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→Scleroderma
=Polymyositis and Dermatomyositis=
=Polymyositis and Dermatomyositis=
=Scleroderma=
=Scleroderma=
*H&P: Women ages 30-65. Skin thickening on the face and extremities.
*Dx: CREST syndrome includes Calcinosis, Raynaud's phenomenon, esophageal dysmotility (GERD), sclerodactly, and telangiectasias. It involves the distal extremities and face only, is slowly progressive, has positive anti-centromere antibodies, a fair prognosis, and is associated with pulmonary hypertension. Diffuse scleroderma has generalized skin involvement, is rapidly progressive, has anti Scl-70 (topoisomerase I) antibodies, a poor prognosis, weaker association with calcinosis and telangiectasias, is associated with pulmonary interstitial fibrosis, and is more likely to trigger renal failure. '''Both can cause scleroderma renal crisis, which presents as acute malignant hypertension'''
*Tx: '''ACEI (captopril is preferred) is 1st line for scleroderma renal crisis, even though ACEI are generally avoided in acute renal failure. Also give IV nitroprusside if there is concomitant malignant HTN'''
=Missed Concepts=
=Missed Concepts=
*Hemochromatosis diagnosis and association with HCC
*Hemochromatosis diagnosis and association with HCC