknet.org/patients/your-kidney-disease/iga-nephropathy/disease-information
In both adult and paediatric patients at risk for progressive kidney disease, renin-angiotensin-aldosterone system inhibitors, i.e. ACEi (angiotensin converting enzyme inhibitors) or ARBs (angiotensin receptor blockers) constitute the basis of therapy, since these drugs have reduce arterial blood pressure, and, by lowering the blood pressure in the glomerulus of the kidneys, reduce protein loss in the urine and slow down scarring of the kidneys. This treatment usually begins at the time of diagnosis and is calibrated on the severity of proteinuria and the occurrence of hypertension. […] A new class of drugs to protects the kidneys, which like ACEi and ARBs have very little side effects, are so-called SGLT2-inhibitors. These drugs, e.g., dapagliflozine and empagliflozine, block glucose uptake in the nephron such that the urine suddenly contains a lot of sugar and this relief from work apparently protects the kidney function. For children the therapy with SGLT1-Inhibitors is not yet authorised nor has it been investigated in clinical trials, so its use still needs to be confirmed in this population.
- #22 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Sparsentan is a non-immunosuppressive, novel dual endothelin and angiotensin receptor antagonist, which received accelerated approval from the US Food and Drug Administration (FDA) for the treatment of IgAN in February 2023. The PROTECT trial was a multicenter randomized controlled trial that compared the effects of irbesartan (300 mg) with sparsentan (400 mg) on proteinuria in patients with persistent proteinuria despite maximal ACE inhibitor or ARB therapy. Results showed that the sparsentan group exhibited significantly less proteinuria and a trend toward preserved GFR compared to the irbesartan group. […] Budesonide, in the form of oral nefecon, is a novel therapy for IgAN. This formulation consists of budesonide packaged in a pH-sensitive capsule, which preferentially releases the active drug at the terminal ileum near Peyer patchesâthe site of significant IgA-producing lymphoid tissue. This targeted delivery system enables extensive first-pass metabolism, minimizing systemic effects. Results from the NEFIGAN and NeflgArd trials have shown significant reductions in proteinuria and preservation of GFR with the use of nefecon. Ongoing data analysis from these trials continues to provide insights into its efficacy.
- #23 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The US Food and Drug Administration (FDA) has approved several agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo); sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist; atrasentan (Vanrafia), an endothelin-1 receptor antagonist; and iptacopan (Fabhalta), a complement inhibitor. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. Most nephrologists agree with not using immunosuppression when the GFR is less than 30 mL/min/1.73 m2, although others would suggest a different GFR threshold. Regardless, the lower the GFR, the higher the risk of adverse events and the lower the likelihood of benefiting from immunosuppressants. Immunosuppression should definitely be avoided when the biopsy shows large amounts of interstitial fibrosis and tubular atrophy.
- #24 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Sparsentan is a non-immunosuppressive, novel dual endothelin and angiotensin receptor antagonist, which received accelerated approval from the US Food and Drug Administration (FDA) for the treatment of IgAN in February 2023. The PROTECT trial was a multicenter randomized controlled trial that compared the effects of irbesartan (300 mg) with sparsentan (400 mg) on proteinuria in patients with persistent proteinuria despite maximal ACE inhibitor or ARB therapy. Results showed that the sparsentan group exhibited significantly less proteinuria and a trend toward preserved GFR compared to the irbesartan group. […] Budesonide, in the form of oral nefecon, is a novel therapy for IgAN. This formulation consists of budesonide packaged in a pH-sensitive capsule, which preferentially releases the active drug at the terminal ileum near Peyer patchesâthe site of significant IgA-producing lymphoid tissue. This targeted delivery system enables extensive first-pass metabolism, minimizing systemic effects. Results from the NEFIGAN and NeflgArd trials have shown significant reductions in proteinuria and preservation of GFR with the use of nefecon. Ongoing data analysis from these trials continues to provide insights into its efficacy.
- #25 First-in-class treatment delivers major advance for incurable kidney disease | The George Institute for Global Healthhttps://www.georgeinstitute.org/media-releases/first-in-class-treatment-delivers-major-advance-for-incurable-kidney-disease
The latest findings from the PROTECT phase III trial show sparsentan – a novel treatment for IgA nephropathy – significantly reduces proteinuria, or abnormal protein levels in the urine, compared to standard treatment irbesartan. […] This data shows sparsentan was responsible for a rapid and sustained reduction in proteinuria, and this has the potential to prevent progressive kidney function loss in patients with the disease. […] Sparsentan represents a new class of drug that promises great benefit for patients with IgA nephropathy. […] The PROTECT trial will continue for another 110 weeks, to validate the finding that sparsentan compared to irbesartan not only reduces proteinuria, but also slows the decline of estimated glomerular filtration rate (eGFR) – another measure of kidney function.
- #26 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. […] Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Immunosuppression has become controversial. Corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. […] The US Food and Drug Administration (FDA) has approved the following agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo), Sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist, Atrasentan, an endothelin type A receptor antagonist, Iptacopan (Fabhalta), a complement inhibitor. […] Kidney transplantation is effective in patients with IgA nephropathy that has progressed to end-stage kidney disease. However, the disorder frequently recurs after transplantation.
- #27 IgA Nephropathy (IgAN) | National Kidney Foundationhttps://www.kidney.org/kidney-topics/iga-nephropathy
Endothelin receptor [type A] antagonists (ERAs): this is a newer class of medicines approved for IgAN that helps the blood vessels in your kidneys relax. This lowers the amount of protein in your urine and is expected to help slow down the CKD disease process. […] Corticosteroids (often called steroids): these medicines help to calm your immune system (your body’s defense system), so it makes less IgA. This helps give your kidneys a chance to relax and try to recover. Steroids are usually only recommended for a short period of time (6-9 months). This helps to maximize the benefits while trying to minimize the risk of side effects like high blood sugar, weight gain, and bone weakening. […] To lower the risk of side effects from steroids even more, a targeted release version of budesonide is available. It is a capsule that is taken by mouth and delivers budesonide (a steroid) directly to the part of your body where much of this IgA is made.
- #28 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Corticosteroids are crucial in reducing proteinuria in IgAN, particularly when levels exceed 1g/d. Steroid administration has especially been shown to decrease proteinuria. Typically, a tapering course of prednisone is prescribed for 2 to 4 months. […] The TESTING trial, which focused on patients with IgAN and proteinuria exceeding 1 g/d, demonstrated that treatment with methylprednisolone for 6 to 9 months significantly slowed disease progression compared to placebo. However, due to an excess of infections and adverse events, the initial dose of methylprednisolone had to be reduced, and cotrimoxazole prophylaxis for opportunistic infections was added. Nonetheless, longer-term data suggests that these effects may not be long-lasting. […] In recent years, the approval of several novel medications holds promise for significantly improving outcomes in IgAN.
- #29 IgA nephropathy (Berger disease) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/iga-nephropathy/diagnosis-treatment/drc-20352274
There’s no cure for IgA nephropathy. There’s also no sure way to predict how much the disease will affect your health over time. Some people need only medical tests to track whether the disease is getting worse. […] For others, medicines can slow the disease from becoming worse and help manage symptoms. […] Medicines to treat IgA nephropathy include: […] High blood pressure drugs. Medicines called angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) can lower blood pressure and reduce how much protein the body loses. […] Medicines that calm the immune system. These also are called immunosuppressants. They include corticosteroids and other strong medicines that can lessen the body’s defenses. They might keep the immune system from making the proteins that attack the glomeruli. These medicines can cause serious side effects, such as high blood pressure, high blood sugar and a greater risk of infection. Your doctor helps you choose an immunosuppressant medicine. Many new treatments have been approved or are being tested in clinical trials. Talk with your doctor about the latest advances in the treatment of IgA nephropathy.
- #30 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Corticosteroids are crucial in reducing proteinuria in IgAN, particularly when levels exceed 1g/d. Steroid administration has especially been shown to decrease proteinuria. Typically, a tapering course of prednisone is prescribed for 2 to 4 months. […] The TESTING trial, which focused on patients with IgAN and proteinuria exceeding 1 g/d, demonstrated that treatment with methylprednisolone for 6 to 9 months significantly slowed disease progression compared to placebo. However, due to an excess of infections and adverse events, the initial dose of methylprednisolone had to be reduced, and cotrimoxazole prophylaxis for opportunistic infections was added. Nonetheless, longer-term data suggests that these effects may not be long-lasting. […] In recent years, the approval of several novel medications holds promise for significantly improving outcomes in IgAN.
- #31 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
Guidelines for IgA nephropathy from Kidney Disease: Improving Global Outcomes (KDIGO) suggest that a 6-month course of corticosteroid therapy may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control), and a glomerular filtration rate (GFR) 50 mL/min/1.73m2. […] The KDIGO guidelines suggest not treating with corticosteroids combined with cyclophosphamide or azathioprine unless the patient has crescentic IgA nephropathy with rapidly deteriorating kidney function. […] The Supportive Versus Immunosuppressive Therapy for the Treatment of Progressive IgA Nephropathy (STOP-IgAN) trial showed that after 3 years, full clinical remission had occurred in 5% of patients in the supportive-care group, as compared with 17% of patients who received immunosuppression with steroids plus cyclophosphamide followed by azathioprine.
- #32 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
Because the clinical presentation and prognosis in IgA nephropathy varies widely, treatment must be individualized. All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Control of proteinuria is prudent, since there is a mostly linear association between the severity of proteinuria and decline in estimated glomerular filtration rate (GFR). Use of immunosuppression should be determined by considering the rate of progression, comorbidities, and whether alarming features are present on biopsy. However, immunosuppression has become controversial. Currently, corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. Sodiumglucose transporter 2 (SGLT2) inhibitors, which have an established role in chronic kidney disease generally, have also demonstrated benefit in IgA nephropathy.
- #33 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. […] Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Immunosuppression has become controversial. Corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. […] The US Food and Drug Administration (FDA) has approved the following agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo), Sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist, Atrasentan, an endothelin type A receptor antagonist, Iptacopan (Fabhalta), a complement inhibitor. […] Kidney transplantation is effective in patients with IgA nephropathy that has progressed to end-stage kidney disease. However, the disorder frequently recurs after transplantation.
- #34 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Sparsentan is a non-immunosuppressive, novel dual endothelin and angiotensin receptor antagonist, which received accelerated approval from the US Food and Drug Administration (FDA) for the treatment of IgAN in February 2023. The PROTECT trial was a multicenter randomized controlled trial that compared the effects of irbesartan (300 mg) with sparsentan (400 mg) on proteinuria in patients with persistent proteinuria despite maximal ACE inhibitor or ARB therapy. Results showed that the sparsentan group exhibited significantly less proteinuria and a trend toward preserved GFR compared to the irbesartan group. […] Budesonide, in the form of oral nefecon, is a novel therapy for IgAN. This formulation consists of budesonide packaged in a pH-sensitive capsule, which preferentially releases the active drug at the terminal ileum near Peyer patchesâthe site of significant IgA-producing lymphoid tissue. This targeted delivery system enables extensive first-pass metabolism, minimizing systemic effects. Results from the NEFIGAN and NeflgArd trials have shown significant reductions in proteinuria and preservation of GFR with the use of nefecon. Ongoing data analysis from these trials continues to provide insights into its efficacy.
- #35 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The primary end point was UPCR 0.2 g/day and a decrease in eGFR 5 mL/min/1.73 m2 from baseline at 3 years. There was no significant difference in the annual decline in eGFR between the two groups, and patients in the immunosuppression arm were more likely to experience significant adverse effects (severe infections, impaired glucose tolerance, and weight gain of more than 5 kg in the first year of treatment). […] A 2015 Cochrane review of immunosuppressive therapy for IgA nephropathy concluded that corticosteroid therapy may lower risks of kidney disease progression, proteinuria, doubling of serum creatinine, and need for dialysis or transplantation. However, the review concluded that the optimal management of IgA nephropathy remains uncertain, and larger controlled trials are needed. […] The phase 2b NEFIGAN trial demonstrated a 24% decrease in mean UPCR in patients receiving a novel targeted-release formulation of oral budesonide that delivers the drug to the distal ileum, thus targeting the Peyer patches; this agent is twice as potent as prednisone.
- #36 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Sparsentan is a non-immunosuppressive, novel dual endothelin and angiotensin receptor antagonist, which received accelerated approval from the US Food and Drug Administration (FDA) for the treatment of IgAN in February 2023. The PROTECT trial was a multicenter randomized controlled trial that compared the effects of irbesartan (300 mg) with sparsentan (400 mg) on proteinuria in patients with persistent proteinuria despite maximal ACE inhibitor or ARB therapy. Results showed that the sparsentan group exhibited significantly less proteinuria and a trend toward preserved GFR compared to the irbesartan group. […] Budesonide, in the form of oral nefecon, is a novel therapy for IgAN. This formulation consists of budesonide packaged in a pH-sensitive capsule, which preferentially releases the active drug at the terminal ileum near Peyer patchesâthe site of significant IgA-producing lymphoid tissue. This targeted delivery system enables extensive first-pass metabolism, minimizing systemic effects. Results from the NEFIGAN and NeflgArd trials have shown significant reductions in proteinuria and preservation of GFR with the use of nefecon. Ongoing data analysis from these trials continues to provide insights into its efficacy.
- #37 IgA nephropathy (IgAN): symptoms, treatments, diagnosis and causeshttps://www.kidneyresearchuk.org/conditions-symptoms/iga-nephropathy/
Current treatments focus on: […] You may also be offered medications called immunosuppressives which dampen down your immune system. […] In November 2023, NICE issued final draft guidance recommending the use of the first licensed treatment for IgAN, called targeted-release budesonide. Targeted-release budesonide is a type of corticosteroid (a drug that mimics the effects of hormones produced by the adrenal glands) that reduces the production of IgA antibodies in the small intestine to prevent build-up in the kidneys which causes inflammation and scarring, ultimately leading to potential kidney failure. Budesonide will only be used as an add-on to existing care alongside angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARBs).
- #38 IgA Nephropathy (Berger’s Disease) | Doctorhttps://patient.info/doctor/iga-nephropathy-bergers-disease-pro
The National Institute for Health and Care Excellence (NICE) has recommended targeted-release budesonide as an option for treating primary IgAN when there is a risk of rapid disease progression in adults with a urine protein-to-creatinine ratio of 1.5 g/g or more. It can only be used as an adjunct to optimised standard care including the highest tolerated licensed dose of angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARBs), unless these are contraindicated. […] There is evidence that tonsillectomy slows down the progression of IgA nephropathy. However, it is not currently recommended in guidelines, unless otherwise indicated due to recurrent infection.
- #39 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The FDA has approved targeted-release budesonide (Tarpeyo) for proteinuria reduction in adults with primary IgA nephropathy who are at risk for rapid disease progression; the FDA issued accelerated approval for this indication in 2021 and converted it to full approval in December 2023. […] The Dapagliflozin and Prevention of Adverse outcomes in Chronic Kidney Disease (DAPA-CKD) and the Study of Heart and Kidney Protection With Empagliflozin trials confirmed that the SGLT2 inhibitors dapagliflozin and empagliflozin have nephroprotective effects in nondiabetic patients with albuminuric chronic kidney disease, and that the benefit extended to patients with eGFR 30 mL/min/1.73 m2. […] Current evidence does not support the use of fish oil as monotherapy, but some physicians combine fish oil with other therapies. KDIGO guidelines suggest that fish oil may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D). […] Tonsillectomy is a controversial treatment for IgA nephropathy. […] Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%).
- #40 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The US Food and Drug Administration (FDA) has approved several agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo); sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist; atrasentan (Vanrafia), an endothelin-1 receptor antagonist; and iptacopan (Fabhalta), a complement inhibitor. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. Most nephrologists agree with not using immunosuppression when the GFR is less than 30 mL/min/1.73 m2, although others would suggest a different GFR threshold. Regardless, the lower the GFR, the higher the risk of adverse events and the lower the likelihood of benefiting from immunosuppressants. Immunosuppression should definitely be avoided when the biopsy shows large amounts of interstitial fibrosis and tubular atrophy.
- #41 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. […] Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Immunosuppression has become controversial. Corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. […] The US Food and Drug Administration (FDA) has approved the following agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo), Sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist, Atrasentan, an endothelin type A receptor antagonist, Iptacopan (Fabhalta), a complement inhibitor. […] Kidney transplantation is effective in patients with IgA nephropathy that has progressed to end-stage kidney disease. However, the disorder frequently recurs after transplantation.
- #42 IgA nephropathy – Wikipediahttps://en.wikipedia.org/wiki/IgA_nephropathy
However, in patients with aggressive Berger’s disease 6 months regimen of steroids in addition to other medications may lessen proteinuria and preserve renal function. […] Cyclophosphamide (traded as endoxan and cytoxan) and Isotretinoin have commonly been used, often with anti-platelet/anticoagulants in patients with Aggressive Berger’s disease, however, the side effect profile of these drugs, including long term risk of malignancy and sterility, made them an unfavorable choice for use in young adults. […] However, one recent study, in a carefully selected high risk population of patients with declining GFR, showed that a combination of steroids and cyclophosphamide for the initial 3 months followed by azathioprine for a minimum of 2 years resulted in a significant preservation of renal function.
- #43 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
Guidelines for IgA nephropathy from Kidney Disease: Improving Global Outcomes (KDIGO) suggest that a 6-month course of corticosteroid therapy may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control), and a glomerular filtration rate (GFR) 50 mL/min/1.73m2. […] The KDIGO guidelines suggest not treating with corticosteroids combined with cyclophosphamide or azathioprine unless the patient has crescentic IgA nephropathy with rapidly deteriorating kidney function. […] The Supportive Versus Immunosuppressive Therapy for the Treatment of Progressive IgA Nephropathy (STOP-IgAN) trial showed that after 3 years, full clinical remission had occurred in 5% of patients in the supportive-care group, as compared with 17% of patients who received immunosuppression with steroids plus cyclophosphamide followed by azathioprine.
- #44 IgA nephropathy – Wikipediahttps://en.wikipedia.org/wiki/IgA_nephropathy
However, in patients with aggressive Berger’s disease 6 months regimen of steroids in addition to other medications may lessen proteinuria and preserve renal function. […] Cyclophosphamide (traded as endoxan and cytoxan) and Isotretinoin have commonly been used, often with anti-platelet/anticoagulants in patients with Aggressive Berger’s disease, however, the side effect profile of these drugs, including long term risk of malignancy and sterility, made them an unfavorable choice for use in young adults. […] However, one recent study, in a carefully selected high risk population of patients with declining GFR, showed that a combination of steroids and cyclophosphamide for the initial 3 months followed by azathioprine for a minimum of 2 years resulted in a significant preservation of renal function.
- #45 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The US Food and Drug Administration (FDA) has approved several agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo); sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist; atrasentan (Vanrafia), an endothelin-1 receptor antagonist; and iptacopan (Fabhalta), a complement inhibitor. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. Most nephrologists agree with not using immunosuppression when the GFR is less than 30 mL/min/1.73 m2, although others would suggest a different GFR threshold. Regardless, the lower the GFR, the higher the risk of adverse events and the lower the likelihood of benefiting from immunosuppressants. Immunosuppression should definitely be avoided when the biopsy shows large amounts of interstitial fibrosis and tubular atrophy.
- #46 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. […] Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Immunosuppression has become controversial. Corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. […] The US Food and Drug Administration (FDA) has approved the following agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo), Sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist, Atrasentan, an endothelin type A receptor antagonist, Iptacopan (Fabhalta), a complement inhibitor. […] Kidney transplantation is effective in patients with IgA nephropathy that has progressed to end-stage kidney disease. However, the disorder frequently recurs after transplantation.
- #47 IgA Nephropathy (Bergerâs Disease): Causes, Symptoms, & Treatmenthttps://www.webmd.com/a-to-z-guides/what-is-iga-nephropathy
Currently, there is no cure for IgA nephropathy, but there have been a lot of new medications developed and approved for the treatment of IgA nephropathy that seem to control protein loss and help decrease the fall in kidney function over time, says Rovin. These include drugs that act on the pathways that result in formation of abnormal IgA molecules, drugs that decrease inflammation in the kidneys, and drugs that act within the kidney to slow disease progression. […] Clinical trials of some drugs that slow the progressive decline in people with other types of chronic kidney diseases may show promise for people with IgA nephropathy. […] These meds can: […] Reduce the protein in your urine. Two newer medications that do that are sparsentan (Filspari) and iptacopan (Fabhalta). Sparsentan blocks a receptor that can reduce blood flow in the kidney. Less blood flow means waste is not efficiently filtered, leading to protein in the urine. One advantage of sparsentan over other medications for IgA nephropathy is that it does not suppress your immune system, lowering your risk for infections. Iptacopan acts on a specific process in the kidneys that, when disrupted, can release more protein into the urine.
- #48 IgA nephropathy (Berger disease) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/iga-nephropathy/diagnosis-treatment/drc-20352274
Omega-3 fatty acids. These healthy fats might curb inflammation in the glomeruli without harmful side effects. You can get omega-3s from fish oil supplements. But don’t buy those off the shelf. Ask your doctor if prescription fish oil supplements might help you. […] Cholesterol medicine. If you have high cholesterol, this type of medicine can help control it and slow down kidney damage. […] Diuretics. These can help control swelling in the hands and feet called edema. […] The main goal of treatment is to keep you from needing dialysis or a kidney transplant. But either of those treatments can be lifesaving if your kidneys stop working well enough on their own.
- #49 IgA Nephropathy (Berger’s Disease) Treatment Optionshttps://www.healthline.com/health/treatment-options-for-iga-nephropathy
Non-immunosuppressive medications for IgA nephropathy include: angiotensin-converting enzyme inhibitors (ACE inhibitors), angiotensin receptor blockers (ARBs). […] Another non-immunosuppressive medication is sparsentan, an endothelin and angiotensin II receptor antagonist. It lowers levels of protein in the urine in people with IgA nephropathy whose condition may progress quickly. […] Immunosuppressive medications for IgA nephropathy include: cyclophosphamide, corticosteroids, such as prednisone. […] Budesonide is another corticosteroid a doctor may prescribe for IgA nephropathy. It helps decrease inflammation in the kidneys and reduce levels of protein in the urine in people whose condition may progress quickly. […] Doctors may prescribe medications called statins to help decrease cholesterol levels.
- #50 IgA nephropathy (Berger disease) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/iga-nephropathy/diagnosis-treatment/drc-20352274
Omega-3 fatty acids. These healthy fats might curb inflammation in the glomeruli without harmful side effects. You can get omega-3s from fish oil supplements. But don’t buy those off the shelf. Ask your doctor if prescription fish oil supplements might help you. […] Cholesterol medicine. If you have high cholesterol, this type of medicine can help control it and slow down kidney damage. […] Diuretics. These can help control swelling in the hands and feet called edema. […] The main goal of treatment is to keep you from needing dialysis or a kidney transplant. But either of those treatments can be lifesaving if your kidneys stop working well enough on their own.
- #51 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
Treatment can slow the progress of the disease and prevent ESRD. If you have IgA nephropathy, your healthcare provider will probably refer you to a nephrologist, a kidney disease specialist. […] Treatment for IgA nephropathy includes medication to: […] Regulate blood pressure with angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), or other medicines […] Remove extra fluid with a diuretic […] Control your immune system to lower kidney inflammation with prescribed steroids such as prednisone or cyclophosphamide, a different medicine […] Lower your cholesterol levels with medications such as statins. […] Some research shows that a tonsillectomy (tonsil removal) can benefit a small portion of people who have IgA nephropathy. Talk to your healthcare provider to see if this may be an option for you.
- #52 IgA nephropathy (Berger’s disease) – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutubehttps://www.augustahealth.com/disease/iga-nephropathy-bergers-disease/
No cure exists for IgA nephropathy, but certain medications can slow its course. […] For others, a number of medications can slow disease progress and help manage symptoms. […] Medications to treat IgA nephropathy include: High blood pressure medications. Taking angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) can lower your blood pressure and reduce protein loss. […] Omega-3 fatty acids. These fats, available in dietary fish oil supplements, might reduce inflammation in the glomeruli without harmful side effects. […] Immunosuppressants. In some cases, corticosteroid medications, such as prednisone, and other potent drugs that suppress the immune response (immunosuppressants) might keep your immune system from attacking your glomeruli. […] Statin therapy. If you have high cholesterol, cholesterol-lowering medications can help control it and slow the progression of kidney damage. […] Diuretics. These help remove extra fluid from your blood. Removing extra fluid can help improve blood pressure control. […] The ultimate goal is to avoid the need for kidney dialysis or kidney transplantation. But in some cases, dialysis or transplantation is necessary.
- #53 IgA nephropathy (Berger disease) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/iga-nephropathy/diagnosis-treatment/drc-20352274
Omega-3 fatty acids. These healthy fats might curb inflammation in the glomeruli without harmful side effects. You can get omega-3s from fish oil supplements. But don’t buy those off the shelf. Ask your doctor if prescription fish oil supplements might help you. […] Cholesterol medicine. If you have high cholesterol, this type of medicine can help control it and slow down kidney damage. […] Diuretics. These can help control swelling in the hands and feet called edema. […] The main goal of treatment is to keep you from needing dialysis or a kidney transplant. But either of those treatments can be lifesaving if your kidneys stop working well enough on their own.
- #54 IgA Nephropathy (Berger’s Disease) Treatment Optionshttps://www.healthline.com/health/treatment-options-for-iga-nephropathy
A healthcare professional may recommend a variety of lifestyle changes to help you manage IgA nephropathy. […] These dietary modifications may include: Limiting the amount of sodium in your diet, following a diet that is low in saturated fats and cholesterol, shifting toward a plant-based diet. […] A healthcare professional may also advise you to eat protein in moderation. […] Fish oil supplements that contain omega-3 fatty acids may help slow kidney damage by lowering blood pressure. […] High blood pressure and heart or cardiovascular problems may be treated with medication. […] Kidney failure may require dialysis or a kidney transplant. […] Participating in a clinical trial may be a treatment option for some people with IgA nephropathy. […] Treatment options include non-immunosuppressive and immunosuppressive medications and cholesterol-lowering medications. Your doctor might also recommend lifestyle and dietary changes to manage IgA nephropathy. […] Further treatments, such as dialysis and kidney transplant, may be required for complications from IgA nephropathy.
- #55 IgA nephropathy – Wikipediahttps://en.wikipedia.org/wiki/IgA_nephropathy
A 1994 study by Mayo Clinic found that long-term treatment with omega3 fatty acid-rich fish oil, which does not have the drawbacks of immunosuppressive therapy, was associated with slight reduction of progression to kidney failure, without, however, reducing proteinuria in a subset of patients with high risk of worsening kidney function. […] The events that tend to lead to progressive kidney failure are not unique to IgA nephropathy, and non-specific measures to reduce the same would be equally useful. These include a low-protein diet and optimal control of blood pressure. […] In December 2021, budesonide (Tarpeyo) was approved for medical use in the US to reduce proteinuria in adults with primary IgA nephropathy at risk of rapid disease progression. […] Sparsentan is a therapy recently approved in the USA for treating primary IgA nephropathy. It is a dual endothelin angiotensin receptor antagonist that uniquely combines angiotensin and endothelin inhibition without immunosuppression. In clinical trials, sparsentan demonstrated significant reductions in proteinuria and better preservation of kidney function than irbesartan, a standard treatment.
- #56 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The FDA has approved targeted-release budesonide (Tarpeyo) for proteinuria reduction in adults with primary IgA nephropathy who are at risk for rapid disease progression; the FDA issued accelerated approval for this indication in 2021 and converted it to full approval in December 2023. […] The Dapagliflozin and Prevention of Adverse outcomes in Chronic Kidney Disease (DAPA-CKD) and the Study of Heart and Kidney Protection With Empagliflozin trials confirmed that the SGLT2 inhibitors dapagliflozin and empagliflozin have nephroprotective effects in nondiabetic patients with albuminuric chronic kidney disease, and that the benefit extended to patients with eGFR 30 mL/min/1.73 m2. […] Current evidence does not support the use of fish oil as monotherapy, but some physicians combine fish oil with other therapies. KDIGO guidelines suggest that fish oil may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D). […] Tonsillectomy is a controversial treatment for IgA nephropathy. […] Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%).
- #57 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The FDA has approved targeted-release budesonide (Tarpeyo) for proteinuria reduction in adults with primary IgA nephropathy who are at risk for rapid disease progression; the FDA issued accelerated approval for this indication in 2021 and converted it to full approval in December 2023. […] The Dapagliflozin and Prevention of Adverse outcomes in Chronic Kidney Disease (DAPA-CKD) and the Study of Heart and Kidney Protection With Empagliflozin trials confirmed that the SGLT2 inhibitors dapagliflozin and empagliflozin have nephroprotective effects in nondiabetic patients with albuminuric chronic kidney disease, and that the benefit extended to patients with eGFR 30 mL/min/1.73 m2. […] Current evidence does not support the use of fish oil as monotherapy, but some physicians combine fish oil with other therapies. KDIGO guidelines suggest that fish oil may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D). […] Tonsillectomy is a controversial treatment for IgA nephropathy. […] Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%).
- #58 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The FDA has approved targeted-release budesonide (Tarpeyo) for proteinuria reduction in adults with primary IgA nephropathy who are at risk for rapid disease progression; the FDA issued accelerated approval for this indication in 2021 and converted it to full approval in December 2023. […] The Dapagliflozin and Prevention of Adverse outcomes in Chronic Kidney Disease (DAPA-CKD) and the Study of Heart and Kidney Protection With Empagliflozin trials confirmed that the SGLT2 inhibitors dapagliflozin and empagliflozin have nephroprotective effects in nondiabetic patients with albuminuric chronic kidney disease, and that the benefit extended to patients with eGFR 30 mL/min/1.73 m2. […] Current evidence does not support the use of fish oil as monotherapy, but some physicians combine fish oil with other therapies. KDIGO guidelines suggest that fish oil may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D). […] Tonsillectomy is a controversial treatment for IgA nephropathy. […] Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%).
- #59 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The tonsils are a significant store for MALT, and IgA levels are known to decrease post-tonsillectomy. In addition, a correlation exists between tonsillar dendritic cell concentration and crescent formation in IgAN patients. Multiple retrospective studies have demonstrated the benefits of tonsillectomy in inducing remission or slowing IgAN progression, particularly in Asian populations, notably Japan. Tonsillectomy in conjunction with pulse steroid therapy has also been more recently studied and found to be effective in progressive IgAN. […] For individuals who progress to ESRD from IgAN, renal transplantation is a viable option. However, the risk of IgAN recurrence in the transplanted kidney persists. A significant study reported a recurrence rate of 23% in transplanted kidneys at 15 years, correlating with a 3.7-fold increase in graft rejection. Despite the recurrence of IgA deposits in the transplanted kidney, progression to recurrent ESRD with the grafted kidney is rare. Utilizing anti-thymocyte globulin and maintaining immunosuppression with corticosteroids can mitigate the recurrence of IgAN. In addition, treatment with ACE inhibitors or ARBs may help delay the progression of recurrent disease in allografts.
- #60 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The tonsils are a significant store for MALT, and IgA levels are known to decrease post-tonsillectomy. In addition, a correlation exists between tonsillar dendritic cell concentration and crescent formation in IgAN patients. Multiple retrospective studies have demonstrated the benefits of tonsillectomy in inducing remission or slowing IgAN progression, particularly in Asian populations, notably Japan. Tonsillectomy in conjunction with pulse steroid therapy has also been more recently studied and found to be effective in progressive IgAN. […] For individuals who progress to ESRD from IgAN, renal transplantation is a viable option. However, the risk of IgAN recurrence in the transplanted kidney persists. A significant study reported a recurrence rate of 23% in transplanted kidneys at 15 years, correlating with a 3.7-fold increase in graft rejection. Despite the recurrence of IgA deposits in the transplanted kidney, progression to recurrent ESRD with the grafted kidney is rare. Utilizing anti-thymocyte globulin and maintaining immunosuppression with corticosteroids can mitigate the recurrence of IgAN. In addition, treatment with ACE inhibitors or ARBs may help delay the progression of recurrent disease in allografts.
- #61 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
Because the clinical presentation and prognosis in IgA nephropathy varies widely, treatment must be individualized. All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Control of proteinuria is prudent, since there is a mostly linear association between the severity of proteinuria and decline in estimated glomerular filtration rate (GFR). Use of immunosuppression should be determined by considering the rate of progression, comorbidities, and whether alarming features are present on biopsy. However, immunosuppression has become controversial. Currently, corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. Sodiumglucose transporter 2 (SGLT2) inhibitors, which have an established role in chronic kidney disease generally, have also demonstrated benefit in IgA nephropathy.
- #62 IgA Nephropathy (Berger’s Disease) | Doctorhttps://patient.info/doctor/iga-nephropathy-bergers-disease-pro
The National Institute for Health and Care Excellence (NICE) has recommended targeted-release budesonide as an option for treating primary IgAN when there is a risk of rapid disease progression in adults with a urine protein-to-creatinine ratio of 1.5 g/g or more. It can only be used as an adjunct to optimised standard care including the highest tolerated licensed dose of angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARBs), unless these are contraindicated. […] There is evidence that tonsillectomy slows down the progression of IgA nephropathy. However, it is not currently recommended in guidelines, unless otherwise indicated due to recurrent infection.
- #63 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The tonsils are a significant store for MALT, and IgA levels are known to decrease post-tonsillectomy. In addition, a correlation exists between tonsillar dendritic cell concentration and crescent formation in IgAN patients. Multiple retrospective studies have demonstrated the benefits of tonsillectomy in inducing remission or slowing IgAN progression, particularly in Asian populations, notably Japan. Tonsillectomy in conjunction with pulse steroid therapy has also been more recently studied and found to be effective in progressive IgAN. […] For individuals who progress to ESRD from IgAN, renal transplantation is a viable option. However, the risk of IgAN recurrence in the transplanted kidney persists. A significant study reported a recurrence rate of 23% in transplanted kidneys at 15 years, correlating with a 3.7-fold increase in graft rejection. Despite the recurrence of IgA deposits in the transplanted kidney, progression to recurrent ESRD with the grafted kidney is rare. Utilizing anti-thymocyte globulin and maintaining immunosuppression with corticosteroids can mitigate the recurrence of IgAN. In addition, treatment with ACE inhibitors or ARBs may help delay the progression of recurrent disease in allografts.
- #64 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
With the right treatment, many people can keep their kidneys as healthy as possible and slow the diseases progression. […] If IgA nephropathy progresses to kidney failure, you may need to consider dialysis or a kidney transplant. Your care team will discuss your treatment options with you. […] IgA nephropathy is typically a lifelong, progressive kidney disease. That means it usually gets worse as time goes on. But medications can help slow the conditions progression. […] With proper treatment, many people with IgA nephropathy can live long, fulfilling lives.
- #65 IgA Nephropathy Treatment: Medication, Surgery, & Morehttps://www.health.com/iga-nephropathy-treatment-8702877
If Berger’s disease worsens and causes kidney failure, there are a few additional treatment options that your provider can use to help reduce symptoms. These include: Dialysis: This procedure uses a filtering machine to manually remove toxins and extra fluid from the blood when the kidneys can no longer effectively perform that function. […] Kidney transplant: A donated healthy kidney replaces a diseased kidney during this surgical procedure. […] There are also a few treatment options your healthcare provider may recommend that fall under the complementary and alternative medicine (CAM) category. […] Certain studies have shown that taking fish oil as a supplementary treatment for IgA nephropathy may offer some benefits. […] Some research has indicated that getting a tonsillectomy even if you don’t have currently inflamed or infected tonsils may be beneficial for people with progressive Berger’s disease, particularly when used in combination with corticosteroid treatment.
- #66 IgA nephropathy (IgAN) | American Kidney Fundhttps://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/iga-nephropathy
There are 2 new FDA-approved treatments for IgA nephropathy. Both lower the level of protein in urine (proteinuria), and work well in adults who have a high chance of their IgA nephropathy getting worse. […] If you have kidney failure from IgA nephropathy, you will need to go on dialysis or have a kidney transplant. A transplant is not a cure for any type of kidney disease, but is considered a treatment. […] Talk to your healthcare providers early on and often about treatment options, clinical trials, and any new symptoms to help prevent future damage. […] If you are close to kidney failure, it’s important to have open conversations with your healthcare team about treatment options and what is best for you. Treatments available for kidney failure include dialysis (hemodialysis or peritoneal dialysis) and a kidney transplant.
- #67 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The FDA has approved targeted-release budesonide (Tarpeyo) for proteinuria reduction in adults with primary IgA nephropathy who are at risk for rapid disease progression; the FDA issued accelerated approval for this indication in 2021 and converted it to full approval in December 2023. […] The Dapagliflozin and Prevention of Adverse outcomes in Chronic Kidney Disease (DAPA-CKD) and the Study of Heart and Kidney Protection With Empagliflozin trials confirmed that the SGLT2 inhibitors dapagliflozin and empagliflozin have nephroprotective effects in nondiabetic patients with albuminuric chronic kidney disease, and that the benefit extended to patients with eGFR 30 mL/min/1.73 m2. […] Current evidence does not support the use of fish oil as monotherapy, but some physicians combine fish oil with other therapies. KDIGO guidelines suggest that fish oil may be given to patients who have persistent proteinuria 1 g/d despite 36 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D). […] Tonsillectomy is a controversial treatment for IgA nephropathy. […] Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%).
- #68 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
All patients should be given supportive therapy to control hypertension and proteinuria, including renin-angiotensin system blockade and dietary sodium restriction. […] Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. […] Immunosuppression has become controversial. Corticosteroids are the option with the most convincing evidence to support their use, but if used, they should be administered only to carefully selected patients, and preferably should not be given for more than 6 months. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. […] The US Food and Drug Administration (FDA) has approved the following agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo), Sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist, Atrasentan, an endothelin type A receptor antagonist, Iptacopan (Fabhalta), a complement inhibitor. […] Kidney transplantation is effective in patients with IgA nephropathy that has progressed to end-stage kidney disease. However, the disorder frequently recurs after transplantation.
- #69 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The tonsils are a significant store for MALT, and IgA levels are known to decrease post-tonsillectomy. In addition, a correlation exists between tonsillar dendritic cell concentration and crescent formation in IgAN patients. Multiple retrospective studies have demonstrated the benefits of tonsillectomy in inducing remission or slowing IgAN progression, particularly in Asian populations, notably Japan. Tonsillectomy in conjunction with pulse steroid therapy has also been more recently studied and found to be effective in progressive IgAN. […] For individuals who progress to ESRD from IgAN, renal transplantation is a viable option. However, the risk of IgAN recurrence in the transplanted kidney persists. A significant study reported a recurrence rate of 23% in transplanted kidneys at 15 years, correlating with a 3.7-fold increase in graft rejection. Despite the recurrence of IgA deposits in the transplanted kidney, progression to recurrent ESRD with the grafted kidney is rare. Utilizing anti-thymocyte globulin and maintaining immunosuppression with corticosteroids can mitigate the recurrence of IgAN. In addition, treatment with ACE inhibitors or ARBs may help delay the progression of recurrent disease in allografts.
- #70 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The tonsils are a significant store for MALT, and IgA levels are known to decrease post-tonsillectomy. In addition, a correlation exists between tonsillar dendritic cell concentration and crescent formation in IgAN patients. Multiple retrospective studies have demonstrated the benefits of tonsillectomy in inducing remission or slowing IgAN progression, particularly in Asian populations, notably Japan. Tonsillectomy in conjunction with pulse steroid therapy has also been more recently studied and found to be effective in progressive IgAN. […] For individuals who progress to ESRD from IgAN, renal transplantation is a viable option. However, the risk of IgAN recurrence in the transplanted kidney persists. A significant study reported a recurrence rate of 23% in transplanted kidneys at 15 years, correlating with a 3.7-fold increase in graft rejection. Despite the recurrence of IgA deposits in the transplanted kidney, progression to recurrent ESRD with the grafted kidney is rare. Utilizing anti-thymocyte globulin and maintaining immunosuppression with corticosteroids can mitigate the recurrence of IgAN. In addition, treatment with ACE inhibitors or ARBs may help delay the progression of recurrent disease in allografts.
- #71 IgA Nephropathy (Berger’s Disease) Treatment Optionshttps://www.healthline.com/health/treatment-options-for-iga-nephropathy
A healthcare professional may recommend a variety of lifestyle changes to help you manage IgA nephropathy. […] These dietary modifications may include: Limiting the amount of sodium in your diet, following a diet that is low in saturated fats and cholesterol, shifting toward a plant-based diet. […] A healthcare professional may also advise you to eat protein in moderation. […] Fish oil supplements that contain omega-3 fatty acids may help slow kidney damage by lowering blood pressure. […] High blood pressure and heart or cardiovascular problems may be treated with medication. […] Kidney failure may require dialysis or a kidney transplant. […] Participating in a clinical trial may be a treatment option for some people with IgA nephropathy. […] Treatment options include non-immunosuppressive and immunosuppressive medications and cholesterol-lowering medications. Your doctor might also recommend lifestyle and dietary changes to manage IgA nephropathy. […] Further treatments, such as dialysis and kidney transplant, may be required for complications from IgA nephropathy.
- #72 IgA Nephropathy: Stages, Symptoms, Diagnosis, Treatmenthttps://www.health.com/iga-nephropathy-stages-8702883
In these early stages, your provider will want to focus on avoiding complications from IgA nephropathy, such as high blood pressure or diabetes, and may suggest the following: Maintaining healthy blood pressure and blood sugar levels, Taking medications (like statins) to help protect against heart disease and further kidney damage, Considering the pros and cons of medications that target the immune system directly (like corticosteroids) before too much kidney damage has occurred, Recommended lifestyle changes, like eating a kidney-friendly diet and avoiding smoking. […] They can generally prevent the disease from worsening by prescribing medications such as: Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs): Control high blood pressure and swelling and reduce protein levels in the urine, Corticosteroids: Suppress an overactive immune system and reduce inflammation, Statins: Lower cholesterol levels and prevent heart disease from developing.
- #73 IgA Nephropathy (Berger’s Disease) Treatment Optionshttps://www.healthline.com/health/treatment-options-for-iga-nephropathy
A healthcare professional may recommend a variety of lifestyle changes to help you manage IgA nephropathy. […] These dietary modifications may include: Limiting the amount of sodium in your diet, following a diet that is low in saturated fats and cholesterol, shifting toward a plant-based diet. […] A healthcare professional may also advise you to eat protein in moderation. […] Fish oil supplements that contain omega-3 fatty acids may help slow kidney damage by lowering blood pressure. […] High blood pressure and heart or cardiovascular problems may be treated with medication. […] Kidney failure may require dialysis or a kidney transplant. […] Participating in a clinical trial may be a treatment option for some people with IgA nephropathy. […] Treatment options include non-immunosuppressive and immunosuppressive medications and cholesterol-lowering medications. Your doctor might also recommend lifestyle and dietary changes to manage IgA nephropathy. […] Further treatments, such as dialysis and kidney transplant, may be required for complications from IgA nephropathy.
- #74 IgA Nephropathy: Stages, Symptoms, Diagnosis, Treatmenthttps://www.health.com/iga-nephropathy-stages-8702883
Because this is the last stage before kidney failure, treatment from a nephrologist (a doctor who specializes in treating kidney disease) is necessary to slow the damage and assess the potential for kidney failure. […] While these treatment options can help add years and quality of life to your prognosis, note that neither of these procedures is a cure for IgA nephropathy. […] Follow your treatment plan closely and discuss it with your healthcare provider if you have any questions or concerns. Research shows that prescribed medications for IgA nephropathy help control blood pressure and reduce the amount of protein in urine, ultimately slowing or preventing damage to the kidneys. […] Your healthcare provider may also recommend lifestyle changes to support this goal, including: Eating a low-sodium diet to reduce inflammation and manage high blood pressure or cholesterol levels, Monitoring your fluid intake to reduce overall inflammation in the body, Exercising regularly to maintain an ideal weight for your body, Quitting or avoiding smoking to help slow kidney damage and lower kidney failure risk. […] Treatment plans that include prescription medications, lifestyle changes, and potential surgical procedures can help slow or prevent the progression of IgA nephropathy through the stages and improve your quality of life.
- #75 IgA nephropathy (Berger disease) – Hancock Healthhttps://www.hancockhealth.org/mayo-health-library/iga-nephropathy-berger-disease/
Omega-3 fatty acids. These healthy fats might curb inflammation in the glomeruli without harmful side effects. […] Cholesterol medicine. If you have high cholesterol, this type of medicine can help control it and slow down kidney damage. […] Diuretics. These can help control swelling in the hands and feet called edema. […] The main goal of treatment is to keep you from needing dialysis or a kidney transplant. But either of those treatments can be lifesaving if your kidneys stop working well enough on their own. […] To help keep your kidneys healthier: […] Take steps to lower your blood pressure. This can help slow kidney damage from IgA nephropathy. […] Eat less protein. Ask your doctor if you should cut back on protein. This may help slow IgA nephropathy from becoming worse and protect your kidneys.
- #76 IgA Nephropathy: Stages, Symptoms, Diagnosis, Treatmenthttps://www.health.com/iga-nephropathy-stages-8702883
Because this is the last stage before kidney failure, treatment from a nephrologist (a doctor who specializes in treating kidney disease) is necessary to slow the damage and assess the potential for kidney failure. […] While these treatment options can help add years and quality of life to your prognosis, note that neither of these procedures is a cure for IgA nephropathy. […] Follow your treatment plan closely and discuss it with your healthcare provider if you have any questions or concerns. Research shows that prescribed medications for IgA nephropathy help control blood pressure and reduce the amount of protein in urine, ultimately slowing or preventing damage to the kidneys. […] Your healthcare provider may also recommend lifestyle changes to support this goal, including: Eating a low-sodium diet to reduce inflammation and manage high blood pressure or cholesterol levels, Monitoring your fluid intake to reduce overall inflammation in the body, Exercising regularly to maintain an ideal weight for your body, Quitting or avoiding smoking to help slow kidney damage and lower kidney failure risk. […] Treatment plans that include prescription medications, lifestyle changes, and potential surgical procedures can help slow or prevent the progression of IgA nephropathy through the stages and improve your quality of life.
- #77 IgA Nephropathy Treatment & Management: Approach Considerations, Medical Care, Diethttps://emedicine.medscape.com/article/239927-treatment
The US Food and Drug Administration (FDA) has approved several agents to reduce proteinuria in adults with IgA nephropathy who are at risk of rapid disease progression: a targeted-release formulation of the corticosteroid budesonide (Tarpeyo); sparsentan (Filspari), a dual angiotensin II and endothelin-1 receptor antagonist; atrasentan (Vanrafia), an endothelin-1 receptor antagonist; and iptacopan (Fabhalta), a complement inhibitor. […] Cyclophosphamide should be reserved for rapidly progressive crescentic glomerulonephritis. Most nephrologists agree with not using immunosuppression when the GFR is less than 30 mL/min/1.73 m2, although others would suggest a different GFR threshold. Regardless, the lower the GFR, the higher the risk of adverse events and the lower the likelihood of benefiting from immunosuppressants. Immunosuppression should definitely be avoided when the biopsy shows large amounts of interstitial fibrosis and tubular atrophy.
- #78 Furthering Managed Care Through Advances in IgA Nephropathy Therapyhttps://www.managedhealthcareexecutive.com/view/furthering-managed-care-through-advances-in-iga-nephropathy-therapy
In December 2021 and February 2023, respectively, the FDA granted accelerated approval to budesonide (Tarpeyo; Calliditas Therapeutics) and sparsentan (Filspari; Travere Therapeutics) for reduction of proteinuria in adults with IgAN to reduce disease progression. […] In December 2023, the FDA granted full approval to budesonide to reduce loss of kidney function in all patients with IgAN who are at risk for disease progression; this marked the first drug approval for IgAN. […] In August 2024, the FDA granted accelerated approval to iptacopan (Fabhalta; Novartis) for reduction of proteinuria in adults with primary IgAN at risk of rapid disease progression. […] In September 2024, the FDA granted full approval to sparsentan to slow kidney function decline in adults with primary IgAN who are at risk of disease progression.
- #79 Furthering Managed Care Through Advances in IgA Nephropathy Therapyhttps://www.managedhealthcareexecutive.com/view/furthering-managed-care-through-advances-in-iga-nephropathy-therapy
As of September 2024, phase 3 trials investigating the safety and efficacy of at least 11 pharmaceutical agents to treat IgAN are ongoing or have been completed. […] The KDIGO 2021 guidelines for IgAN include recommendations for diagnosis, prognosis and treatment. […] KDIGO emphasizes that kidney biopsy provides the only conclusive evidence to support a diagnosis of IgAN, as no validated diagnostic serum or urine biomarkers exist for the disease apart from estimated glomerular filtration rate (eGFR) and proteinuria. […] With no FDA-approved treatments at the time of its writing, the 2021 KDIGO guidelines underscore strategies not based upon immunosuppression. […] Aiming to slow disease progression, this supportive care is centered upon lifestyle modification (e.g., exercise, smoking cessation, weight loss, reduction of sodium in the diet), blood pressure control and renin-angiotensin system (RAS) inhibition.
- #80 Furthering Managed Care Through Advances in IgA Nephropathy Therapyhttps://www.managedhealthcareexecutive.com/view/furthering-managed-care-through-advances-in-iga-nephropathy-therapy
For patients who remain at high risk of progressive CKD (proteinuria 0.75-1 g/day) despite at least 90 days of optimized supportive care, the work group recommends offering participation in a clinical trial and consideration of immunosuppressive therapy, including a six-month course of glucocorticoids. […] However, glucocorticoids should be given with caution or, in certain situations (e.g., diabetes, obesity, severe osteoporosis), avoided entirely, as they do not impart a proven clinical benefit in IgAN. […] As of September 2024, phase 3 trials investigating the safety and efficacy of at least 11 pharmaceutical agents to treat IgAN are ongoing or have been completed. […] Investigated strategies include use of a targeted-release steroid, anticomplement therapies, modulators of lymphocyte activity and anti-endothelin or anti-aldosterone antagonists.
- #81 IgA Nephropathy Treatment Market – How Therapeutics Space is Evolving?https://www.delveinsight.com/blog/iga-nephropathy-treatment-market
The future of the IgA Nephropathy market is optimistic with emerging IgAN therapies such as Nefecon (Calliditas Therapeutics), Sparsentan (Travere Therapeutics), Iptacopan (Novartis Pharmaceuticals), Narsoplimab (Omeros), Atrasentan (Chinook Therapeutics Inc.), among others. […] Among the emerging IgA Nephropathy therapies, the most promising ones include Narsoplimab- a Phase III Monoclonal antibody, Iptacopan a Phase III Small molecule, Nefecon a Phase III Glucocorticoid receptor agonist, among others.
- #82 IgA Nephropathy: Current Treatment and New Insightshttps://www.mdpi.com/2073-4468/12/2/40
Several immunosuppressive treatment regimens have been studied but documented no evidence of efficacy in IgAN. […] There are few data to support the efficacy of mycophenolate mofetil (MMF) as a first-line treatment for IgAN in the past. […] The promising felzartamab, a fully human IgG1 monoclonal antibody designed to deplete CD38+ plasma cells, is in a phase II clinical trial for patients with IgAN and an increased risk of disease progression. […] All the investigation agents are summarized in Table 2.
- #83https://journals.lww.com/jasn/fulltext/2024/01000/treatment_of_iga_nephropathy__a_rapidly_evolving.12.aspx
Therapies with direct effect on disease pathogenesis are increasingly becoming available. While targeted-release budesonide has garnered the most attention, anti-B-cell strategies and selective complement inhibition will most likely prove their added value. […] The Study of the Effect and Safety of Sparsentan in the Treatment of Patients With IgA Nephropathy trial randomized 404 IgA nephropathy patients with persistent proteinuria (1 g/d) despite full-dose RAASi to either sparsentan or irbesartan. Interim analysis after the 9-month follow-up showed that sparsentan produced a meaningful reduction in proteinuria (between-group relative reduction of 41%). […] Systemic glucocorticoids have potent effects across the spectrum of immune function such that suppression of Gd-IgA1 production and subsequent immune complex formation and glomerular inflammation could be expected.
- #84 Furthering Managed Care Through Advances in IgA Nephropathy Therapyhttps://www.managedhealthcareexecutive.com/view/furthering-managed-care-through-advances-in-iga-nephropathy-therapy
The landscape of IgAN treatment is rapidly evolving. As of September 2024, there are ongoing or recently completed phase 3 trials to investigate the safety and efficacy of at least 11 pharmaceutical agents to manage IgAN. […] The primary aim of IgAN treatment is to prevent or delay progression to ESRD and preserve kidney function. In its 2021 clinical practice guideline for the management of glomerular diseases, Kidney Disease: Improving Global Outcomes (KDIGO) issued guidance for the management of IgAN absent any FDA-approved treatments. […] Recent advancements in understanding the pathogenesis of IgAN as an immune-mediated disease and the acceptance of surrogate outcomes for accelerated drug approval have led to a rapid increase in clinical trials exploring new therapeutic strategies that target specific disease pathways.
- #85 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
With the right treatment, many people can keep their kidneys as healthy as possible and slow the diseases progression. […] If IgA nephropathy progresses to kidney failure, you may need to consider dialysis or a kidney transplant. Your care team will discuss your treatment options with you. […] IgA nephropathy is typically a lifelong, progressive kidney disease. That means it usually gets worse as time goes on. But medications can help slow the conditions progression. […] With proper treatment, many people with IgA nephropathy can live long, fulfilling lives.
- #86 IgA Nephropathy Treatment: Medication, Surgery, & Morehttps://www.health.com/iga-nephropathy-treatment-8702877
Recent findings suggest that probiotics might be helpful for improving IgA nephropathy symptoms and quality of life, reducing inflammation, and delaying disease progression. […] Although there isn’t a cure for IgA nephropathy yet, available treatment options can help prevent or delay damage to your kidneys, slowing the progression of the condition. […] In many cases, IgA nephropathy does not worsen over time. Instead, it improves or stays stable with treatment. […] It’s also considered rare for Berger’s disease to progress quickly. Around 1 in 5 people with IgA nephropathy will develop kidney failure within 10 years of being diagnosed. […] Many people who have Berger’s disease can live a healthy life.