IgA nephropathy
| IgA nephropathy | |
|---|---|
 |
|
| Immunoglobulin A dimer | |
| Classification and external resources | |
| Specialty | urology |
| ICD-10 | N02.8 |
| ICD-9-CM | 583.9 |
| OMIM | 161950 |
| DiseasesDB | 1353 |
| MedlinePlus | 000466 |
| eMedicine | med/886 |
| MeSH | D005922 |
IgA nephropathy (IgAN), also known as IgA nephritis, Berger disease (/bɛərˈʒeɪ/) (and variations), or synpharyngitic glomerulonephritis, is a disease of the kidney (or nephropathy), specifically it is a form of glomerulonephritis or an inflammation of the glomeruli of the kidney.
IgA nephropathy is the most common glomerulonephritis worldwide. Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus. There are other diseases associated with glomerular IgA deposits, the most common being IgA vasculitis (formerly known as Henoch–Schönlein purpura [HSP]), which is considered by many to be a systemic form of IgA nephropathy. HSP presents with a characteristic purpuric skin rash, arthritis, and abdominal pain and occurs more commonly in young adults (16–35 years old). HSP is associated with a more benign prognosis than IgA nephropathy. In IgA nephropathy there is a slow progression to chronic kidney failure in 25–30% of cases during a period of 20 years.
The classic presentation (in 40–50% of the cases) is episodic hematuria, which usually starts within a day or two of a non-specific upper respiratory tract infection (hence synpharyngitic), as opposed to , which occurs some time (weeks) after initial infection. Less commonly gastrointestinal or urinary infection can be the inciting agent. All of these infections have in common the activation of mucosal defenses and hence IgA antibody production. Groin pain can also occur. The gross hematuria resolves after a few days, though microscopic hematuria may persist. These episodes occur on an irregular basis every few months and in most patients eventually subsides, although it can take many years. Renal function usually remains normal, though rarely, acute kidney failure may occur (see below). This presentation is more common in younger adults.
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