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IgA Nephropathy

Epidemiology. Most common lesion causing primary glomerulonephritis in the developed worldPeak incidence in 2nd and 3rd decades of life2:1 male to female predominanceMost common in Asians and Caucasians50% of new GN in Japan40% of Japanese ESRD pts10% of new US GN pts30% of new Western Europe

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IgA Nephropathy

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    1. IgA Nephropathy UNC Medicine Morning Report Sally Ravanos, MD December 5, 2008

    2. Epidemiology Most common lesion causing primary glomerulonephritis in the developed world Peak incidence in 2nd and 3rd decades of life 2:1 male to female predominance Most common in Asians and Caucasians 50% of new GN in Japan 40% of Japanese ESRD pts 10% of new US GN pts 30% of new Western European GN pts

    3. Pathogenesis Mesangial deposition of polymeric IgA of IgA1 subclass IgG and complement deposition are also common Four elements contributing to severity/course: Synthesis/release of IgA immune complexes with characteristics that favor mesangial deposition Ability of reticuloendothelial system to remove pathogenic IgA immune complexes or aggregates Mesangial cell affinity for IgA accumulation Inherent tendency of kidney to respond to injury with glomerulosclerosis and interstitial fibrosis

    4. Pathogenesis (contd) Increased plasma IgA alone isnt sufficient to cause disease Anionic IgA, overrepresentation of lambda light chains Abnormal sialylation and galactosylation in hinge regions of IgA

    5. Clinical Features Three different presentations One or recurrent episodes of gross hematuria usually following a URI (synpharyngitic hematuria) 40-50% Microscopic hematuria and mild proteinuria incidentally detected on routine examination 30-40% Nephrotic syndrome or acute rapidly progressive glomerulonephritis (edema, hypertension, renal insufficiency) <10%

    6. Diagnosis Generally based on clinical history and laboratory data Diagnosis can only be confirmed with renal biopsy Biopsy only pursued if signs of more progressive disease are present such as protein excretion >0.5-1g/day, elevated plasma Cr, and hypertension

    7. Pathology Light Microscopy

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