Pleural Effusion as an Unusual Complication in a Woman with Primary Sjögren's Syndrome: A Case Report




Healy, Jack
Malik, Rehan


0000-0002-3600-4455 (Healy, Jack)

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BACKGROUND: Sjögren's syndrome (SS) is a chronic, systemic, autoimmune condition causing lymphocytic damage to exocrine glands. Immune-mediated dysfunction of the lacrimal and salivary glands leads to the classic symptoms of SS: dry eyes (xeropthalmia) and dry mouth (xerostomia). The combination of these two symptoms is also known as sicca syndrome. Sjögren's Syndrome is classified as either primary (occurring in isolation, without another diagnosed autoimmune condition) or secondary (occurring with a comorbid autoimmune disorder, such as systemic lupus erythematosus or rheumatoid arthritis). In addition to sicca symptoms, patients with Sjögren's Syndrome may have extra-glandular involvement in multiple organ systems. Lung manifestations most frequently involve the airways and interstitium. Inflammation usually spares the serosa (pericardium and pleura), and pleural effusion in this population is rare (< 1% reported incidence). CASE INFORMATION: A 79-year-old female with primary Sjögren's syndrome presented to the ED for chest pain. Her medical history also included COPD, anemia, and atrial fibrillation with previous cardioversion. She described the pain as sudden onset, bilateral, squeezing chest discomfort that awoke her from sleep earlier that morning. Her pain was exacerbated by lying down and inspiration, and non-responsive to over-the-counter analgesics. She endorsed dyspnea and vomiting but denied cough, diaphoresis, or pain radiating to her jaw or back. In the ED, she was tachypneic but otherwise in stable condition. She was admitted for further evaluation and treatment. Workup for ACS, heart failure, COPD exacerbation, pneumonia, pneumothorax, flu, and Covid-19 were negative. CT and X-ray suggested new bilateral pleural effusions with no evidence of embolism. Thoracentesis drained 870 mL of fluid and confirmed the diagnosis. Analysis of the collected sample showed weakly exudative pleural fluid with a protein ratio of 0.58, and cytology negative for malignancy. After eight days of supportive care (IV furosemide and respiratory therapy) she was discharged in stable condition, with evidence of resolving pleural effusion on imaging. CONCLUSIONS: The pulmonary structures typically involved in Sjögren's syndrome are the airways and interstitium, not the pleura. The most commonly seen pulmonary compilations in SS are pneumonitis, bronchiolitis, and bronchiectasis. As such, this case describes an unusual complication of pleural effusion in the setting of primary Sjogren's Syndrome. The mechanism underlying pleural effusion in SS in unknown. One theory suggests that CD4+ T cells may activate B lymphocytes, which produce autoantibodies against lung tissue, causing buildup of fluid in the pleural space.