4-21-09-Treatment of Idiopathic Membranous Nephropathy

4-21-09-Treatment of Idiopathic Membranous Nephropathy -...

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Treatment of Idiopathic Membranous Nephropathy Alexander Usorov, MD 4/21/09
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Idiopathic MN Leading cause of Nephrotic syndrome in white adults Incidence rate has remained constant over 3 decades As opposed to IgA Nephropathy and FSGS 2ry MN accounts for up to 1/3 rd cases and a/w autoimmune dz (SLE, Hep B/C, NSAIDs, neoplasms) Both IMN and 2ry MN have similar clinical presentations Therefore, r/o 2ry causes first
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Clinical Manifestations All ages (but rare in children) All races M>F 2:1 Peak incidence during 4 th and 5 th decade of life NS in 60-70% of pts at the time of presentation As per Dr. Cattran, about 60% of pts who present with subnephrotic-range proteinuria will progress to full NS within 1-2 years from presentation Microscopic hematuria in 30-40% Mostly normotensive pts (10-20 % with HTN) <20% of pts have significant renal insufficiency
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Natural History MN is a chronic dz with spontaneous remission and relapses The rule of 1/3 rd : Spontaneous remission within the first 2 years after presentation Lower rates of spontaneous remission if pts present with higher degree of proteinuria (ie 8g/24hr) 2 nd leading cause of ESRD among 1ry GNs Increased risk of thromboembolic and CV events Acute changes in proteinuria or rate of loss of renal fxn should raise the possibility of a superimposed event (RVT, crescentic GN, anti-GBM disease)
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Copyright ©2008 American Society of Nephrology Fervenza, F. C. et al. Clin J Am Soc Nephrol 2008;3:905-919 Figure 1. (A and B) Light microscopy showing segmental sclerosis (arrow; A, periodic acid Schiff stain) and pinholes (arrow) along the glomerular basement membranes (B, silver stain)
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(C and D) Immunofluorescence microscopy showing granular IgG (C) and C3 (D) along the capillary walls. (E) Electron microscopy showing subepithelial electron-dense deposits and basement membrane spikes (arrows) separating the deposits
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“Idiopathic” MN ? Ronco et al reviewed the rat model of Heymann nephritis and the role of alloimmunization as a new mechanism of MN in Seminars of Immunopathology 2007 Hypothesized that antibodies to neutral endopeptidases, aminopeptidase A, and dipeptidyl peptidase IV or DPPIV (all expressed in podocytes) are responsible for “Idiopathic” MN Salant’s presentation at ASN expanded on that idea Could not find more info on this presentation
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4-21-09-Treatment of Idiopathic Membranous Nephropathy -...

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