ALTERED T-CELL SUBPOPULATIONS AND LYMPHOCYTES EXPRESSING NATURAL-KILLER-CELL PHENOTYPES IN PATIENTS WITH PROGRESSIVE SYSTEMIC-SCLEROSIS

Scleroderma (progressive systemic sclerosis [PSS]) is known to be associated with abnormal T cell immunoregulation. In the present study, we evaluated lymphocyte phenotypes in patients with PSS and normal control subjects by flow cytometry and monoclonal antibodies for total T (CD3), T suppressor (CD8), T helper (CD4), T helper-inducer (CDw29), T suppressor-inducer (CD45R), human leukocyte antigen, DR+B (CD19), DR+T, and natural killer subsets, HNK-1 (CD57) and NKH-1 (CD56) cells. Patients with PSS compared to normal subjects had significantly lower percentages of CD3+ (p < 0.005) and CD8+ (p < 0.05) (similar to several patients with rheumatoid arthritis also evaluated), as well as CD45R (p < 0.05), T+ DR+ (p < 0.05), and NKH-1 (CD56) (p < 0.0005) cells. Patients with PSS late-limited or generalized disease had lower percentages of CD8+, CD19, NKH-1 +, and CDw29, but higher percentages of CD4+, HNK-1, and CD45R cells compared to patients with early stage disease, but these results were not statistically significant. These unique alterations in patients with PSS may prove to be useful in monitoring the stage of disease activity for therapy and further define immunologic defects.