Sjögren’s Syndrome Presenting with Multiple Cystic Lung Lesions: A Rare Pulmonary Manifestation

Abstract

Aims: This case report aims to describe a rare presentation of primary Sjögren’s syndrome (pSS) manifesting as diffuse thin-walled cystic lung disease, emphasizing the need for autoimmune evaluation in such cases to avoid misdiagnosis and enable multidisciplinary management. Case Presentation: A 60-year-old female farmer presented with progressive dyspnoea, dry cough, fever and longstanding sicca symptoms, along with a prior history of myocardial infarction. Examination revealed pallor, respiratory distress and bilateral basal crackles. High-resolution computed tomography (HRCT) demonstrated diffuse thin-walled pulmonary cysts with associated interstitial changes and bronchiectasis. Laboratory evaluation showed normocytic anaemia with elevated ESR and CRP. Autoimmune testing revealed positive antinuclear antibodies (ANA) and anti-Ro/SSA antibodies, while Schirmer’s test confirmed ocular dryness, supporting the diagnosis of pSS with pulmonary involvement consistent with lymphocytic interstitial pneumonia. Discussion: Cystic lung disease in pSS is thought to result from lymphocytic infiltration causing small airway obstruction and cyst formation. Radiologically, these thin-walled cysts associated with interstitial changes may mimic other cystic lung disorders such as pulmonary Langerhans cell histiocytosis and lymphangioleiomyomatosis, posing diagnostic challenges. Conclusion: Diffuse thin-walled cystic lung disease may represent an uncommon but important pulmonary manifestation of primary Sjögren’s syndrome. Recognition of this pattern is essential to prompt autoimmune evaluation, avoid misdiagnosis and enable timely multidisciplinary management.