BACKGROUND: The etiopathogenesis of systemic sclerosis (SSc)--an autoimmunologicaldisease characterized by excess collagen and other connective tissue components in skin and internalorgans--remains unclear. Given the potential role of matrix enzymes, metalloproteinases, and their tissueinhibitors in pulmonary fibrosis, we assayed the serum concentration of tissue inhibitor of metalloproteinases2 (TIMP-2) in patients with SSc and explored its possible correlation with the duration of Raynaud'sphenomenon, the intensity of sclerosis of the skin, and pulmonary lesions. MATERIAL/METHODS: We studied18 SSc patients, 8 with limited SSc and ten with diffuse. The degree of sclerosis of the skin was measuredusing the Total Skin Scale. Chest x-rays and spirometric examinations were also performed. The presenceand type of antinuclear antibodies (ANA) were determined by indirect immunofluorescence and double immunodiffusionin agar gelatin. Serum TIMP-2 concentration was measured by ELISA. RESULTS: The average serum TIMP-2concentration was not significantly higher in SSc patients than in controls. No statistically significantdifference was found in this respect between limited and diffuse SSc. Despite the lack of correlationbetween the duration of Raynaud's phenomenon, the degree of sclerosis of the skin, and the serum concentrationof TIMP-2, the latter concentration was higher in patients with restrictive pulmonary dysfunction inspirometric testing. CONCLUSIONS: Our research, though involving a small group of patients, points tothe probable role of TIMP-2 in the development of pulmonary fibrosis.

Keywords: Adolescent, Enzyme-Linked Immunosorbent Assay, Raynaud Disease, Research Support, Non-U.S. Gov, Respiratory Function Tests, Scleroderma, Systemic, Skin Diseases, Tissue Inhibitor of Metalloproteinase-2