scleroderma - SONIKAPURI SCLERODERMA

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SONIKA PURI
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SCLERODERMA Chronic multisystem disorder with annual incidence of 1 2/100,000 Peak age of onset is 30 50yrs, F >> M TWO SUBSETS: Limited Cutaneous SS (Lcss) hands/face/feet and forearm Diffuse cutanous SS (Dcss) extensive skin involvement including above elbows and knees and involving the trunk. a/w PAH and renal failure; upto SSc patients with PAH have egft < 60ml/min
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REPORTED RENAL MANIFESTATIONS Scleroderma renal crisis Normotensive Scleroderma renal crisis Myeloperoxidase –ANCA associated GN and vasculitis Penicillamine associated renal disease Anti phospholipid associated nephropathy Microalbuminuria and proteinuria Renal Manifestations in scleroderma, Shanmugam International Journal of Rheum,2010
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Anca associated GN More common in pts with LcSS Subacute presentation with progressive renal failure, mild hypertension and proteinuria. Postulated that scleroderma vasculopathy exacerbates interaction of ANCA with endothelium near vascular pole with neutrophil activation in glomerulus. Steroid responsive, min. response to ACE I Biopsy is often rrequired to exclude scleroderma renal crisis Renal Manifestations in scleroderma, Shanmugam International Journal of Rheum,2010
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Penicillamine induced RF Rarely used now however historically used in treatment of Wilsons, cystinuria, RA,PBC 20% of pts develop membranous nephropathy with proteinuria. Resolve with cessation of drug. Severe cases would require plasmapharesis, steroids and immunosuppression. Renal Manifestations in scleroderma, Shanmugam International Journal of Rheum,2010
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Anti phospholipid antibody mediated nephropathy Frequency of Apla in scleroderma is no greater than that seen in general population. 56% of SSc pts were positive for aPL antibody based on single antibody measurement of anticardiolipin (ACL ) or Beta 2 glycoprotein ab of IgM/IgG. IgG ACL was a/w elevated s.cystatin C a/w development of SRC (21% of patient with apl +/none if neg levels) Wielosz et al. –Clinical Rheumatolgy 2009
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(free radical process ) 8 epiprostaglandin F2alpha(vasoconstrictor)
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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scleroderma - SONIKAPURI SCLERODERMA

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