Abstract

Adenosine deaminase (ADA) activity in serum is mainly derived from T lymphocytes. Our purpose was to clarify the significance of ADA activity in systemic sclerosis (PSS) and related disorders. ADA activity was determined with an enzymatic method in 34 patients with PSS, 4 with mixed connective tissue disease (MCTD), 6 with dermatomyositis (DM), 11 with localized scleroderma (LS), and 13 with other collagen diseases. Serum ADA activity was elevated over the mean (+2 standard deviations) of the control in 85% of the patients with PSS, all with MCTD, 83% of those with DM, and 82% of those with LS. The mean values in 10 PSS patients with anti-topoisomerase I antibodies, 14 patients with anti-RNP antibodies, 12 patients with anticentromere antibodies (ACAs), and 5 patients without antinuclear antibodies (ANAs) were 26.1, 24.9, 22.6, and 16.8 IU/L, respectively. In most cases, except for ACA-positive patients, serum ADA activity changed almost in parallel with ANA titers. These results support the notion that T cells are involved in the pathogenesis of PSS and related disorders.

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