Systemic scleroderma

Abstract: A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus erythematosus, polymyositis/dermatomyositis, and Raynaud's phenomenon was conducted in order to develop classification criteria for systemic sclerosis. Preliminary criteria are proposed namely, the finding of either the sole major criterion, i.e., proximal scleroderma, or two or more of the minor criteria, i.e., 1) sclerodactyly, 2) digital pitting scars of fingertips or loss of substance of the distal finger pad, and 3) bilateral basilar pulmonary fibrosis. When applied to the case and comparison patients included in this study, these proposed criteria had a 97% sensitivity for definite systemic sclerosis and 98% specificity.

Abstract: Background Systemic sclerosis (scleroderma) is characterized by immunologic abnormalities, injury of endothelial cells, and tissue fibrosis. Abnormal oxidative stress has been documented in scleroderma and linked to fibroblast activation. Since platelet-derived growth factor (PDGF) stimulates the production of reactive oxygen species (ROS) and since IgG from patients with scleroderma reacts with human fibroblasts, we tested the hypothesis that patients with scleroderma have serum autoantibodies that stimulate the PDGF receptor (PDGFR), activating collagen-gene expression. Methods We analyzed serum from 46 patients with scleroderma and 75 controls, including patients with other autoimmune diseases, for stimulatory autoantibodies to PDGFR by measuring the production of ROS produced by the incubation of purified IgG with mouse-embryo fibroblasts carrying inactive copies of PDGFR α or β chains or the same cells expressing PDGFR α or β. Generation of ROS was assayed with and without specific PDGFR inhibitors. ...

Abstract: Scleroderma was described centuries ago, and yet today the physician has little to offer other than the diagnosis. The etiology remains obscure; the course is unpredictable, and therapy is of little benefit. The term "scleroderma" is limited, for literally it means merely "hard skin." Under this label are included disease processes varying from the sclerosis of a bit of skin to overwhelming systemic reactions terminating in rapid death. While innumerable case reports and reviews of scleroderma have appeared in the literature, we believed that a longterm clinical study, unique in the extremely large number of patients observed, would add to our knowledge of a poorly understood entity. Classification The classification of scleroderma used in the Section of Dermatology at the Mayo Clinic is as follows: Localized Morphea Generalized morphea Linear scleroderma En coup de sabre Hemiatrophy Systemic Acrosclerosis Diffuse scleroderma In this paper we will be concerned only with the