Nefropatia iga (choroba bergera)
Rokowania, prognozy i postęp choroby
Nefropatia IgA (choroba Bergera) to przewlekła choroba nerek o zmiennym przebiegu, dotykająca głównie młodych dorosłych. Kluczowymi czynnikami prognostycznymi są białkomocz ≥ 1 g/dobę (z 46-krotnie wyższym ryzykiem ESRD w porównaniu do białkomoczu < 500 mg/dobę), utrzymujące się nadciśnienie tętnicze oraz wynik klasyfikacji Oxford MEST-C, która ocenia cechy histologiczne takie jak rozrost mezangium (M1), hipercelularność śródbłonkowa (E1), segmentalne stwardnienie kłębuszków (S1), włóknienie śródmiąższowe i zanik kanalików (T), oraz obecność półksiężyców (C1, C2). Dodatkowo, wysokie stężenie kreatyniny (≥ 120 mmol/L), wiek ≥ 30 lat przy rozpoznaniu, obecność barwienia C4d oraz rozległe włóknienie śródmiąższowe pogarszają rokowanie. Kalkulatory prognostyczne, w tym narzędzia międzynarodowe i model Xie et al., uwzględniają m.in. GFR, hemoglobinę, albuminę i ciśnienie skurczowe, umożliwiając indywidualizację oceny ryzyka progresji do ESRD.
Przebieg nefropatii IgA jest nieprzewidywalny – około 15-30% pacjentów rozwija schyłkową niewydolność nerek (ESRD) w ciągu 10-20 lat, a około 30% wymaga dializy lub przeszczepu nerki. Przeszczep jest skuteczny, jednak ryzyko nawrotu choroby wynosi 20-40%, co zwiększa ryzyko utraty przeszczepu (2,8-krotnie wyższe). Leczenie koncentruje się na kontroli białkomoczu, nadciśnienia i immunosupresji, a także na ograniczeniu spożycia białka i stosowaniu leków modyfikujących przebieg choroby (np. blokada RAAS, kwasy omega-3). Wczesna diagnoza i monitorowanie są kluczowe dla poprawy rokowania, a postępy w terapii, w tym nowe metody leczenia, są obecnie przedmiotem badań klinicznych. Izolowany mikroskopowy krwiomocz z łagodnym białkomoczem wskazuje na korzystne rokowanie.
Rokowanie w nefropatii IgA (choroba Bergera)
Nefropatia IgA (choroba Bergera) jest częstą przewlekłą chorobą nerek, która dotyka głównie młodych dorosłych. Przebieg choroby jest zróżnicowany i trudno przewidzieć jej rozwój u poszczególnych pacjentów.12 Do 50% pacjentów z nefropatią IgA doświadcza łagodnego przebiegu choroby, natomiast u pozostałych może ona przebiegać bardziej agresywnie.3
Czynniki prognostyczne
Rokowanie w nefropatii IgA można przewidzieć na podstawie kilku kluczowych czynników. Trzy główne czynniki ryzyka związane z koniecznością dializoterapii lub zgonem pacjenta to:4
- Białkomocz ≥ 1 g/dobę – pacjenci z utrzymującym się białkomoczem na tym poziomie mają 46-krotnie wyższe ryzyko rozwoju schyłkowej niewydolności nerek (ESRD) w porównaniu z pacjentami z białkomoczem poniżej 500 mg/dobę
- Utrzymujące się nadciśnienie tętnicze
- Wynik klasyfikacji Oxford MEST-C (w niektórych algorytmach uwzględniana jest również kategoria dla półksiężyców)
Dodatkowo, innymi czynnikami prognostycznymi związanymi z gorszym rokowaniem są:56
- Wysokie stężenie kreatyniny w surowicy (≥ 120 mmol/L) przy rozpoznaniu
- Nadciśnienie tętnicze (ciśnienie rozkurczowe ≥ 95 mmHg lub konieczność stosowania leków przeciwnadciśnieniowych)
- Rozległe włóknienie śródmiąższowe i zanik kanalików w biopsji nerki
- Obecność barwienia C4d w biopsji
- Wiek ≥ 30 lat w momencie pojawienia się objawów choroby
- Ciężkie zmiany w początkowej biopsji, zwłaszcza ślady stwardnienia (bliznowacenia) lub tworzenia półksiężyców
Klasyfikacja MEST-C i jej znaczenie prognostyczne
Klasyfikacja Oxford nefropatii IgA, znana również jako skala MEST-C, obejmuje pięć cech histologicznych, które są niezależnymi predyktorami wyniku klinicznego:7
| Cecha | Znaczenie kliniczne |
|---|---|
| M1 (rozrost mezangium) | Gorsze wyniki niż M0 |
| E1 (hipercelularność śródbłonkowa) | Gorsze przeżycie nerek u pacjentów nieleczonych immunosupresyjnie; lepsze przeżycie nerek przy immunosupresji |
| S1 (segmentalne stwardnienie kłębuszków) | Predyktor gorszych wyników |
| T (włóknienie śródmiąższowe/zanik kanalików) | Najsilniejszy predyktor gorszych wyników |
| C1 (półksiężyce w 1-24% kłębuszków) | Predyktor gorszych wyników bez immunosupresji, ale nie przy stosowaniu immunosupresji |
| C2 (półksiężyce w ≥25% kłębuszków) | Predyktor gorszych wyników niezależnie od immunosupresji |
Kalkulatory prognostyczne
Opracowano szereg kalkulatorów do określania rokowania u pacjentów z nefropatią IgA:8
- Międzynarodowe narzędzie predykcyjne IgAN w momencie biopsji – dorośli
- Międzynarodowe narzędzie predykcyjne IgAN w momencie biopsji – dzieci
- Międzynarodowe narzędzie predykcyjne IgAN po biopsji – dorośli
- Międzynarodowe narzędzie predykcyjne IgAN po biopsji – dzieci
Dodatkowo, Xie i wsp. opracowali kalkulator do szacowania ryzyka progresji do ESRD u pacjentów z nefropatią IgA, oparty na kohorcie 619 chińskich pacjentów. Kalkulator ten wykorzystuje cztery zmienne:9
- Współczynnik filtracji kłębuszkowej
- Poziom hemoglobiny
- Poziom albuminy w surowicy
- Skurczowe ciśnienie krwi
Długotwerminowe rokowanie i postęp choroby
Nefropatia IgA charakteryzuje się zmiennym przebiegiem, co może prowadzić do różnych scenariuszy:1011
- Powolna utrata funkcji nerek
- Całkowita niewydolność nerek, która może ostatecznie wymagać dializy lub przeszczepu nerki
- Nawrót nefropatii IgA po przeszczepie nerki
Około 30% pacjentów z nefropatią IgA ostatecznie utraci funkcję nerek i będzie wymagać przeszczepu lub dializoterapii.12 Badania sugerują, że u około 15-20% pacjentów rozwinie się schyłkowa niewydolność nerek (ESRD) w ciągu 10 lat od wystąpienia choroby, a u 25-30% pacjentów w ciągu 20 lat.13 Według innych danych, około 20% osób z nefropatią IgA typowo rozwija ESRD w ciągu 10 lat od wystąpienia choroby.14 Szacuje się również, że nawet połowa osób z IgAN może rozwinąć schyłkową niewydolność nerek.15
Dane z badań z 2018 roku sugerują, że nefropatia IgA może skrócić oczekiwaną długość życia o około 10 lat.16 Jednakże, przy odpowiednim leczeniu i opiece medycznej, wiele osób z nefropatią IgA może prowadzić względnie normalne życie.17
Remisja i odpowiedź na leczenie
Około połowa pacjentów z nefropatią IgA może uzyskać całkowitą remisję podczas leczenia, podczas gdy pozostała połowa może wykazywać pewien stopień trwającej choroby.18 Niektórzy pacjenci doświadczają całkowitej remisji, inni żyją normalnie dzięki lekom leczącym objawy (takim jak leki na nadciśnienie, leki immunosupresyjne, kwasy tłuszczowe omega-3, suplementy witaminy E), a jeszcze inni doświadczają całkowitej niewydolności nerek i wymagają dializy lub przeszczepu nerki.19
Głównym celem leczenia jest uniknięcie konieczności dializy lub przeszczepu nerki. Jednakże, jeśli nerki przestaną funkcjonować wystarczająco dobrze samodzielnie, oba te zabiegi mogą ratować życie.20 Wiele nowych metod leczenia zostało zatwierdzonych lub jest testowanych w badaniach klinicznych, dlatego warto rozmawiać z lekarzem o najnowszych postępach w leczeniu nefropatii IgA.21
Nawrót po przeszczepie nerki
Przeszczep nerki jest zazwyczaj skuteczny, ale nefropatia IgA może powrócić w nowej nerce.22 Ryzyko nawrotu IgAN po przeszczepie nerki wynosi około 27,7% (95% CI: 20,6-35,3%) po 11 latach.23 Według innych źródeł, około 20-40% pacjentów doświadczy nawrotu objawów po przeszczepie nerki, zazwyczaj w ciągu 2,5 roku po transplantacji, choć może to nastąpić wcześniej lub później.24
W badaniach wykazano, że wskaźnik utraty przeszczepu u pacjentów z nawrotem choroby był 2,8-krotnie wyższy niż u pacjentów bez nawrotu.25 Jednak wyniki trzech badań wykazały, że jak dotąd nie ma wiarygodnych parametrów klinicznych ani biomarkerów pozwalających przewidzieć nawrót IgAN po transplantacji.26 Częstość utraty przeszczepu w najnowszych badaniach była niższa niż w poprzednich porównywalnych badaniach, co może być wyjaśnione powszechnym stosowaniem prednizonu i blokady układu renina-angiotensyna-aldosteron (RAAS).27
Korzystne czynniki prognostyczne
Izolowany krwiomocz mikroskopowy z łagodnym białkomoczem sugeruje korzystne rokowanie, wskazując na łagodny i bezobjawowy przebieg choroby.28 Wczesna diagnoza, właściwe leczenie i regularne monitorowanie mogą znacznie poprawić rokowanie u pacjentów z nefropatią IgA. Ograniczenie spożycia białka może pomóc w spowolnieniu progresji nefropatii IgA i ochronie nerek.29
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Materiały źródłowe
- #1 IgA nephropathy (IgAN): symptoms, treatments, diagnosis and causeshttps://www.kidneyresearchuk.org/conditions-symptoms/iga-nephropathy/
IgA nephropathy (IgAN) is a common chronic kidney disease which mainly affects young adults. […] Around 30 per cent of IgAN patients will go on to lose kidney function and will require a transplant or life on dialysis. There are currently no reliable tests available to predict which of these patients, known as progressors, will lose their kidney function. […] It can be difficult to predict how or if the disease will develop. IgAN is a variable condition, so a number of things could happen: […] Your kidneys may slowly lose function […] Your kidneys may fail completely, which could lead to you eventually requiring dialysis or a kidney transplant […] IgAN may recur after a kidney transplant.
- #2 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. […] 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. […] 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. […] Ask your doctor if you should cut back on protein. This may help slow IgA nephropathy from becoming worse and protect your kidneys.
- #3 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/books/NBK538214/
Up to 50% of IgAN patients experience a benign course. Prognosis can be somewhat predictable, particularly based on the Oxford classification outlined in the Pathology section. Any of the MEST criteria (and crescents) serve as poor prognostic indicators. Isolated microscopic hematuria with mild proteinuria suggests a favorable prognosis, indicating a benign and asymptomatic disease course. Additionally, individuals of East Asian origin are significantly associated with an elevated risk of ESRD. […] Three risk factors highly associated with dialysis or death include: […] Proteinuria of 1 g/d or more […] Sustained hypertension […] Oxford classification MEST-C score (to which some algorithms also include a category for crescents). […] Patients with sustained proteinuria of 1 g/d or higher have a 46-fold increased risk of ESRD than those with less than 500 mg/d. About half of the patients with IgAN may obtain complete remission with treatment, whereas the remaining half may show some degree of ongoing disease.
- #4 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/books/NBK538214/
Up to 50% of IgAN patients experience a benign course. Prognosis can be somewhat predictable, particularly based on the Oxford classification outlined in the Pathology section. Any of the MEST criteria (and crescents) serve as poor prognostic indicators. Isolated microscopic hematuria with mild proteinuria suggests a favorable prognosis, indicating a benign and asymptomatic disease course. Additionally, individuals of East Asian origin are significantly associated with an elevated risk of ESRD. […] Three risk factors highly associated with dialysis or death include: […] Proteinuria of 1 g/d or more […] Sustained hypertension […] Oxford classification MEST-C score (to which some algorithms also include a category for crescents). […] Patients with sustained proteinuria of 1 g/d or higher have a 46-fold increased risk of ESRD than those with less than 500 mg/d. About half of the patients with IgAN may obtain complete remission with treatment, whereas the remaining half may show some degree of ongoing disease.
- #5 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Other predictors of poor renal outcomes include the following: High serum creatinine level ( 120 mmol/L) at presentation; Hypertension (diastolic pressure 95 mm Hg or need for antihypertensive treatment); Proteinuria: Urinary protein excretion 1 g/24 hr is associated with 56% renal survival and 3.5 g/24 hr with 7% renal survival; Extensive interstitial fibrosis and tubular atrophy on kidney biopsy; C4d staining on biopsy. […] Calculators for determining prognosis in patients with IgA nephropathy include the following: International IgAN Prediction Tool at biopsy – Adults; International IgAN Prediction Tool at biopsy – Pediatrics; International IgAN Prediction Tool post-biopsy – Adults; International IgAN Prediction Tool post-biopsy Pediatric. […] A calculator for estimating the risk of progression to ESRD in patients with IgA nephropathy has been developed by Xie et al, based on a cohort of 619 Chinese patients. It has yet to be validated in other ethnic groups. The calculator uses four variables: Glomerular filtration rate; Hemoglobin level; Serum albumin level; Systolic blood pressure.
- #6 What is IgA Nephropathy? – Immunoglobulin A [Berger’s Disease]https://igan.org/faq/
IgA nephropathy cannot be cured, but medications can help slow it down. […] The prognosis for individuals with IgA nephropathy varies depending on the severity of the condition, kidney biopsy findings, the amount of protein in the urine, response to treatment, and overall kidney function. With proper management and medical care, many individuals with IgA nephropathy can lead a relatively normal life. […] It is difficult to predict from initial clinical signs and biopsy results just which patients will have which course. Statistical analyses have yielding conflicting results, but some factors do seem linked to a poor prognosis. Unremittingly heavy proteinuria, uncontrolled hypertension, declining glomerular filtration rate, high serum creatinine, and being age 30 or older at the time of apparent onset of the disease are some of the factors tied to a poor prognosis. Severe lesions in the initial biopsy, especially evidence of sclerosis [scarring] or crescent formation are also ominous signs.
- #7 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Although IgA nephropathy usually follows a benign course, end-stage kidney disease (ESKD) develops in 15-20% of patients within 10 years of onset and in about 25-30% of patients by 20 years. Efforts have been made to determine clinical and histologic features associated with progression to ESKD. […] The Oxford classification of IgA nephropathy, or MEST score, published in 2009, comprises four histologic features that are independent predictors of clinical outcome. […] The clinical significance of the individual MEST-C features is as follows: M1 Worse outcomes than M0; E1 Worse renal survival in patients not on immunosuppression and improved renal survival with immunosuppression; S1 Predictive of worse outcomes; T Strongest predictor of worse outcomes; C1 Predictive of worse outcomes if no immunosuppression is given, but not if immunosuppression is used; C2 is predictive of worse outcomes regardless of immunosuppression.
- #8 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Other predictors of poor renal outcomes include the following: High serum creatinine level ( 120 mmol/L) at presentation; Hypertension (diastolic pressure 95 mm Hg or need for antihypertensive treatment); Proteinuria: Urinary protein excretion 1 g/24 hr is associated with 56% renal survival and 3.5 g/24 hr with 7% renal survival; Extensive interstitial fibrosis and tubular atrophy on kidney biopsy; C4d staining on biopsy. […] Calculators for determining prognosis in patients with IgA nephropathy include the following: International IgAN Prediction Tool at biopsy – Adults; International IgAN Prediction Tool at biopsy – Pediatrics; International IgAN Prediction Tool post-biopsy – Adults; International IgAN Prediction Tool post-biopsy Pediatric. […] A calculator for estimating the risk of progression to ESRD in patients with IgA nephropathy has been developed by Xie et al, based on a cohort of 619 Chinese patients. It has yet to be validated in other ethnic groups. The calculator uses four variables: Glomerular filtration rate; Hemoglobin level; Serum albumin level; Systolic blood pressure.
- #9 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Other predictors of poor renal outcomes include the following: High serum creatinine level ( 120 mmol/L) at presentation; Hypertension (diastolic pressure 95 mm Hg or need for antihypertensive treatment); Proteinuria: Urinary protein excretion 1 g/24 hr is associated with 56% renal survival and 3.5 g/24 hr with 7% renal survival; Extensive interstitial fibrosis and tubular atrophy on kidney biopsy; C4d staining on biopsy. […] Calculators for determining prognosis in patients with IgA nephropathy include the following: International IgAN Prediction Tool at biopsy – Adults; International IgAN Prediction Tool at biopsy – Pediatrics; International IgAN Prediction Tool post-biopsy – Adults; International IgAN Prediction Tool post-biopsy Pediatric. […] A calculator for estimating the risk of progression to ESRD in patients with IgA nephropathy has been developed by Xie et al, based on a cohort of 619 Chinese patients. It has yet to be validated in other ethnic groups. The calculator uses four variables: Glomerular filtration rate; Hemoglobin level; Serum albumin level; Systolic blood pressure.
- #10 IgA nephropathy (IgAN): symptoms, treatments, diagnosis and causeshttps://www.kidneyresearchuk.org/conditions-symptoms/iga-nephropathy/
IgA nephropathy (IgAN) is a common chronic kidney disease which mainly affects young adults. […] Around 30 per cent of IgAN patients will go on to lose kidney function and will require a transplant or life on dialysis. There are currently no reliable tests available to predict which of these patients, known as progressors, will lose their kidney function. […] It can be difficult to predict how or if the disease will develop. IgAN is a variable condition, so a number of things could happen: […] Your kidneys may slowly lose function […] Your kidneys may fail completely, which could lead to you eventually requiring dialysis or a kidney transplant […] IgAN may recur after a kidney transplant.
- #11 IgA Nephropathy (Bergerâs Disease): Diet, Symptoms, and Morehttps://www.healthline.com/health/iga-nephropathy-bergers
Theres no cure for IgA nephropathy and no reliable way to tell how it will affect each person individually. For most people, the disease progresses slowly. […] Research from 2018 suggests having IgA nephropathy can decrease a persons lifespan by about 10 years. […] Roughly 20% of people with IgA nephropathy typically develop ESRD within 10 years of onset. Some signs may indicate an unfavorable prognosis, including: long-term hypertension, persistent blood in the urine, prolonged protein in the urine (above 1 gram/day), high serum creatinine level, widespread intestinal fibrosis of the kidneys. […] People with ESRD require dialysis treatment or a kidney transplant. A kidney transplant is typically successful, but IgA nephropathy can come back (recur) in the new kidney. […] About 20-40% of people will have symptom recurrence after a kidney transplant. Recurrence typically happens within 2.5 years after transplantation but can occur before or after that. […] Recent research has found that IgA nephropathy can decrease a persons lifespan by about 10 years. […] For many people, IgA nephropathy progresses slowly. Some people may also develop kidney failure and other life threatening complications.
- #12 IgA nephropathy (IgAN): symptoms, treatments, diagnosis and causeshttps://www.kidneyresearchuk.org/conditions-symptoms/iga-nephropathy/
IgA nephropathy (IgAN) is a common chronic kidney disease which mainly affects young adults. […] Around 30 per cent of IgAN patients will go on to lose kidney function and will require a transplant or life on dialysis. There are currently no reliable tests available to predict which of these patients, known as progressors, will lose their kidney function. […] It can be difficult to predict how or if the disease will develop. IgAN is a variable condition, so a number of things could happen: […] Your kidneys may slowly lose function […] Your kidneys may fail completely, which could lead to you eventually requiring dialysis or a kidney transplant […] IgAN may recur after a kidney transplant.
- #13 IgA Nephropathy: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/239927-overview
Although IgA nephropathy usually follows a benign course, end-stage kidney disease (ESKD) develops in 15-20% of patients within 10 years of onset and in about 25-30% of patients by 20 years. Efforts have been made to determine clinical and histologic features associated with progression to ESKD. […] The Oxford classification of IgA nephropathy, or MEST score, published in 2009, comprises four histologic features that are independent predictors of clinical outcome. […] The clinical significance of the individual MEST-C features is as follows: M1 Worse outcomes than M0; E1 Worse renal survival in patients not on immunosuppression and improved renal survival with immunosuppression; S1 Predictive of worse outcomes; T Strongest predictor of worse outcomes; C1 Predictive of worse outcomes if no immunosuppression is given, but not if immunosuppression is used; C2 is predictive of worse outcomes regardless of immunosuppression.
- #14 IgA Nephropathy (Bergerâs Disease): Diet, Symptoms, and Morehttps://www.healthline.com/health/iga-nephropathy-bergers
Theres no cure for IgA nephropathy and no reliable way to tell how it will affect each person individually. For most people, the disease progresses slowly. […] Research from 2018 suggests having IgA nephropathy can decrease a persons lifespan by about 10 years. […] Roughly 20% of people with IgA nephropathy typically develop ESRD within 10 years of onset. Some signs may indicate an unfavorable prognosis, including: long-term hypertension, persistent blood in the urine, prolonged protein in the urine (above 1 gram/day), high serum creatinine level, widespread intestinal fibrosis of the kidneys. […] People with ESRD require dialysis treatment or a kidney transplant. A kidney transplant is typically successful, but IgA nephropathy can come back (recur) in the new kidney. […] About 20-40% of people will have symptom recurrence after a kidney transplant. Recurrence typically happens within 2.5 years after transplantation but can occur before or after that. […] Recent research has found that IgA nephropathy can decrease a persons lifespan by about 10 years. […] For many people, IgA nephropathy progresses slowly. Some people may also develop kidney failure and other life threatening complications.
- #15 What is IgA Nephropathy? – Immunoglobulin A [Berger’s Disease]https://igan.org/faq/
In certain instances, IgA nephropathy, a kidney disease characterized by inflammation of the glomeruli, can advance to end-stage renal disease (renal failure) if not adequately addressed. It is imperative for individuals with this condition to undergo routine monitoring and receive timely medical care to avert the risk of kidney failure. […] Some individuals experience complete remission, while others live normal lives with medications to treat their symptoms (such as high blood pressure meds, immunosuppressants, omega 3 fatty acids vitamin E supplements), yet others experience complete kidney failure (end-stage kidney disease) and require either dialysis or a kidney transplant depending on the severity. It is estimated that as many as half of those affected with IgAN will develop end-stage renal disease. This poses one large problem there arent enough kidneys and many patients wait on the transplant list for years.
- #16 IgA Nephropathy (Bergerâs Disease): Diet, Symptoms, and Morehttps://www.healthline.com/health/iga-nephropathy-bergers
Theres no cure for IgA nephropathy and no reliable way to tell how it will affect each person individually. For most people, the disease progresses slowly. […] Research from 2018 suggests having IgA nephropathy can decrease a persons lifespan by about 10 years. […] Roughly 20% of people with IgA nephropathy typically develop ESRD within 10 years of onset. Some signs may indicate an unfavorable prognosis, including: long-term hypertension, persistent blood in the urine, prolonged protein in the urine (above 1 gram/day), high serum creatinine level, widespread intestinal fibrosis of the kidneys. […] People with ESRD require dialysis treatment or a kidney transplant. A kidney transplant is typically successful, but IgA nephropathy can come back (recur) in the new kidney. […] About 20-40% of people will have symptom recurrence after a kidney transplant. Recurrence typically happens within 2.5 years after transplantation but can occur before or after that. […] Recent research has found that IgA nephropathy can decrease a persons lifespan by about 10 years. […] For many people, IgA nephropathy progresses slowly. Some people may also develop kidney failure and other life threatening complications.
- #17 What is IgA Nephropathy? – Immunoglobulin A [Berger’s Disease]https://igan.org/faq/
IgA nephropathy cannot be cured, but medications can help slow it down. […] The prognosis for individuals with IgA nephropathy varies depending on the severity of the condition, kidney biopsy findings, the amount of protein in the urine, response to treatment, and overall kidney function. With proper management and medical care, many individuals with IgA nephropathy can lead a relatively normal life. […] It is difficult to predict from initial clinical signs and biopsy results just which patients will have which course. Statistical analyses have yielding conflicting results, but some factors do seem linked to a poor prognosis. Unremittingly heavy proteinuria, uncontrolled hypertension, declining glomerular filtration rate, high serum creatinine, and being age 30 or older at the time of apparent onset of the disease are some of the factors tied to a poor prognosis. Severe lesions in the initial biopsy, especially evidence of sclerosis [scarring] or crescent formation are also ominous signs.
- #18 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/books/NBK538214/
Up to 50% of IgAN patients experience a benign course. Prognosis can be somewhat predictable, particularly based on the Oxford classification outlined in the Pathology section. Any of the MEST criteria (and crescents) serve as poor prognostic indicators. Isolated microscopic hematuria with mild proteinuria suggests a favorable prognosis, indicating a benign and asymptomatic disease course. Additionally, individuals of East Asian origin are significantly associated with an elevated risk of ESRD. […] Three risk factors highly associated with dialysis or death include: […] Proteinuria of 1 g/d or more […] Sustained hypertension […] Oxford classification MEST-C score (to which some algorithms also include a category for crescents). […] Patients with sustained proteinuria of 1 g/d or higher have a 46-fold increased risk of ESRD than those with less than 500 mg/d. About half of the patients with IgAN may obtain complete remission with treatment, whereas the remaining half may show some degree of ongoing disease.
- #19 What is IgA Nephropathy? – Immunoglobulin A [Berger’s Disease]https://igan.org/faq/
In certain instances, IgA nephropathy, a kidney disease characterized by inflammation of the glomeruli, can advance to end-stage renal disease (renal failure) if not adequately addressed. It is imperative for individuals with this condition to undergo routine monitoring and receive timely medical care to avert the risk of kidney failure. […] Some individuals experience complete remission, while others live normal lives with medications to treat their symptoms (such as high blood pressure meds, immunosuppressants, omega 3 fatty acids vitamin E supplements), yet others experience complete kidney failure (end-stage kidney disease) and require either dialysis or a kidney transplant depending on the severity. It is estimated that as many as half of those affected with IgAN will develop end-stage renal disease. This poses one large problem there arent enough kidneys and many patients wait on the transplant list for years.
- #20 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. […] 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. […] 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. […] Ask your doctor if you should cut back on protein. This may help slow IgA nephropathy from becoming worse and protect your kidneys.
- #21 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. […] 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. […] 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. […] Ask your doctor if you should cut back on protein. This may help slow IgA nephropathy from becoming worse and protect your kidneys.
- #22 IgA Nephropathy (Bergerâs Disease): Diet, Symptoms, and Morehttps://www.healthline.com/health/iga-nephropathy-bergers
Theres no cure for IgA nephropathy and no reliable way to tell how it will affect each person individually. For most people, the disease progresses slowly. […] Research from 2018 suggests having IgA nephropathy can decrease a persons lifespan by about 10 years. […] Roughly 20% of people with IgA nephropathy typically develop ESRD within 10 years of onset. Some signs may indicate an unfavorable prognosis, including: long-term hypertension, persistent blood in the urine, prolonged protein in the urine (above 1 gram/day), high serum creatinine level, widespread intestinal fibrosis of the kidneys. […] People with ESRD require dialysis treatment or a kidney transplant. A kidney transplant is typically successful, but IgA nephropathy can come back (recur) in the new kidney. […] About 20-40% of people will have symptom recurrence after a kidney transplant. Recurrence typically happens within 2.5 years after transplantation but can occur before or after that. […] Recent research has found that IgA nephropathy can decrease a persons lifespan by about 10 years. […] For many people, IgA nephropathy progresses slowly. Some people may also develop kidney failure and other life threatening complications.
- #23 Recurrence of IgA nephropathy after kidney transplantation: experience from the Swiss transplant cohort study | BMC Nephrology | Full Texthttps://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-022-02802-x
Recurrence of IgA nephropathy (IgAN) after kidney transplantation occurs in about 30% of patients. The relevance of recurrence for the long-term graft survival is expected to increase, since graft survival continues to improve. […] Our study showed a recurrence risk of 28% at 11 years after transplantation, which is consistent with previous literature. However, the predictive value of known biomarkers, such as serum Gd-IgA1 and IgA-IgG IC, for IgAN recurrence could not be confirmed. […] The risk for IgAN recurrence was 27.7% (95%-CI: 20.6-35.3%) after 11 years. […] The rate of graft failure in patients with recurrence was 2.8-fold higher than in patients without recurrence. […] In a univariable and multivariable analysis, serum concentration of total IgA, total IgG, Gd-IgA1 and IgA-IgG IC did not predict the recurrence of IgA nephropathy.
- #24 IgA Nephropathy (Bergerâs Disease): Diet, Symptoms, and Morehttps://www.healthline.com/health/iga-nephropathy-bergers
Theres no cure for IgA nephropathy and no reliable way to tell how it will affect each person individually. For most people, the disease progresses slowly. […] Research from 2018 suggests having IgA nephropathy can decrease a persons lifespan by about 10 years. […] Roughly 20% of people with IgA nephropathy typically develop ESRD within 10 years of onset. Some signs may indicate an unfavorable prognosis, including: long-term hypertension, persistent blood in the urine, prolonged protein in the urine (above 1 gram/day), high serum creatinine level, widespread intestinal fibrosis of the kidneys. […] People with ESRD require dialysis treatment or a kidney transplant. A kidney transplant is typically successful, but IgA nephropathy can come back (recur) in the new kidney. […] About 20-40% of people will have symptom recurrence after a kidney transplant. Recurrence typically happens within 2.5 years after transplantation but can occur before or after that. […] Recent research has found that IgA nephropathy can decrease a persons lifespan by about 10 years. […] For many people, IgA nephropathy progresses slowly. Some people may also develop kidney failure and other life threatening complications.
- #25 Recurrence of IgA nephropathy after kidney transplantation: experience from the Swiss transplant cohort study | BMC Nephrology | Full Texthttps://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-022-02802-x
Recurrence of IgA nephropathy (IgAN) after kidney transplantation occurs in about 30% of patients. The relevance of recurrence for the long-term graft survival is expected to increase, since graft survival continues to improve. […] Our study showed a recurrence risk of 28% at 11 years after transplantation, which is consistent with previous literature. However, the predictive value of known biomarkers, such as serum Gd-IgA1 and IgA-IgG IC, for IgAN recurrence could not be confirmed. […] The risk for IgAN recurrence was 27.7% (95%-CI: 20.6-35.3%) after 11 years. […] The rate of graft failure in patients with recurrence was 2.8-fold higher than in patients without recurrence. […] In a univariable and multivariable analysis, serum concentration of total IgA, total IgG, Gd-IgA1 and IgA-IgG IC did not predict the recurrence of IgA nephropathy.
- #26 Recurrence of IgA nephropathy after kidney transplantation: experience from the Swiss transplant cohort study | BMC Nephrology | Full Texthttps://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-022-02802-x
The incidence of graft loss in our cohort was however lower than in the comparable previous studies, which might be explained by the prevalent use of prednisone and RAAS blockade. […] The results of 3 studies showed that there are so far no reliable clinical parameters or biomarkers to predict the recurrence of IgAN after transplantation.
- #27 Recurrence of IgA nephropathy after kidney transplantation: experience from the Swiss transplant cohort study | BMC Nephrology | Full Texthttps://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-022-02802-x
The incidence of graft loss in our cohort was however lower than in the comparable previous studies, which might be explained by the prevalent use of prednisone and RAAS blockade. […] The results of 3 studies showed that there are so far no reliable clinical parameters or biomarkers to predict the recurrence of IgAN after transplantation.
- #28 IgA Nephropathy (Berger Disease) – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/books/NBK538214/
Up to 50% of IgAN patients experience a benign course. Prognosis can be somewhat predictable, particularly based on the Oxford classification outlined in the Pathology section. Any of the MEST criteria (and crescents) serve as poor prognostic indicators. Isolated microscopic hematuria with mild proteinuria suggests a favorable prognosis, indicating a benign and asymptomatic disease course. Additionally, individuals of East Asian origin are significantly associated with an elevated risk of ESRD. […] Three risk factors highly associated with dialysis or death include: […] Proteinuria of 1 g/d or more […] Sustained hypertension […] Oxford classification MEST-C score (to which some algorithms also include a category for crescents). […] Patients with sustained proteinuria of 1 g/d or higher have a 46-fold increased risk of ESRD than those with less than 500 mg/d. About half of the patients with IgAN may obtain complete remission with treatment, whereas the remaining half may show some degree of ongoing disease.
- #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. […] 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. […] 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. […] Ask your doctor if you should cut back on protein. This may help slow IgA nephropathy from becoming worse and protect your kidneys.