Triple-Negative Sjogren’s Syndrome and Recurrent Pneumothorax: An Uncommon Presentation of Autoimmune Disease

Sjogren's syndrome (SS) is a chronic exocrinopathy caused by lymphocytic infiltration and is associated with numerous manifestations and morbidities. We discuss a case of a 60-year-old female who presented to the Acute Medical Assessment Unit complaining of progressive shortness of breath for one month, not associated with chest pain or lower limb swelling. She also reported joint pain involving both wrists and proximal interphalangeal (PIP) joints, oral dryness, hair loss, and numerous tongue ulcerations. Blood workup revealed triple-negative SS, negative rheumatoid factor, anti-SSA and anti-SSB, a high erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer of 640. A diagnosis of SS was made. Nevertheless, her CT chest showed massive left-sided pneumothorax; subsequently, a chest tube was urgently inserted. The chest tube was removed two days later with complete resolution on chest X-ray (CXR). However, one week later, she presented with a recurrent pneumothorax that persisted and required surgical intervention that led to complete recovery afterward. Pneumothorax is an extremely rare but potentially unfavorable complication related to SS, with only two cases reported in the literature so far and usually associated with underlying lung pathology.