Uncommon cardiovascular and pulmonary complications prompt diagnosis of primary Sjögren's syndrome: a case report and literature review

Pulmonary involvement is the most common extra-glandular manifestation in primary Sjögren’s syndrome (pSS), adversely affecting patient prognosis. Nonspecific interstitial pneumonia, pulmonary embolism, and pulmonary hypertension are infrequently observed in patients with Sjögren’s syndrome, thereby complicating the diagnostic process. This case report presents a patient with a five-year history of pulmonary fibrosis, referred for worsening shortness of breath. The patient was later diagnosed with pSS, which included pulmonary involvement, pulmonary hypertension, and pulmonary thrombosis. This case highlights the need to recognize pulmonary manifestations of pSS and the complexities of managing patients, especially regarding complications like pulmonary hypertension and thrombosis. Pulmonary disease treatment in pSS should be guided by the extent and progression of pulmonary involvement, utilizing glucocorticoids or other immunosuppressive drugs based on experiences from other immune-mediated diseases.