Crescentic_IgA_Nephropathy[1][1]

Crescentic_IgA_Nephropathy[1][1] - Nephrology Grand Rounds...

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Nephrology Grand Rounds March 16 th , 2010 Aditya Mattoo
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Outline Background Epidemiology Clinical Presentation Prognosis Pathogenesis Histology Treatment Recurrence in Transplant
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Background First described by Berger et al in 1968. Characterized by predominant IgA deposition in the glomerular mesangium. Most common form of primary glomerulonephritis around the world. Berger et al. J Urol Nephrol, 74:p694, 1968.
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Demographics Clinical onset in second and third decades of life. 80% of patients are between the ages of 16-35 years at the time of diagnosis. Male predominance of 2:1 in Japan to as high as 6:1 in northern Europe and US. Asians and Caucasians more prone to develop IgAN than people of African descent.
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Demographics There appears to be a familial clustering of IgAN which shows strong family predisposition in about 10% of cases. In the U.S., regions in Kentucky, Alabama and other parts of the Southeast exhibit a higher incidence of IgAN. In other parts of the world, familial clustering of IgAN seems to be more common in Southern France and Italy. Many genetic studies are underway, trying to establish common susceptibility genes in familial IgA.
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IgAN Nationwide
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Epidemiology IgAN prevalence as a percentage of primary GN: In Japan, 50% of new cases of GN are IgAN (causing 40% of all ESRD). 30% of new GN cases in Western Europe and Australia. 10% in general US population (exception Native Americans from New Mexico with prevalence of rate of 38%) Crescentic IgAN (CIgAN) is seen in approximately 7% of patients with IgAN. However, a study conducted by Shouno et al reported that by increasing the number of serial sections examined for any single biopsy specimen from the standard 20 to 100 sections, the finding of a segmental necrotizing lesion increases to 30%. Donadio et al. NEJM, 347:p738, 2002. Shouno et al. Acta Pathol Jpn, 43:p723, 1993.
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Clinical Presentation IgAN is highly variable, both clinically and pathologically. Clinical features range from asymptomatic hematuria to RPGN. Classic flare includes painless hematuria concurrent with the onset of viral illness (e.g. pharyngitis, gastroenteritis, etc.)
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Clinical Presentation Approximately 40-50% of patients present with one or recurrent episodes of gross hematuria. Another 30-40% have microscopic hematuria and usually mild proteinuria incidentally detected on a routine examination. Gross hematuria will eventually occur in 20- 25% of these patients.
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Clinical Presentation Of the patients with gross hematuria secondary to IgAN, up to 40% will develop transient renal failure. Less than 10% present with either nephrotic syndrome or RPGN (characterized by edema, hypertension, and renal dysfunction).
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Clinical Presentation: Crescentic vs Non-crescentic Crescentic IgAN Non–crescentic IgAN P value Number of patients 35 229 Sex male/female 26/9 147/82 NS Mean age years 33 ± 12.5
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Crescentic_IgA_Nephropathy[1][1] - Nephrology Grand Rounds...

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