Primitive Sjögren’s Syndrome Revealed by Bilateral Pleurisy in a Teenager: An Unexpected Case

Abstract

Primary Sjögren's syndrome (PSS) is a rare autoimmune disease in children and adolescents. The initial pleural manifestations are exceptional. We report the case of a 16-year-old adolescent hospitalized for bilateral chest pain with pleurisy. The initial diagnosis of tuberculosis was suspected due to the clinical context and local endemicity, but microbiological and histological examinations were negative. The presence of anti-Sjögren's syndrome type A (anti-SSA) antibodies and a salivary gland biopsy confirming chronic sialadenitis allowed for the diagnosis of PSS. Management by corticosteroid therapy resulted in a significant clinical improvement. Although rare, PSS should be considered in the differential diagnosis of lymphocytic pleurisy, especially when anti-infective treatments fail. Pleuropulmonary manifestations are poorly described and can delay the diagnosis. This report highlights the diversity of presentations of PSS and the importance of a multidisciplinary approach for early diagnosis, allowing for improved prognosis of systemic complications.