Sjögren's Syndrome in Progressive Systemic Sclerosis

Abstract

Consecutive patients (35) with progressive systemic sclerosis were prospectively evaluated for evidence of Sjogren''s syndrome. Six of the 35 (17%) were judged to have the disorder. This is a higher prevalence than in most reports, but much lower than that recently reported by Alarcon-Segovia and associates. An additional 17 of the 35 patients (48%) had significant fibrosis in the absence of sufficient mononuclear cell infiltrates to confirm the diagnosis of Sjogren''s syndrome. This group had particularly aggressive scleroderma with serious visceral features, and 5 died after a short duration of illness. No significant abnormalities were found in biopsies from 6 patients with the mixed connective tissue disease syndrome, 5 with Raynaud''s phenomenon alone, or in 29 autopsy control subjects who had no evidence of connective tissue disease. Fibrosis in the absence of mononuclear infiltration in minor salivary glands of patients with progressive systemic sclerosis indicates a poor prognosis.