Sjögren's syndrome in progressive systemic sclerosis (scleroderma)

Abstract Sicca features of Sjogren's syndrome were investigated in 25 consecutive patients with progressive systemic sclerosis by means of clinical examination, Schirmer's tests, rose bengal staining tests, secretory parotid sialographies, scintillation scanning of salivary glands with 99-m Tc pertechnetate, radionuclide salivary excretion studies and lip biopsies for study of minor salivary glands. All 25 patients had at least one abnormal test and all but 3 patients had more than two abnormal tests. Pathologic findings in minor salivary glands included both lymphocytic infiltration and duct cell proliferation characteristic of Sjogren's syndrome, as well as collagen infiltration and disruption attributable to scleroderma. However, the finding of lacrimal and major salivary gland involvement indicates that Sjogren's syndrome does occur in the majority of patients with progressive systemic sclerosis. Because Sjogren's syndrome coexists almost exclusively with autoimmune disorders, our findings support the contention that progressive systemic sclerosis is related to autoimmunity.