IgA nephropathy

Posted by e-Medical PPT
IgA nephropathy (also known as IgA nephritis,Berger's disease) is a form of glomerulonephritis (inflammation  of the glomeruli of the kidney). IgA nephropathy is the most common glomerulonephritis throughout the world.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 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 yrs old). HSP is associated with a more benign prognosis than IgA nephropathy. In IgA nephropathy there is a slow progression to chronic renal failure in 25-30% of cases during a period of 20 years.
The classic presentation  is episodic frank hematuria  which usually starts within a day or two of a non-specific upper respiratory tract infection as opposed to post-streptococcal glomerulonephritis which occurs some time  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. Loin 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 renal failure may occur.This presentation is more common in younger adults.
Very rarely (5% each), the presenting history is:
    * Nephrotic syndrome
    * Acute renal failure (either as a complication of the frank hematuria, when it usually recovers, or due to rapidly progressive glomerulonephritis which often leads to chronic renal failure)
    * Chronic renal failure (no previous symptoms, presents with anemia, hypertension and other symptoms of renal failure, in people who probably had longstanding undetected microscopic hematuria and/or proteinuria)
A kidney biopsy is necessary to confirm the diagnosis.The biopsy specimen shows proliferation of the mesangium, with IgA deposits on immunofluorescence and electron microscopy.
IgA nephropathy has a very variable course, ranging from a benign recurrent hematuria up to a rapid progression to chronic renal failure. Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. Also, IgA nephropathy recurs in transplants despite the use of ciclosporin, azathioprine or mycophenolate mofetil and steroids  in these patients.

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