Primary Sjogren’s Syndrome Presenting As Pleuritis and Pleural Effusion

Sjogren's syndrome is an autoimmune condition characterized by infiltration of exocrine glands but, in rare cases, can have extraglandular involvement with pleural effusion being an exceedingly rare form. Here we present a case of Sjogren's pleuritis resulting in pleural effusion, a rare initial presentation for Sjogren's syndrome. A woman in her 20s presented to the emergency department after a recent hospitalization for pneumonia, pleural effusion, and pulmonary embolism. She presented to the hospital after discharge with pleuritic chest pain, dyspnea, and a productive cough. She was subsequently found to have a large left pleural effusion that necessitated left video-assisted thoracoscopic surgery with pleural biopsy and decortication of the lung. Infectious etiologies were ruled out during hospitalization. The patient's pleural fluid studies revealed lymphocytic predominance, and biopsy results were consistent with the autoimmune origin, more specifically, Sjogren's serositis. While Sjogren's syndrome is an autoimmune disorder most commonly affecting lacrimal and salivary gland function, it is important to consider extra-glandular involvement in patients with positive serologies and unexplained phenomena. This manuscript aims to provide information regarding diagnostic tools and criteria to arrive at a diagnosis and effectively treat pleural effusion secondary to Sjogren's serositis.