Sjögren syndrome associated with inflammatory muscle diseases

Abstract

Objectives: Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland function that may affect multiple organ systems. The association of SS with inflammatory myopathies (IM), a group of diseases characterized by chronic inflammation of striated muscle and skin has been infrequently described. Methods: We present two cases diagnosed with SS who developed IM. We have also conducted a review of the English literature to depict all available clinical evidence on the clinical association of SS with IM. Results: Two female patients diagnosed with SS developed polymyositis (PM) and inclusion body myositis (IBM) respectively. The literature review identified 24 cases with coexistence of the two autoimmune conditions (SS and IM). Twenty-two patients were females and two males. Eight patients were diagnosed with IBM, 15 were diagnosed with PM and 1 with dermatomyositis. All patients had biopsy proven IM. Conclusions: There is evidence of clinical association of primary SS and IM especially with IBM and PM. Patients with SS and symptoms of muscle weakness should be investigated for associated IM.