Primary Sjogren’s Syndrome Presenting as Autoimmune Cytopenia

Abstract

Sjogren’s syndrome (SS) is a chronic systemic autoimmune disease, characterized by lymphocytic infiltration of lacrimal and salivary glands. Although extra glandular manifestations are uncommon, they can occur with the musculoskeletal, renal, pulmonary and hematological disease. We report the case of a 35-year-old woman presented to us with persistent unexplained bicytopenia (anemia and thrombocytopenia). Antinuclear antibody and direct Coombs test were positive. Anti-Ro/SSA and anti-La/SSB antibodies were also positive in high titer. The final diagnosis of primary SS with autoimmune cytopenia was made. Cytopenias in SS are rarely reported. Our case illustrates that clinically significant cytopenias may present as an extraglandular manifestation of SS.