Nefropatia iga (choroba bergera)
Etiologia i przyczyny
Nefropatia IgA (choroba Bergera) jest jedną z najczęstszych przyczyn kłębuszkowego zapalenia nerek i przewlekłej niewydolności nerek, prowadzącą u 20-50% pacjentów do dializoterapii lub transplantacji. Patogeneza opiera się na odkładaniu w mezangium kłębuszków nerkowych kompleksów immunologicznych zawierających nieprawidłowo glikozylowaną IgA1 (z deficytem galaktozy w regionie zawiasowym O-glikanów), co wywołuje aktywację komórek mezangialnych, proliferację, włóknienie śródmiąższowe oraz uszkodzenie filtracyjne. Etiologia jest poligeniczna i wieloczynnikowa, z udziałem genów takich jak C1GALT1, C1GALT1C1, IGAN1, IGAN2, SPRY2 oraz regionów HLA-DRB1 i HLA-DQB1, a także czynników środowiskowych, w tym infekcji górnych dróg oddechowych, które wyzwalają nieprawidłową odpowiedź immunologiczną. Dziedziczność szacowana jest na 40-50%, a choroba ma charakter autoimmunologiczny, z produkcją autoprzeciwciał przeciwko Gd-IgA1. Wtórna nefropatia IgA może być związana z chorobami wątroby (marskość, WZW B i C), chorobami żołądkowo-jelitowymi (np. celiakia), schorzeniami autoimmunologicznymi, zakażeniami (w tym HIV) oraz lekami (np. inhibitory TNF-α).
Wprowadzenie do nefropatii IgA
Nefropatia IgA, znana również jako choroba Bergera, jest jedną z najczęstszych przyczyn kłębuszkowego zapalenia nerek i niewydolności nerek. Jest to powszechna forma glomerulonefrytu charakteryzująca się odkładaniem immunoglobuliny A (IgA) w błonie podstawnej kłębuszków nerkowych. W wyniku tego procesu dochodzi do nieprawidłowych reakcji immunologicznych, które prowadzą do złogów IgA w kłębuszkach, zwiększonej przepuszczalności podocytów i włóknienia śródmiąższowego.12
Choroba ta jest spowodowana gromadzeniem się białka zwalczającego zarazki, zwanego immunoglobuliną A (IgA), w nerkach. Prowadzi to do stanu zapalnego, który z czasem może upośledzić zdolność nerek do filtrowania odpadów z krwi.34
Nefropatia IgA jest trzecią najczęstszą przyczyną chorób nerek po tych wywołanych przez cukrzycę i nadciśnienie, a u 20-50% osób z nefropatią IgA ostatecznie rozwinie się niewydolność nerek wymagająca dializy lub przeszczepu nerki.56
Etiologia wieloczynnikowa nefropatii IgA
Dokładna przyczyna nefropatii IgA pozostaje nie w pełni poznana, ale badania wskazują, że choroba ma złożoną etiologię, obejmującą czynniki genetyczne, środowiskowe i immunologiczne.78
Uważa się, że rozwój nefropatii IgA następuje w wyniku kombinacji czynników środowiskowych i genetycznej predyspozycji. Chociaż nefropatia IgA często poprzedzona jest chorobą zakaźną, która wywołuje nieprawidłową odpowiedź immunologiczną, warto zauważyć, że sama nefropatia IgA nie ma etiologii zakaźnej. Nie ma dowodów sugerujących, że konkretne czynniki zakaźne powodują nefropatię IgA. Zamiast tego układ odpornościowy wydaje się być aktywowany przez różne kliniczne i subkliniczne czynniki wyzwalające, wraz z czynnikami genetycznymi związanymi z glikozylacją IgA.910
Podłoże autoimmunologiczne
Eksperci medyczni uważają, że nefropatia IgA jest chorobą autoimmunologiczną, co oznacza, że układ odpornościowy atakuje nerki. U osób z nefropatią IgA występuje wyższy poziom nieprawidłowego typu IgA – przeciwciała, któremu brakuje specjalnej cząsteczki cukru (galaktozy). Inne przeciwciała atakują następnie tę IgA i tworzą skupiska, które utykają w systemie filtracyjnym nerek i powodują jego uszkodzenie.1112
Nefropatia IgA jest autoimmunologiczną chorobą nerek wynikającą z konsekwencji zwiększonego poziomu krążącej IgA1 z deficytem galaktozy w regionie zawiasowym O-glikanów. Aby doszło do uszkodzenia nerek, koniecznych jest kilka dodatkowych procesów, w tym synteza krążących przeciwciał skierowanych przeciwko nieprawidłowo glikozylowanym O-glikanom regionu zawiasowego w celu utworzenia kompleksów immunologicznych, akumulacja kompleksów w mezangium i aktywacja komórek mezangialnych. Na ekspresję tych procesów wpływają prawdopodobnie czynniki genetyczne.13
Czynniki genetyczne
Nefropatia IgA ma komponent genetyczny, co oznacza, że może występować rodzinnie. Mniej niż 10% przypadków jest spowodowanych rodzinną nefropatią IgA, podczas gdy ponad 90% przypadków ma charakter sporadyczny. Zidentyfikowano co najmniej 18 różnych segmentów genów związanych z nefropatią IgA. Niektóre z tych genów obejmują C1GALT1 (enzym transferaza galaktozylowa) i C1GALT1C1 (molekularny chaperon).1415
Badacze odkryli pewne markery genetyczne, co oznacza, że może istnieć związek między wariantem genetycznym a nefropatią IgA. Zwiększona częstość występowania określonych grup HLA (HLA-DRB1 i HLA-DQB1) została zgłoszona w niektórych populacjach. Badanie genetycznego ryzyka nefropatii IgA w populacji chińskiej Han zidentyfikowało trzy regiony genów nie-HLA (FBXL21, CCR6 i STAT3) i jeden region genu HLA (GABBR1) o sugestywnym znaczeniu; dalsza analiza zidentyfikowała pięć nowych genów podatności, TGFBI, CCR6, STAT3, GABBR1 i CFB, które mogą być zaangażowane w nefropatię IgA.1617
Zidentyfikowano co najmniej 30 specyficznych lokalizacji genów, które mogą wpływać na ryzyko rozwoju nefropatii IgA u danej osoby. Naukowcy uważają, że nefropatia IgA ma charakter poligeniczny, co oznacza, że do wywołania choroby potrzebne są mutacje w kilku genach. Szacują jednak, że geny te odpowiadają jedynie za około 11% ryzyka nefropatii IgA.18
Geny specyficznie związane z rodzinną nefropatią IgA obejmują: IGAN1 na chromosomie 6, IGAN2 na chromosomie 2, SPRY2 na chromosomie 13. Geny związane z rodzinną nefropatią IgA wydają się podążać za wzorcem dziedziczenia autosomalnie dominującego. Oznacza to, że jeśli rodzic ma mutację genetyczną, istnieje 50% szans na przekazanie jej dziecku. Badania szacują dziedziczność nefropatii IgA na poziomie między 40% a 50%.19
Występuje również zwiększone ryzyko nefropatii IgA w związku z niską liczbą kopii genu defensyny (DEFA1A3). Niska całkowita liczba kopii wykazała również istotny związek z dysfunkcją nerek u pacjentów z nefropatią IgA.20
Czynniki środowiskowe
Głównym środowiskowym czynnikiem wyzwalającym nefropatię IgA jest niedawna infekcja górnych dróg oddechowych (URI), przeziębienie lub ból gardła. W wielu przypadkach wydaje się to prowokować rozwój nefropatii IgA. Objawy kliniczne nefropatii IgA, w tym krwiomocz, są silnie związane z poprzedzającymi infekcjami wirusowymi lub bakteryjnymi, najczęściej infekcją górnych dróg oddechowych.2122
U większości osób proces chorobowy zazwyczaj zaczyna się od przeziębienia, bólu gardła lub infekcji dróg oddechowych. Gdy układ odpornościowy zwalcza infekcję, niektóre osoby wytwarzają bardzo wysoki poziom przeciwciał IgA. Kiedy w krwi jest zbyt wiele tych przeciwciał, mogą one utknąć w kłębuszkach (małych filtrach w nerkach). Gdy się tam zaklinują, powodują stan zapalny, bliznowacenie i uszkodzenie nerek.23
Stres, inny powszechnie rozumiany czynnik ryzyka chorób nerek, może również przyczyniać się do rozwoju nefropatii IgA.24
Wtórne przyczyny nefropatii IgA
Nefropatia IgA może być wtórna do innych chorób. Wtórna nefropatia IgA może wynikać z różnych stanów, w tym przyczyn wątrobowych, żołądkowo-jelitowych, autoimmunologicznych, dermatologicznych, zakaźnych i związanych z lekami.25
Choroby wątroby
Choroba wątroby jest często związana z nefropatią IgA poprzez różne mechanizmy, co czyni ją najczęstszą przyczyną wtórnej nefropatii IgA. Obejmują one marskość wątroby i przewlekłe zakażenia wirusem zapalenia wątroby typu B i C.2627
Złogi kłębuszkowe IgA to częste znalezisko w marskości wątroby, występujące u ponad jednej trzeciej pacjentów. Chorobie wątroby towarzyszy upośledzenie usuwania kompleksów zawierających IgA przez komórki Kupffera, predysponując pacjentów do odkładania IgA w nerkach.28
Choroby żołądkowo-jelitowe
Znaczenie tkanki limfatycznej związanej z błoną śluzową (MALT) zyskało ostatnio znaczenie w zrozumieniu patologii różnych chorób, szczególnie nefropatii IgA, ponieważ produkcja IgA odbywa się głównie w tkance śluzowej. Zaburzenia żołądkowo-jelitowe obejmują różnorodne stany, z których każdy ma odrębne implikacje dla nefropatii IgA.29
Złogi kłębuszkowe IgA występują u maksymalnie jednej trzeciej pacjentów z enteropatią glutenową. Większość z tych pacjentów nie ma klinicznych objawów choroby. Jednak nefropatia IgA i nadwrażliwość na gluten są ze sobą powiązane, a wycofanie glutenu z diety tych pacjentów skutkowało kliniczną i immunologiczną poprawą choroby nerek.30
Choroba trzewna jest silnie skorelowana z nefropatią IgA i ma wspólne pochodzenie autoimmunologiczne. Choroba zapalna jelit stanowi czynnik ryzyka nefropatii IgA. Nieprawidłowa mikrobiota jelitowa, składająca się z komensalnych bakterii jelitowych, jest silnie związana z nefropatią IgA.3132
Choroby autoimmunologiczne
Zaburzenia autoimmunologiczne, w tym zespół Sjögrena, spondyloartropatia, toczeń rumieniowaty układowy i choroba Behçeta, zostały powiązane z rozwojem nefropatii IgA.3334
Nie jest to powszechne, aby osoby z nefropatią IgA miały rodzinną historię choroby nerek. Zamiast tego, są bardziej skłonne do posiadania rodzinnej historii chorób związanych z układem odpornościowym, takich jak łuszczyca lub astma.35
Infekcje
Nefropatia IgA została zgłoszona u pacjentów z zakażeniem HIV, zarówno u osób rasy białej, jak i czarnej, pomimo rzadkości typowej nefropatii IgA w populacji czarnej.36
Większość przypadków nefropatii IgA jest idiopatyczna, ale początek lub zaostrzenie choroby jest często poprzedzone infekcją dróg oddechowych. Zgłaszano związek z niektórymi bakteriami, takimi jak Haemophilus parainfluenzae.37
Zespół badawczy odkrył, że osoby z chorobą miały znacznie większą liczbę bakterii Neisseria na migdałkach. Sugeruje to dużą zmianę w naszym zrozumieniu źródła IgA i bodźca do jej produkcji w tej chorobie. Odkrycia sugerują, że niektóre mikroby w gardle, takie jak Neisseria, mogą nadmiernie stymulować odpowiedź immunologiczną i ostatecznie prowadzić do uszkodzenia nerek.38
Inne powiązane stany chorobowe
Zaburzenia dermatologiczne obejmują szereg stanów, z których niektóre są związane z nefropatią IgA.39
Nefropatia IgA indukowana lekami obejmuje diagnozowanie czasu uszkodzenia nerek w związku z podawaniem leków i wykluczenie innych potencjalnych przyczyn. Leki takie jak inhibitory czynnika martwicy nowotworów-α (TNF-α) były wiązane z nefropatią IgA ze względu na ich wpływ na odpowiedź immunologiczną organizmu.4041
Chociaż rzadko, pierwotne choroby płuc, takie jak sarkoidoza, idiopatyczne włóknienie płuc lub krwotok płucny, mogą być związane z nefropatią IgA. Dodatkowo, procesy onkologiczne, takie jak choroby mieloproliferacyjne, rak żołądka, nowotwory płuc, raki komórek nerkowych i inne nowotwory złośliwe, również mogą być związane z IgA.4243
Możliwe jest również, że szczepionki mRNA przeciwko COVID-19 mogą być związane ze zwiększoną częstością występowania nefropatii IgA.44
Mechanizmy patofizjologiczne
Nieprawidłowości glikozylacji IgA
Niedawno zaproponowana teoria koncentruje się na nieprawidłowościach cząsteczki IgA1. Podstawowa wada w nefropatii IgA nie jest nerkowa, ale systemowa. Innymi słowy, złogi mezangialne kompleksów IgA-glikan są związane z wyższym niż normalnie poziomem nieprawidłowo glikozylowanej IgA1. Stymuluje to proliferację komórek mezangialnych, ekspansję macierzy mezangialnej, z wydzielaniem chemikaliów, które ostatecznie prowadzą do stwardnienia kłębuszków nerkowych i odkładania się włóknistej tkanki śródmiąższowej.4546
Osoby z nefropatią IgA mają wyższy poziom IgA z mniejszą ilością galaktozy niż jest to typowe. Nefropatia IgA wynika z nieprawidłowej glikozylacji O-regionu zawiasowego IgA1, co promuje tworzenie krążących kompleksów immunologicznych IgA1-przeciwciało, prowadzących do aktywacji komórek mezangialnych i złogów.4748
Kluczową wadą molekularną u osób z nefropatią IgA jest nieprawidłowa O-glikozylacja przeciwciał IgA. Badania rodzin wykazały, że problemy w O-glikozylacji IgA są powszechne u osób z nefropatią IgA i mają w dużej mierze pochodzenie genetyczne, chociaż dokładne zaangażowane geny były nieznane. Badania potwierdziły, że warianty genów C1GALT1 i C1GALT1C1 były znacznie częstsze u osób z wysokim poziomem markera Gd-IgA1.49
Podwyższone poziomy IgA z niedoborem galaktozy są związane z pewnymi stanami autoimmunologicznymi, szczególnie nefropatią IgA. W tym schorzeniu układ odpornościowy produkuje nieprawidłową formę IgA, która ma niedobór galaktozy.50
Tworzenie kompleksów immunologicznych
Pierwotna nefropatia IgA charakteryzuje się złogami przeciwciał IgA w kłębuszkach. Uważa się, że rozpoczyna się od wadliwej glikozylacji podklasy IgA1 IgA, co prowadzi do nadprodukcji IgA1 z niedoborem galaktozy (Gd-IgA1), wystąpienia autoprzeciwciał anty-Gd-IgA1 i mezangialnych złogów nefrytotwórczych kompleksów immunologicznych.51
Nie ma jasnego znanego wyjaśnienia akumulacji IgA. Egzogenne antygeny dla IgA nie zostały zidentyfikowane w nerkach, ale możliwe, że ten antygen został usunięty, zanim choroba się ujawniła. Zaproponowano również, że sama IgA może być antygenem.52
Obecność kompleksów immunologicznych składających się z IgA z niedoborem galaktozy i/lub kompleksów IgA-IgG sprzyja ich wiązaniu z receptorami i złogom w kłębuszkach/mezangium. Złogi stymulują odpowiedź zapalną i aktywację układu dopełniacza, po których następuje podział komórek mezangialnych i nadprodukcja macierzy.53
Czynniki ryzyka nefropatii IgA
Istnieją różne czynniki ryzyka dla nefropatii IgA. Obejmują one:54
- Płeć: W Ameryce Północnej i Europie Zachodniej nefropatia IgA dotyka co najmniej dwa razy więcej mężczyzn niż kobiet5556
- Pochodzenie etniczne: Nefropatia IgA jest bardziej powszechna u osób rasy białej i pochodzenia azjatyckiego niż u osób rasy czarnej5758
- Wiek: Nefropatia IgA najczęściej rozwija się między późną nastoletniością a późnymi latami 30.5960
- Historia rodzinna: Nefropatia IgA wydaje się występować w niektórych rodzinach6162
- Osobista lub rodzinna historia nefropatii IgA lub plamicy Henocha-Schönleina, formy zapalenia naczyń, która wpływa na wiele części ciała6364
Podsumowanie
Nefropatia IgA (choroba Bergera) to złożona choroba nerek o wieloczynnikowej etiologii. Chociaż dokładna przyczyna pozostaje niepewna, badania wskazują, że kluczową rolę odgrywają zarówno czynniki genetyczne, jak i środowiskowe. Nieprawidłowości w glikozylacji IgA prowadzą do tworzenia kompleksów immunologicznych, które odkładają się w kłębuszkach nerkowych, powodując stan zapalny i uszkodzenie nerek.6566
Wtórna nefropatia IgA może wynikać z różnych stanów, w tym chorób wątroby, stanów żołądkowo-jelitowych (takich jak choroba trzewna), zaburzeń autoimmunologicznych i infekcji. Czynniki ryzyka obejmują płeć męską, pochodzenie azjatyckie lub europejskie oraz historię rodzinną.6768
Zrozumienie etiologii nefropatii IgA ma kluczowe znaczenie dla opracowania lepszych terapii tej choroby, która pozostaje znaczącą przyczyną niewydolności nerek na całym świecie.6970
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Materiały źródłowe
- #1 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Immunoglobulin A (IgA) nephropathy, or IgAN, also known as Berger disease, is one of the leading causes of glomerulonephritis and renal failure. This disease is a prevalent form of glomerulonephritis characterized by the deposition of IgA in the glomerular basement membrane. […] IgAN results from abnormal immune reactions, resulting in IgA deposits within the glomerulus, increased podocyte permeability, and interstitial fibrosis. […] Although IgAN is often preceded by an infectious disease, which triggers a dysregulated immune response, it is noteworthy that IgAN itself is not of an infectious etiology. No evidence suggests that specific infectious agents cause IgAN. Instead, the immune system appears to be activated by various clinical and subclinical triggers, along with genetic factors related to IgA glycosylation.
- #2 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/books/NBK538214/
Immunoglobulin A (IgA) nephropathy, or IgAN, also known as Berger disease, is one of the leading causes of glomerulonephritis and renal failure. This disease is a prevalent form of glomerulonephritis characterized by the deposition of IgA in the glomerular basement membrane. […] IgAN results from abnormal immune reactions, resulting in IgA deposits within the glomerulus, increased podocyte permeability, and interstitial fibrosis. […] Although IgAN is often preceded by an infectious disease, which triggers a dysregulated immune response, it is noteworthy that IgAN itself is not of an infectious etiology. No evidence suggests that specific infectious agents cause IgAN. Instead, the immune system appears to be activated by various clinical and subclinical triggers, along with genetic factors related to IgA glycosylation.
- #3 IgA nephropathy (Berger disease) | Altru Health Systemhttps://www.altru.org/health-library/conditions/iga-nephropathy-berger-disease
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: […] Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. […] Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. […] Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. […] Infections. These include HIV and some bacterial infections.
- #4 IgA nephropathy (Berger disease) | Health Library | Memorial Health Systemhttps://www.mhsystem.org/health-library/con-20166955/
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. This causes a type of swelling called inflammation that, over time, can make it harder for the kidneys to filter waste from the blood. […] Researchers dont know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. […] The exact cause of IgA nephropathy is unknown. But these factors might raise the risk of getting it: Sex. In North America and Western Europe, IgA nephropathy affects at least twice as many men as it does women. […] Immunoglobulin A (IgA) is a type of protein called an antibody. The immune system makes IgA to help attack germs and fight infections. But with IgA nephropathy, this protein collects in the glomeruli. This causes inflammation and affects their filtering ability over time.
- #5 What is IgA Nephropathy?https://ckd.responsumhealth.com/iga-nephropathy
What is IgA Nephropathy? […] IgA nephropathy is the third most common type of kidney disease. Learn about its causes, risk factors, and symptoms, as well as how its diagnosed and how its treated. […] Experts think that IgAN is an autoimmune disease, which is when cells that are designed to protect your body start attacking it instead. People who have IgAN have a higher level of Immunoglobulin A (IgA) in their blood, which contains less of a type of sugar, called galactose. […] There may also be a genetic component to the diseases development. […] IgAN is the third most common type of kidney disease, after those caused by diabetes and hypertension, and it can occur to people of all ages, sexes, and races. Scientists have identified certain factors, however, that can increase your risk. […] These risk factors include: Male sex, Late teen to early 30s, Family history of IgAN or IgA vasculitis, Asian or Caucasian.
- #6 IgA Nephropathy (Bergerâs Disease): Causes, Symptoms, & Treatmenthttps://www.webmd.com/a-to-z-guides/what-is-iga-nephropathy
If you have this digestive problem, your body cant process gluten, a protein found in most grains. […] HIV and some bacterial infections are linked to an increased risk of IgA nephropathy. […] Its not common for people with IgA nephropathy to have a family history of kidney disease. Instead, they are more likely to have a family history of diseases involving the immune system, such as psoriasis or asthma. […] IgA nephropathy is a disease that progresses slowly for most people. The goal of early detection and treatment is to keep your kidneys working well and avoid kidney failure. Research suggests that 20%-50% of people with IgA nephropathy will eventually develop kidney failure and need dialysis or a kidney transplant.
- #7 Causes and Risk Factors of IgA Nephropathyhttps://www.health.com/iga-nephropathy-causes-8702882
Immunoglobulin A nephropathy (also known as IgA nephropathy or Berger’s disease) is an autoimmune disease that affects the kidneys. It develops when too much of the protein IgA builds up in the kidneys, damaging the organ’s network that filters waste and fluids from the blood. […] It’s not entirely clear what causes IgA nephropathy to develop, but research suggests that genetics and environmental factors can influence the condition to develop. […] Researchers don’t know exactly why this happens, but they have proposed a few theories: genetics and environmental triggers. […] There appears to be a genetic component to having IgA nephropathy. Studies suggest that your chances of developing IgA nephropathy increase if you have a family history of the disease. […] Experts believe that a combination of genetic and environmental factors contribute to IgA nephropathy’s development.
- #8 IgA Nephropathy (Berger’s Disease): ERKNet for Patientshttps://www.erknet.org/patients/your-kidney-disease/iga-nephropathy/disease-information
IgA Nephropathy (IgAN), in past sometimes known as Bergers Disease, is an inflammatory disease of the kidney, the most common primary glomerulonephritis worldwide as well a leading cause of End-Stage Kidney Disease (ESKD). […] The exact cause of the disease has not been fully determined. Extensive research in recent years has found that several mechanisms underlie the disease. It is presumed that the development of the disease occurs due to a combination of environmental factors and the presence of genetic predisposition. […] IgAN can be secondary to other diseases, e.g. systemic diseases, including: Ankylosing Spondylitis(AS) Rheumatoid arthritis Reiters Syndrome Coeliac Disease Inflammatory Bowel Disease Alcoholic Liver Disease Sarcoidosis Psoriasis Hepatitis B or C. […] IgA vasculitis (IgAV), previously Schoenlein-Henoch purpura, is an inflammatory disease of small blood vessels associated with the deposition of IgA antibodies. Immune complexes can be observed in the vessels of many different organs, such as the skin, lungs, as well as the kidneys. Much scientific evidence points to a link between IgAV and IgAN. A common ethology of both diseases is very likely, although the prognosis is different, with better outcomes for IgAV.
- #9 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Immunoglobulin A (IgA) nephropathy, or IgAN, also known as Berger disease, is one of the leading causes of glomerulonephritis and renal failure. This disease is a prevalent form of glomerulonephritis characterized by the deposition of IgA in the glomerular basement membrane. […] IgAN results from abnormal immune reactions, resulting in IgA deposits within the glomerulus, increased podocyte permeability, and interstitial fibrosis. […] Although IgAN is often preceded by an infectious disease, which triggers a dysregulated immune response, it is noteworthy that IgAN itself is not of an infectious etiology. No evidence suggests that specific infectious agents cause IgAN. Instead, the immune system appears to be activated by various clinical and subclinical triggers, along with genetic factors related to IgA glycosylation.
- #10 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/books/NBK538214/
Immunoglobulin A (IgA) nephropathy, or IgAN, also known as Berger disease, is one of the leading causes of glomerulonephritis and renal failure. This disease is a prevalent form of glomerulonephritis characterized by the deposition of IgA in the glomerular basement membrane. […] IgAN results from abnormal immune reactions, resulting in IgA deposits within the glomerulus, increased podocyte permeability, and interstitial fibrosis. […] Although IgAN is often preceded by an infectious disease, which triggers a dysregulated immune response, it is noteworthy that IgAN itself is not of an infectious etiology. No evidence suggests that specific infectious agents cause IgAN. Instead, the immune system appears to be activated by various clinical and subclinical triggers, along with genetic factors related to IgA glycosylation.
- #11 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
IgA nephropathy, or Bergers disease, is a kidney disease in which IgA antibodies build up abnormally. Over time, it leads to kidney damage or kidney failure. […] Medical experts believe IgA nephropathy is an autoimmune disease. That means your immune system attacks your kidneys. […] People with IgA nephropathy have a higher level of IgA with less galactose than is typical. […] For some people, there’s a genetic family history of IgA nephropathy. Medical experts have discovered some genetic markers. That means there may be a link between a genetic variation and IgA nephropathy. […] Risk factors for IgA nephropathy include a genetic family history of IgA nephropathy. […] Although IgA can affect anyone, you may be more likely to have it if you’re male, in your late teens to late 30s, Asian or European.
- #12 IgA Nephropathy (Bergerâs Disease): Causes, Symptoms, & Treatmenthttps://www.webmd.com/a-to-z-guides/what-is-iga-nephropathy
Immunoglobulin A (IgA) nephropathy, also called Bergers disease, is an autoimmune disease that affects your kidneys. […] When you have IgA nephropathy, your blood has a higher level of an abnormal type of IgA, an antibody that lacks a special sugar molecule. Other antibodies then target this IgA and form clumps that get stuck in the filtration system of your kidneys and damage it. […] Scientists dont know what triggers IgA nephropathy. Its not contagious. Your genes play a role in whether you get it. The disease can run in families, so you may get it if one of your relatives has it or had it. But the disease also tends to have a trigger that sets it off. […] Certain conditions affecting the liver, such as cirrhosis, and chronic hepatitis B and C infections, may be linked to setting off IgA nephropathy.
- #13 IgA Nephropathy (Berger’s Disease) | Doctorhttps://patient.info/doctor/iga-nephropathy-bergers-disease-pro
Since its first description by Berger and Hinglais in 1968, IgA nephropathy (IgAN) has remained the most common form of idiopathic glomerulonephritis leading to chronic kidney disease (CKD) in developed countries. […] IgAN is an autoimmune renal disease arising from consequences of increased circulating levels of IgA1 with galactose-deficient hinge-region O-glycans. For this to cause kidney injury, several further processes are required, including synthesis of circulating antibodies directed against the aberrantly glycosylated O-linked hinge-region glycans to form immune complexes, accumulation of the complexes in the mesangium, and activation of mesangial cells. Genetic factors apparently influence the expression of these processes. […] A familial form of IgA nephropathy, inherited as an autosomal dominant condition, has also been described.
- #14 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The potential etiologies underlying IgAN include the factors mentioned below. […] Fewer than 10% of cases are due to familial IgAN. […] More than 90% of cases are sporadic. […] Many systemic conditions are associated with IgA deposits, causing IgAN pathology. […] Recent reports suggest the pathways leading to glomerular injury are similar between primary and secondary IgAN. […] The significance of mucosal-associated lymphoid tissue (MALT) has recently gained prominence in understanding the pathology of various diseases, especially IgAN, as IgA production predominantly occurs in mucosal tissue. […] Gastrointestinal disorders span a diverse range of conditions, each with distinct implications for IgAN. […] Liver disease is often associated with IgAN through various mechanisms, making it the most common cause of secondary IgAN.
- #15 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
IgAN results from abnormal immune reactions, resulting in IgA deposits within the glomerulus, increased podocyte permeability, and interstitial fibrosis. […] Although IgAN is often preceded by an infectious disease, which triggers a dysregulated immune response, it is noteworthy that IgAN itself is not of an infectious etiology. No evidence suggests that specific infectious agents cause IgAN. Instead, the immune system appears to be activated by various clinical and subclinical triggers, along with genetic factors related to IgA glycosylation. […] The potential etiologies underlying IgAN include the factors mentioned below. […] Fewer than 10% of cases are due to familial IgAN. […] At least 18 different gene segments associated with IgAN have been identified. […] Some of these genes include the C1GALT1 (a galactosyl transferase enzyme) and C1GALT1C1 (a molecular chaperone).
- #16 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
IgA nephropathy, or Bergers disease, is a kidney disease in which IgA antibodies build up abnormally. Over time, it leads to kidney damage or kidney failure. […] Medical experts believe IgA nephropathy is an autoimmune disease. That means your immune system attacks your kidneys. […] People with IgA nephropathy have a higher level of IgA with less galactose than is typical. […] For some people, there’s a genetic family history of IgA nephropathy. Medical experts have discovered some genetic markers. That means there may be a link between a genetic variation and IgA nephropathy. […] Risk factors for IgA nephropathy include a genetic family history of IgA nephropathy. […] Although IgA can affect anyone, you may be more likely to have it if you’re male, in your late teens to late 30s, Asian or European.
- #17 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
IgA nephropathy has been reported in patients with HIV infection, both Whites and Blacks, despite the rarity of typical IgA nephropathy in the Black population. […] Although IgA nephropathy is usually a sporadic disease, data suggest that genetic factors are important in susceptibility to development of mesangial glomerulonephritis. Several cases of familial disease have been reported in Italy and the United States, and an autosomal dominant form has been linked to band 6q22-23. […] Additionally, increased frequency of specific HLA groups (HLA-DRB1 and HLA-DQB1) has been reported in some populations. […] A study of genetic risk for IgA nephropathy in Han Chinese identified three non-HLA gene regions (FBXL21, CCR6, and STAT3) and one HLA gene region (GABBR1) with suggestive significance; further analysis identified five novel susceptibility genes, TGFBI, CCR6, STAT3, GABBR1, and CFB, that may be involved in IgA nephropathy.
- #18 Is IgA Nephropathy Genetic?https://www.healthline.com/health/kidney-disease/is-iga-nephropathy-genetic
Immunoglobulin A (IgA) nephropathy, aka Berger disease, is a rare autoimmune disease that affects your kidneys. Though rare, IgA nephropathy (IgAN) is one of the leading causes of kidney failure. […] More than 90% of IgA cases are sporadic, meaning they develop due to changes your body experiences during your lifetime. Scientists still arent sure of the exact causes of these cases. […] But the remaining cases (less than 10%) are familial, meaning youve inherited genetic changes (mutations) from your parents that increase your risk of developing IgAN. […] Genome studies have identified as many as 30 specific gene locations that may affect a persons risk of developing IgAN. […] Scientists think IgAN is polygenic in nature, meaning it takes mutations in several of these genes to cause the disease. Still, they estimate that these genes only account for about 11% of IgAN risk.
- #19 Is IgA Nephropathy Genetic?https://www.healthline.com/health/kidney-disease/is-iga-nephropathy-genetic
Genes linked specifically to familial IgAN include: IGAN1 on chromosome 6, IGAN2 on chromosome 2, SPRY2 on chromosome 13. […] Genes linked to familial IgAN appear to follow an autosomal dominant inheritance pattern. That means if a parent has the genetic mutation, they have a 50% chance of passing it on to their child. […] Studies estimate the heritability of IgAN to be between 40% and 50%. […] Due to its genetic component, race and ethnicity may play a role in IgAN risk. Studies suggest that people of Asian descent are more likely to develop IgAN than people of European or African descent.
- #20 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Ai and colleagues reported increased risk for IgA nephropathy in association with low copy number of the -defensin gene (DEFA1A3). Low total copy numbers also showed significant association with kidney dysfunction in patients with IgA nephropathy. […] Single-nucleotide polymorphisms (SNPs) of the enabled homolog gene (ENAH) have been associated with increased susceptibility to childhood IgA nephropathy, as well as to the development of proteinuria and gross hematuria, and pathological progression in children with the disease.
- #21 Causes and Risk Factors of IgA Nephropathyhttps://www.health.com/iga-nephropathy-causes-8702882
A primary environmental trigger for IgA nephropathy is a recent upper respiratory infection (URI), cold, or sore throat. In many cases, this appears to prompt the development of IgA nephropathy. […] Stress, another commonly understood risk factor for kidney disease, may also contribute to the development of IGA nephropathy. […] For some people, IgA nephropathy can be passed down in families. Research has uncovered certain genetic mutations (or changes) that likely play a role in the development of IgA nephropathy. […] However, it’s estimated that around 10% of cases or less are due to familial (hereditary) IgA nephropathy. This suggests that most cases may actually develop in people without a known family history of the condition. […] Like other autoimmune diseases, there isn’t much specific evidence around why this condition develops in the first place. A combination of genetics, environmental triggers, and circumstances like infections, stress, and lifestyle factors seem to influence the onset and severity of Berger’s disease.
- #22 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN. […] In addition, oncological processes, such as myeloproliferative disease, gastric carcinoma, lung cancers, renal cell carcinomas, and other malignancies, can also be associated with IgA.
- #23 IgA Nephropathy (IgAN) | National Kidney Foundationhttps://www.kidney.org/kidney-topics/iga-nephropathy
IgA nephropathy (IgAN) is a type of kidney disease where antibodies build up in your kidneys and cause damage to your glomeruli (small filters inside your kidneys). […] The exact causes of IgAN are not well understood. Leading research suggests it may be caused by a mix of your genes and environment. For most people, the disease process usually starts with a cold, sore throat, or respiratory infection. As your immune system fights the infection, some people make very high levels of IgA antibodies. When you have too many of these antibodies in your blood, they can get stuck in your glomeruli (small filters in your kidneys). When they get stuck, it causes inflammation, scarring, and kidney damage.
- #24 Causes and Risk Factors of IgA Nephropathyhttps://www.health.com/iga-nephropathy-causes-8702882
A primary environmental trigger for IgA nephropathy is a recent upper respiratory infection (URI), cold, or sore throat. In many cases, this appears to prompt the development of IgA nephropathy. […] Stress, another commonly understood risk factor for kidney disease, may also contribute to the development of IGA nephropathy. […] For some people, IgA nephropathy can be passed down in families. Research has uncovered certain genetic mutations (or changes) that likely play a role in the development of IgA nephropathy. […] However, it’s estimated that around 10% of cases or less are due to familial (hereditary) IgA nephropathy. This suggests that most cases may actually develop in people without a known family history of the condition. […] Like other autoimmune diseases, there isn’t much specific evidence around why this condition develops in the first place. A combination of genetics, environmental triggers, and circumstances like infections, stress, and lifestyle factors seem to influence the onset and severity of Berger’s disease.
- #25 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
More than 90% of cases are sporadic. […] No evidence suggests the involvement of a particular infectious agent despite the association between macroscopic hematuria and mucosal inflammation. […] No evidence indicates hypersensitivity to food antigens, except for a small group of patients with celiac disease. […] Abnormal O-glycosylation of the IgA1 hinge region promotes the formation of circulating IgA1-antibody immune complexes, leading to mesangial cell activation and deposition. […] Many systemic conditions are associated with IgA deposits, causing IgAN pathology. […] Recent reports suggest the pathways leading to glomerular injury are similar between primary and secondary IgAN. […] Secondary IgAN may arise from various conditions, including liver, gastrointestinal, autoimmune, dermatological, infectious, and drug-related causes.
- #26 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
The significance of mucosal-associated lymphoid tissue (MALT) has recently gained prominence in understanding the pathology of various diseases, especially IgAN, as IgA production predominantly occurs in mucosal tissue. […] Liver disease is often associated with IgAN through various mechanisms, making it the most common cause of secondary IgAN. […] Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN.
- #27 IgA nephropathy (Berger disease) | Altru Health Systemhttps://www.altru.org/health-library/conditions/iga-nephropathy-berger-disease
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: […] Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. […] Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. […] Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. […] Infections. These include HIV and some bacterial infections.
- #28 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Most cases of IgA nephropathy are idiopathic, but the onset or exacerbation of the disease is often preceded by a respiratory tract infection. Association with some bacteria, such as Haemophilus parainfluenzae, has been reported. A variety of other disorders have also been linked with IgA nephropathy, as discussed below. […] Glomerular IgA deposition is a common finding in cirrhosis, occurring in more than one-third of patients. Liver disease is accompanied by impaired removal of IgA-containing complexes by the Kupffer cells, predisposing patients to IgA deposition in the kidney. […] Glomerular IgA deposition occurs in up to a third of patients with gluten enteropathy. Most of these patients have no clinical manifestations of the disease. However, IgA nephropathy and gluten hypersensitivity are associated, and withdrawal of gluten from the diet of these patients has resulted in clinical and immunologic improvement of the renal disease.
- #29 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
The potential etiologies underlying IgAN include the factors mentioned below. […] Fewer than 10% of cases are due to familial IgAN. […] More than 90% of cases are sporadic. […] Many systemic conditions are associated with IgA deposits, causing IgAN pathology. […] Recent reports suggest the pathways leading to glomerular injury are similar between primary and secondary IgAN. […] The significance of mucosal-associated lymphoid tissue (MALT) has recently gained prominence in understanding the pathology of various diseases, especially IgAN, as IgA production predominantly occurs in mucosal tissue. […] Gastrointestinal disorders span a diverse range of conditions, each with distinct implications for IgAN. […] Liver disease is often associated with IgAN through various mechanisms, making it the most common cause of secondary IgAN.
- #30 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Most cases of IgA nephropathy are idiopathic, but the onset or exacerbation of the disease is often preceded by a respiratory tract infection. Association with some bacteria, such as Haemophilus parainfluenzae, has been reported. A variety of other disorders have also been linked with IgA nephropathy, as discussed below. […] Glomerular IgA deposition is a common finding in cirrhosis, occurring in more than one-third of patients. Liver disease is accompanied by impaired removal of IgA-containing complexes by the Kupffer cells, predisposing patients to IgA deposition in the kidney. […] Glomerular IgA deposition occurs in up to a third of patients with gluten enteropathy. Most of these patients have no clinical manifestations of the disease. However, IgA nephropathy and gluten hypersensitivity are associated, and withdrawal of gluten from the diet of these patients has resulted in clinical and immunologic improvement of the renal disease.
- #31 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN. […] Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN.
- #32 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
The significance of mucosal-associated lymphoid tissue (MALT) has recently gained prominence in understanding the pathology of various diseases, especially IgAN, as IgA production predominantly occurs in mucosal tissue. […] Liver disease is often associated with IgAN through various mechanisms, making it the most common cause of secondary IgAN. […] Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN.
- #33 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN. […] Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN.
- #34 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
The significance of mucosal-associated lymphoid tissue (MALT) has recently gained prominence in understanding the pathology of various diseases, especially IgAN, as IgA production predominantly occurs in mucosal tissue. […] Liver disease is often associated with IgAN through various mechanisms, making it the most common cause of secondary IgAN. […] Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN.
- #35 IgA Nephropathy (Bergerâs Disease): Causes, Symptoms, & Treatmenthttps://www.webmd.com/a-to-z-guides/what-is-iga-nephropathy
If you have this digestive problem, your body cant process gluten, a protein found in most grains. […] HIV and some bacterial infections are linked to an increased risk of IgA nephropathy. […] Its not common for people with IgA nephropathy to have a family history of kidney disease. Instead, they are more likely to have a family history of diseases involving the immune system, such as psoriasis or asthma. […] IgA nephropathy is a disease that progresses slowly for most people. The goal of early detection and treatment is to keep your kidneys working well and avoid kidney failure. Research suggests that 20%-50% of people with IgA nephropathy will eventually develop kidney failure and need dialysis or a kidney transplant.
- #36 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
IgA nephropathy has been reported in patients with HIV infection, both Whites and Blacks, despite the rarity of typical IgA nephropathy in the Black population. […] Although IgA nephropathy is usually a sporadic disease, data suggest that genetic factors are important in susceptibility to development of mesangial glomerulonephritis. Several cases of familial disease have been reported in Italy and the United States, and an autosomal dominant form has been linked to band 6q22-23. […] Additionally, increased frequency of specific HLA groups (HLA-DRB1 and HLA-DQB1) has been reported in some populations. […] A study of genetic risk for IgA nephropathy in Han Chinese identified three non-HLA gene regions (FBXL21, CCR6, and STAT3) and one HLA gene region (GABBR1) with suggestive significance; further analysis identified five novel susceptibility genes, TGFBI, CCR6, STAT3, GABBR1, and CFB, that may be involved in IgA nephropathy.
- #37 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Most cases of IgA nephropathy are idiopathic, but the onset or exacerbation of the disease is often preceded by a respiratory tract infection. Association with some bacteria, such as Haemophilus parainfluenzae, has been reported. A variety of other disorders have also been linked with IgA nephropathy, as discussed below. […] Glomerular IgA deposition is a common finding in cirrhosis, occurring in more than one-third of patients. Liver disease is accompanied by impaired removal of IgA-containing complexes by the Kupffer cells, predisposing patients to IgA deposition in the kidney. […] Glomerular IgA deposition occurs in up to a third of patients with gluten enteropathy. Most of these patients have no clinical manifestations of the disease. However, IgA nephropathy and gluten hypersensitivity are associated, and withdrawal of gluten from the diet of these patients has resulted in clinical and immunologic improvement of the renal disease.
- #38 Connecting the Dots on Disease | UHN Researchhttps://www.uhnresearch.ca/news/connecting-dots-disease
Researchers link IgA nephropathy to the bacterium Neisseria found in the throat and tonsils. […] A recent study has linked the disease known as Immunoglobulin A nephropathy to the body’s response to various strains of bacteria found in the body, such as Neisseria. […] Previous research has suggested that bacteria in our digestive tract may cause the immune system to malfunction, which may then lead to IgA nephropathy. […] The team found that individuals with the disease had much greater numbers of Neisseria bacteria on their tonsils. […] This suggests a big change in our understanding about the source of IgA and the trigger for its production in this disease. Our findings suggest that certain microbes in the throat such as Neisseria can overstimulate the immune response and ultimately lead to kidney damage, says Dr. Reich.
- #39 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN. […] Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN.
- #40 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN. […] Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN.
- #41 IgA Nephropathy (Berger Disease) – MD Searchlighthttps://mdsearchlight.com/kidney-health/iga-nephropathy-berger-disease/
There are also quite a few systemic conditions that can lead to secondary IgAN. This includes liver, gastrointestinal, autoimmune, and dermatological disorders, along with infections, and drugs. […] Another crucial factor to consider is the role of mucosal-associated lymphoid tissue (MALT) – tissue responsible for defending the body against environmental toxins and microbes. […] Certain gastrointestinal disorders can trigger IgAN. […] Celiac disease, a disease where you cannot tolerate gluten, is closely related to IgAN. […] Patients who have inflammatory bowel diseases like Crohn disease and ulcerative colitis also face a higher risk of developing IgAN, and can have increased mortality rates. […] Autoimmune disorders, allergic skin conditions, and infections – especially those of the upper respiratory tract – all seem to carry a higher risk of developing IgAN. […] Lastly, medications such as tumor necrosis factor- (TNF-) inhibitors, have been associated with IgAN due to their effects on the body’s immune response.
- #42 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Celiac disease is strongly correlated with IgAN and shares an autoimmune origin. […] Inflammatory bowel disease poses a risk factor for IgAN. […] Abnormal intestinal microbiota, comprising commensal gut bacteria, are highly associated with IgAN. […] Autoimmune disorders, including Sjgren syndrome, spondyloarthritis, systemic lupus erythematosus, and Behet disease, have been associated with the development of IgA. […] Dermatological disorders encompass a range of conditions, some of which are associated with IgAN. […] Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN.
- #43 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
Clinical symptoms of IgAN, including gross hematuria, are highly associated with preceding viral or bacterial infections, most often an upper respiratory tract infection (URI). […] Drug-induced IgAN involves diagnosing renal injury timing in relation to medication administration and ruling out other potential causes. […] Although uncommon, primary pulmonary diseases, such as sarcoidosis, idiopathic pulmonary fibrosis, or pulmonary hemorrhage, can be associated with IgAN. […] In addition, oncological processes, such as myeloproliferative disease, gastric carcinoma, lung cancers, renal cell carcinomas, and other malignancies, can also be associated with IgA.
- #44 IgA nephropathy | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/iga-nephropathy?lang=us
IgA nephropathy, also known as IgA nephritis or Berger disease, is a form of glomerulonephritis. […] IgA nephropathy is considered the most common glomerulonephritis worldwide and is a leading cause of chronic kidney disease. […] It is possible that mRNA-based vaccination against COVID-19 may associate with an increased frequency of IgA nephropathy. […] Primary IgA nephropathy is characterized by deposition of IgA antibodies in the glomeruli. This is thought to start with defective glycosylation of IgA subclass IgA1 that results in overproduction of galactose-deficient IgA1 (Gd-IgA1), occurrence of anti-Gd-IgA1 autoantibodies, and mesangial deposition of nephritogenic immune complexes.
- #45 IgA nephropathy – Wikipediahttps://en.wikipedia.org/wiki/IgA_nephropathy
IgA nephropathy (IgAN), also known as Berger’s disease, is a disease of the kidney and the immune system; specifically it is a form of glomerulonephritis or an inflammation of the glomeruli of the kidney. […] Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus. […] There is no clear known explanation for the accumulation of the IgA. Exogenous antigens for IgA have not been identified in the kidney, but it is possible that this antigen has been cleared before the disease manifests itself. It has also been proposed that IgA itself may be the antigen. […] A recently advanced theory focuses on abnormalities of the IgA1 molecule. […] Overall, these studies highlight the intricate relationship between genetics, diet, and IgAN, with potential implications for both mental health and dietary practices.
- #46 Azthena logo with the word Azthenahttps://www.news-medical.net/health/IgA-Nephropathy-Bergers-Disease.aspx
IgA nephropathy (IgAN), also known as Bergers disease, is the leading cause of primary glomerulonephritis all over the world. It is autoimmune in origin, characterized by the stimulation of genetically determined formation of concentration of IgA1 with galactose-deficient O-glycans in the hinge-region (Hit 1) in the blood. These stimulate anti-glycan antibody production, followed by binding with the aberrantly glycated IgA1. […] The basic defect in IgA nephropathy is not renal but systemic. In other words, the mesangial deposition of IgA-glycan complexes is related to a higher-than-normal level of abnormally glycosylated IgA1. This stimulates mesangial cell proliferation, expansion of the mesangial matrix, with the secretion of chemicals which finally result in glomerulosclerosis and interstitial fibrous tissue deposition. There are five gene markers of increased susceptibility to IgAN, such as on: chromosome 6p21, chromosome 1q32 (H locus), chromosome 22q22 (gene cluster). […] There are several risk factors for IgA nephropathy, include white or Asian origin, Family history of IgAN or of Henoch-Schonlein purpura and males between teenage to the late thirties.
- #47 IgA Nephropathy (Berger Disease): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5990-iga-nephropathy
IgA nephropathy, or Bergers disease, is a kidney disease in which IgA antibodies build up abnormally. Over time, it leads to kidney damage or kidney failure. […] Medical experts believe IgA nephropathy is an autoimmune disease. That means your immune system attacks your kidneys. […] People with IgA nephropathy have a higher level of IgA with less galactose than is typical. […] For some people, there’s a genetic family history of IgA nephropathy. Medical experts have discovered some genetic markers. That means there may be a link between a genetic variation and IgA nephropathy. […] Risk factors for IgA nephropathy include a genetic family history of IgA nephropathy. […] Although IgA can affect anyone, you may be more likely to have it if you’re male, in your late teens to late 30s, Asian or European.
- #48 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
More than 90% of cases are sporadic. […] No evidence suggests the involvement of a particular infectious agent despite the association between macroscopic hematuria and mucosal inflammation. […] No evidence indicates hypersensitivity to food antigens, except for a small group of patients with celiac disease. […] Abnormal O-glycosylation of the IgA1 hinge region promotes the formation of circulating IgA1-antibody immune complexes, leading to mesangial cell activation and deposition. […] Many systemic conditions are associated with IgA deposits, causing IgAN pathology. […] Recent reports suggest the pathways leading to glomerular injury are similar between primary and secondary IgAN. […] Secondary IgAN may arise from various conditions, including liver, gastrointestinal, autoimmune, dermatological, infectious, and drug-related causes.
- #49 A Kidney Diseaseâs Genetic Clues Are Uncovered | Columbia University Irving Medical Centerhttps://www.cuimc.columbia.edu/news/kidney-diseases-genetic-clues-are-uncovered
Researchers have uncovered new genetic clues to understanding IgA nephropathy (IgAN), or Bergers disease, an autoimmune kidney disease and a common cause of kidney failure. […] Very little is known about the causes of IgAN, genetic or otherwise, so our discovery represents an important step toward developing better therapies for this disease, said lead author Krzysztof Kiryluk, MD, the Herbert Irving Assistant Professor of Medicine at Columbia University Irving Medical Center (CUIMC). […] The key molecular defect in people with IgAN is abnormal O-glycosylation of IgA antibodies. […] Studies of families have shown that problems in the O-glycosylation of IgA are common in people with IgAN and are largely genetic in origin, although the exact genes involved were unknown. […] The researchers found that variations in two genes, C1GALT1 and C1GALT1C1, were significantly more common in people with high levels of the Gd-IgA1 marker.
- #50 What is IgA Nephropathy? – Immunoglobulin A [Berger’s Disease]https://igan.org/faq/
Making lifestyle changes is essential for managing IgA nephropathy and supporting kidney health. […] Elevated levels of galactose-deficient IgA are associated with certain autoimmune conditions, particularly IgA nephropathy. In this condition, the immune system produces an abnormal form of IgA that is deficient in galactose.
- #51 IgA nephropathy | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/iga-nephropathy?lang=us
IgA nephropathy, also known as IgA nephritis or Berger disease, is a form of glomerulonephritis. […] IgA nephropathy is considered the most common glomerulonephritis worldwide and is a leading cause of chronic kidney disease. […] It is possible that mRNA-based vaccination against COVID-19 may associate with an increased frequency of IgA nephropathy. […] Primary IgA nephropathy is characterized by deposition of IgA antibodies in the glomeruli. This is thought to start with defective glycosylation of IgA subclass IgA1 that results in overproduction of galactose-deficient IgA1 (Gd-IgA1), occurrence of anti-Gd-IgA1 autoantibodies, and mesangial deposition of nephritogenic immune complexes.
- #52 IgA nephropathy – Wikipediahttps://en.wikipedia.org/wiki/IgA_nephropathy
IgA nephropathy (IgAN), also known as Berger’s disease, is a disease of the kidney and the immune system; specifically it is a form of glomerulonephritis or an inflammation of the glomeruli of the kidney. […] Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus. […] There is no clear known explanation for the accumulation of the IgA. Exogenous antigens for IgA have not been identified in the kidney, but it is possible that this antigen has been cleared before the disease manifests itself. It has also been proposed that IgA itself may be the antigen. […] A recently advanced theory focuses on abnormalities of the IgA1 molecule. […] Overall, these studies highlight the intricate relationship between genetics, diet, and IgAN, with potential implications for both mental health and dietary practices.
- #53 IgA Nephropathy (Berger’s Disease): ERKNet voor Patiëntenhttps://www.erknet.org/patients/nl/your-kidney-disease/iga-nephropathy/disease-information
IgA Nephropathy (IgAN), in past sometimes known as Bergers Disease, is an inflammatory disease of the kidney, the most common primary glomerulonephritis worldwide as well a leading cause of End-Stage Kidney Disease (ESKD). […] The exact cause of the disease has not been fully determined. Extensive research in recent years has found that several mechanisms underlie the disease. It is presumed that the development of the disease occurs due to a combination of environmental factors and the presence of genetic predisposition. […] IgAN can be secondary to other diseases, e.g. systemic diseases, including: Ankylosing Spondylitis(AS) Rheumatoid arthritis Reiters Syndrome Coeliac Disease Inflammatory Bowel Disease Alcoholic Liver Disease Sarcoidosis Psoriasis Hepatitis B or C. […] The presence of immune complexes composed of galactose-deficient IgA itself and/or IgA-IgG complexes promotes their binding to receptors and deposition of in the glomeruli/ mesangium. […] The deposits stimulate an inflammatory response and activation of the complement system, followed by mesangial cell division and an overproduction of matrix.
- #54 Causes and Risk Factors of IgA Nephropathyhttps://www.health.com/iga-nephropathy-causes-8702882
Although it’s not clear what actually causes the IgA protein to build up in the kidneys, the following health conditions are strongly linked to developing Berger’s disease: […] Research suggests that several other health conditions may have some connection to this kidney disease, too: […] However, underlying health conditions, genetics, and environmental factors aren’t the only things that can raise your risk of Berger’s disease. Your lifestyle habits may also play a role. […] The exact cause of IgA nephropathy is unknown, but research suggests that it results from a combination of genetic and environmental triggers. Factors like a family history of the disease, male gender, white or Asian ancestry, smoking, and having certain underlying conditions can increase your risk of developing this disease.
- #55 Mayo Clinic Health Library – IgA nephropathy (Berger disease) | Swiss Medical Networkhttps://www.swissmedical.net/en/healtcare-library/con-20166955
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. This causes inflammation and affects their filtering ability over time. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. Infections. These include HIV and some bacterial infections. […] The exact cause of IgA nephropathy is unknown. But these factors might raise the risk of getting it: Sex. In North America and Western Europe, IgA nephropathy affects at least twice as many men as it does women. Ethnicity. IgA nephropathy is more common in white people and people of Asian descent than it is in Black people. Age. IgA nephropathy most often develops between the mid-teens and mid-30s. Family history. IgA nephropathy appears to run in some families.
- #56 Symptoms and Signs of IgA Nephropathy (Berger’s Disease): Treatmenthttps://www.emedicinehealth.com/what_is_iga_nephropathy_bergers_disease/symptom.htm
Berger’s disease (also known as IgA nephropathy) is renal disease that occurs when the protein IgA (immunoglobulin A) deposits or accumulates inside the renal filters (glomeruli) in the kidneys. […] Although the cause of Berger’s disease is IgA accumulation in the kidneys, the underlying reason for its accumulation is unknown. […] Risk factors include the following: Sex: It affects twice as many men as women in Western Europe and North America. Ethnicity: It’s more common in whites and Asians than blacks. Age: It often develops between the late teens to late thirties. Family history: It sometimes occurs in families.
- #57 Mayo Clinic Health Library – IgA nephropathy (Berger disease) | Swiss Medical Networkhttps://www.swissmedical.net/en/healtcare-library/con-20166955
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. This causes inflammation and affects their filtering ability over time. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. Infections. These include HIV and some bacterial infections. […] The exact cause of IgA nephropathy is unknown. But these factors might raise the risk of getting it: Sex. In North America and Western Europe, IgA nephropathy affects at least twice as many men as it does women. Ethnicity. IgA nephropathy is more common in white people and people of Asian descent than it is in Black people. Age. IgA nephropathy most often develops between the mid-teens and mid-30s. Family history. IgA nephropathy appears to run in some families.
- #58 Azthena logo with the word Azthenahttps://www.news-medical.net/health/IgA-Nephropathy-Bergers-Disease.aspx
IgA nephropathy (IgAN), also known as Bergers disease, is the leading cause of primary glomerulonephritis all over the world. It is autoimmune in origin, characterized by the stimulation of genetically determined formation of concentration of IgA1 with galactose-deficient O-glycans in the hinge-region (Hit 1) in the blood. These stimulate anti-glycan antibody production, followed by binding with the aberrantly glycated IgA1. […] The basic defect in IgA nephropathy is not renal but systemic. In other words, the mesangial deposition of IgA-glycan complexes is related to a higher-than-normal level of abnormally glycosylated IgA1. This stimulates mesangial cell proliferation, expansion of the mesangial matrix, with the secretion of chemicals which finally result in glomerulosclerosis and interstitial fibrous tissue deposition. There are five gene markers of increased susceptibility to IgAN, such as on: chromosome 6p21, chromosome 1q32 (H locus), chromosome 22q22 (gene cluster). […] There are several risk factors for IgA nephropathy, include white or Asian origin, Family history of IgAN or of Henoch-Schonlein purpura and males between teenage to the late thirties.
- #59 Mayo Clinic Health Library – IgA nephropathy (Berger disease) | Swiss Medical Networkhttps://www.swissmedical.net/en/healtcare-library/con-20166955
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. This causes inflammation and affects their filtering ability over time. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. Infections. These include HIV and some bacterial infections. […] The exact cause of IgA nephropathy is unknown. But these factors might raise the risk of getting it: Sex. In North America and Western Europe, IgA nephropathy affects at least twice as many men as it does women. Ethnicity. IgA nephropathy is more common in white people and people of Asian descent than it is in Black people. Age. IgA nephropathy most often develops between the mid-teens and mid-30s. Family history. IgA nephropathy appears to run in some families.
- #60 What is IgA Nephropathy?https://ckd.responsumhealth.com/iga-nephropathy
What is IgA Nephropathy? […] IgA nephropathy is the third most common type of kidney disease. Learn about its causes, risk factors, and symptoms, as well as how its diagnosed and how its treated. […] Experts think that IgAN is an autoimmune disease, which is when cells that are designed to protect your body start attacking it instead. People who have IgAN have a higher level of Immunoglobulin A (IgA) in their blood, which contains less of a type of sugar, called galactose. […] There may also be a genetic component to the diseases development. […] IgAN is the third most common type of kidney disease, after those caused by diabetes and hypertension, and it can occur to people of all ages, sexes, and races. Scientists have identified certain factors, however, that can increase your risk. […] These risk factors include: Male sex, Late teen to early 30s, Family history of IgAN or IgA vasculitis, Asian or Caucasian.
- #61 Mayo Clinic Health Library – IgA nephropathy (Berger disease) | Swiss Medical Networkhttps://www.swissmedical.net/en/healtcare-library/con-20166955
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. This causes inflammation and affects their filtering ability over time. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. Infections. These include HIV and some bacterial infections. […] The exact cause of IgA nephropathy is unknown. But these factors might raise the risk of getting it: Sex. In North America and Western Europe, IgA nephropathy affects at least twice as many men as it does women. Ethnicity. IgA nephropathy is more common in white people and people of Asian descent than it is in Black people. Age. IgA nephropathy most often develops between the mid-teens and mid-30s. Family history. IgA nephropathy appears to run in some families.
- #62 IgA Nephropathy (Bergerâs Disease) â Purivo Dialysis Centerhttps://purivodialysiscenter.com/kidney-disease/overview/assessing-your-risk/iga-nephropathy-bergers-disease/
Although the exact cause of IgA nephropathy is unknown, these factors may increase your risk of developing this condition: Sex. In North America and western Europe, IgA nephropathy affects at least twice as many men as it does women; Ethnicity. IgA nephropathy is more common in Caucasians and Asians than it is in blacks; Family history. In some cases, IgA nephropathy appears to run in families, indicating that genetic factors may contribute to the disease.
- #63 IgA Nephropathy – Capital Nephrology Medical Grouphttps://www.capitalnephrology.com/iga-nephropathy
IgA nephropathy is a kidney disorder in which antibodies to a protein called IgA build up in kidney tissue. […] It is also called Bergers disease. […] IgA nephropathy (Bergers disease) occurs when too much of this protein is deposited in the kidneys. […] IgA nephropathy (Bergers disease) is a form of mesangial proliferative nephritis. […] Risk factors include: A personal or family history of IgA nephropathy or Henoch Schonlein purpura, a form of vasculitis that affects many parts of the body.
- #64 IgA nephropathy: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000466.htm
IgA nephropathy is a kidney disorder in which antibodies (called IgA) build up in kidney tissue. Nephropathy is damage, disease, or other problems with the kidney. […] IgA nephropathy occurs when too much of this protein is deposited in the kidneys. IgA builds up inside the small blood vessels of the kidney. Structures in the kidney called glomeruli become inflamed and damaged. […] Risk factors include: A personal or family history of IgA nephropathy or Henoch-Schönlein purpura, a form of vasculitis that affects many parts of the body. […] IgA nephropathy can occur in people of all ages, but it most often affects males in their teens to late 30s.
- #65 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK538214/
Immunoglobulin A (IgA) nephropathy, or IgAN, also known as Berger disease, is one of the leading causes of glomerulonephritis and renal failure. This disease is a prevalent form of glomerulonephritis characterized by the deposition of IgA in the glomerular basement membrane. […] IgAN results from abnormal immune reactions, resulting in IgA deposits within the glomerulus, increased podocyte permeability, and interstitial fibrosis. […] Although IgAN is often preceded by an infectious disease, which triggers a dysregulated immune response, it is noteworthy that IgAN itself is not of an infectious etiology. No evidence suggests that specific infectious agents cause IgAN. Instead, the immune system appears to be activated by various clinical and subclinical triggers, along with genetic factors related to IgA glycosylation.
- #66 Causes and Risk Factors of IgA Nephropathyhttps://www.health.com/iga-nephropathy-causes-8702882
Immunoglobulin A nephropathy (also known as IgA nephropathy or Berger’s disease) is an autoimmune disease that affects the kidneys. It develops when too much of the protein IgA builds up in the kidneys, damaging the organ’s network that filters waste and fluids from the blood. […] It’s not entirely clear what causes IgA nephropathy to develop, but research suggests that genetics and environmental factors can influence the condition to develop. […] Researchers don’t know exactly why this happens, but they have proposed a few theories: genetics and environmental triggers. […] There appears to be a genetic component to having IgA nephropathy. Studies suggest that your chances of developing IgA nephropathy increase if you have a family history of the disease. […] Experts believe that a combination of genetic and environmental factors contribute to IgA nephropathy’s development.
- #67 IgA Nephropathy (Berger Disease) | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/23357
More than 90% of cases are sporadic. […] No evidence suggests the involvement of a particular infectious agent despite the association between macroscopic hematuria and mucosal inflammation. […] No evidence indicates hypersensitivity to food antigens, except for a small group of patients with celiac disease. […] Abnormal O-glycosylation of the IgA1 hinge region promotes the formation of circulating IgA1-antibody immune complexes, leading to mesangial cell activation and deposition. […] Many systemic conditions are associated with IgA deposits, causing IgAN pathology. […] Recent reports suggest the pathways leading to glomerular injury are similar between primary and secondary IgAN. […] Secondary IgAN may arise from various conditions, including liver, gastrointestinal, autoimmune, dermatological, infectious, and drug-related causes.
- #68 IgA nephropathy (Berger disease) | Altru Health Systemhttps://www.altru.org/health-library/conditions/iga-nephropathy-berger-disease
IgA nephropathy (nuh-FROP-uh-thee), also known as Berger disease, is a kidney disease. It happens when a germ-fighting protein called immunoglobulin A (IgA) builds up in the kidneys. […] Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it: […] Genes. IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent. […] Liver diseases. These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections. […] Celiac disease. Eating gluten, a protein found in most grains, triggers this digestive condition. […] Infections. These include HIV and some bacterial infections.
- #69 A Kidney Diseaseâs Genetic Clues Are Uncovered | Columbia University Irving Medical Centerhttps://www.cuimc.columbia.edu/news/kidney-diseases-genetic-clues-are-uncovered
Researchers have uncovered new genetic clues to understanding IgA nephropathy (IgAN), or Bergers disease, an autoimmune kidney disease and a common cause of kidney failure. […] Very little is known about the causes of IgAN, genetic or otherwise, so our discovery represents an important step toward developing better therapies for this disease, said lead author Krzysztof Kiryluk, MD, the Herbert Irving Assistant Professor of Medicine at Columbia University Irving Medical Center (CUIMC). […] The key molecular defect in people with IgAN is abnormal O-glycosylation of IgA antibodies. […] Studies of families have shown that problems in the O-glycosylation of IgA are common in people with IgAN and are largely genetic in origin, although the exact genes involved were unknown. […] The researchers found that variations in two genes, C1GALT1 and C1GALT1C1, were significantly more common in people with high levels of the Gd-IgA1 marker.
- #70 Azthena logo with the word Azthenahttps://www.news-medical.net/health/IgA-Nephropathy-Bergers-Disease.aspx
IgA nephropathy (IgAN), also known as Bergers disease, is the leading cause of primary glomerulonephritis all over the world. It is autoimmune in origin, characterized by the stimulation of genetically determined formation of concentration of IgA1 with galactose-deficient O-glycans in the hinge-region (Hit 1) in the blood. These stimulate anti-glycan antibody production, followed by binding with the aberrantly glycated IgA1. […] The basic defect in IgA nephropathy is not renal but systemic. In other words, the mesangial deposition of IgA-glycan complexes is related to a higher-than-normal level of abnormally glycosylated IgA1. This stimulates mesangial cell proliferation, expansion of the mesangial matrix, with the secretion of chemicals which finally result in glomerulosclerosis and interstitial fibrous tissue deposition. There are five gene markers of increased susceptibility to IgAN, such as on: chromosome 6p21, chromosome 1q32 (H locus), chromosome 22q22 (gene cluster). […] There are several risk factors for IgA nephropathy, include white or Asian origin, Family history of IgAN or of Henoch-Schonlein purpura and males between teenage to the late thirties.
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