IgA nephropathy overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]
Overview
IgA nephropathy (Berger’s disease) is considered the most common primary chronic glomerulonephritis.[1] IgA nephropathy is defined immune-histologically by mesangial deposits of IgA, often accompanied by less intense staining for IgM and/or IgG and C3, in the absence of a systemic disease [2]. IgAN has been differentiated from Henoch- Schönlein purpura (HSP), which is clearly a systemic illness with vasculitis.
The clinical syndrome of IgA nephropathy is often unpredictable, although classically it is recognized as a nephritic syndrome with a presentation of recurrent painless gross hematuria following a respiratory or gastrointestinal tract infection in a young male patient. Nonetheless, asymptomatic IgA nephropathy with microscopic hematuria is not uncommon.[3] Although not frequently performed, the definitive diagnosis to confirm the clinical suspicion of IgA nephropathy is kidney biopsy that not only carries diagnostic benefit, but also has prognostic implications.
IgA nephropathy is a progressive kidney disease that often leads to End Stage Renal Disease (ESRD) due to lack of specific treatments or therapies for this disease. IgA Nephropathy is diagnosed by electron microscopy of a kidney biopsy specimen showing immunological deposits of predominantly glycosylated but non-galactosed linked IgA1 in the mesangium of the kidney glomeruli. These IgA immune complexes deposit comprises of mainly glycosylated immunoglobulin A1 (IgA) with some complement C3 and immunoglobulins G/M (IgG/ IgM).
References
- ↑ D'Amico G (1987). "The commonest glomerulonephritis in the world: IgA nephropathy". Q J Med. 64 (245): 709–27. PMID 3329736.
- ↑ Julian BA, Waldo FB, Rifai A, Mestecky J (1988). "IgA nephropathy, the most common glomerulonephritis worldwide. A neglected disease in the United States?". Am J Med. 84 (1): 129–32. PMID 3337116.
- ↑ Donadio JV, Grande JP (2002). "IgA nephropathy". N Engl J Med. 347 (10): 738–48. doi:10.1056/NEJMra020109. PMID 12213946.