IgA Nephropathy (Berger Disease)
- PMID: 30855802
- Bookshelf ID: NBK538214
IgA Nephropathy (Berger Disease)
Excerpt
Immunoglobulin A (IgA) nephropathy, or IgAN, is one of the leading causes of glomerulonephritis and renal failure. This disease is characterized by the deposition of IgA in the glomerular mesangium. Immune-mediated damage to the basement membrane results in hematuria, proteinuria, and renal insufficiency. Berger and Hinglais were the first to describe the disease in 1968, hence it is also known as Berger disease. Pathologically, a spectrum of glomerular lesions may be seen, yet the most frequently encountered change is mesangial proliferation with prominent IgA deposition.
Recent international collaborative efforts have significantly advanced our understanding of the immunopathogenesis of IgAN, leading to important discoveries. Furthermore, the establishment of multicenter networks has facilitated the holistic design and execution of clinical trials, offering crucial insights into immunotherapy for IgAN.
The clinical course typically progresses gradually, yet between 20% and 50% of affected patients develop end-stage renal disease (ESRD) within 20 years of diagnosis. Prevalence varies based on ethnicity, race, geography, and genetics, with Asian patients being more commonly and severely affected.
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Sections
- Continuing Education Activity
- Introduction
- Etiology
- Epidemiology
- Pathophysiology
- Histopathology
- History and Physical
- Evaluation
- Treatment / Management
- Differential Diagnosis
- Prognosis
- Complications
- Postoperative and Rehabilitation Care
- Deterrence and Patient Education
- Pearls and Other Issues
- Enhancing Healthcare Team Outcomes
- Review Questions
- References
References
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- Pattrapornpisut P, Avila-Casado C, Reich HN. IgA Nephropathy: Core Curriculum 2021. Am J Kidney Dis. 2021 Sep;78(3):429-441. - PubMed
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- Working Group of the International IgA Nephropathy Network and the Renal Pathology Society. Coppo R, Troyanov S, Camilla R, Hogg RJ, Cattran DC, Cook HT, Feehally J, Roberts IS, Amore A, Alpers CE, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D'Agati V, D'Amico G, Emancipator SN, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo AB, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Li LS, Li PK, Liu ZH, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H. The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults. Kidney Int. 2010 May;77(10):921-7. - PubMed
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- Shen PC, He LQ, Tang Y, Wang Q, Wang W, Li J. Clinicopathological characteristics and prognostic factors of asymptomatic IgA nephropathy. J Investig Med. 2010 Mar;58(3):560-5. - PubMed
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