Recurrent Pneumothoraces in a Patient With Anti-Sjögren’s Syndrome A Antibody-Positive Cystic Lung Disease: An Atypical Presentation of Sjögren’s Disease

TL;DR

A rare case of Sjögren’s disease presented as recurring lung issues in a man without typical dryness symptoms.

Contribution

This is the first reported case of male patient with pneumothorax as an initial presentation of anti-SSA antibody-positive pulmonary Sjögren’s disease.

Findings

A 54-year-old male presented with recurrent spontaneous pneumothoraces and cystic lung disease.

Diagnosis of pulmonary Sjögren’s disease was made based on positive SSA-52 antibodies despite absence of sicca symptoms.

The case emphasizes the need to consider autoimmune causes in patients with unexplained cystic lung disease and pneumothorax.

Abstract

Sjögren’s disease (SD) is a systemic autoimmune disorder primarily characterized by sicca symptoms. However, pulmonary manifestations can occur and may precede glandular involvement. Spontaneous pneumothorax as an initial presentation of SD is exceedingly rare, with no prior reports in male patients. We report a case of a 54-year-old male who presented with recurrent, spontaneous pneumothoraces. Imaging revealed cystic lung disease, and histopathology demonstrated features of lymphoid interstitial pneumonia and constrictive bronchiolitis. A comprehensive autoimmune workup was notable for positive SSA-52 (Ro52) antibodies, leading to a diagnosis of pulmonary SD despite the absence of sicca symptoms. Following bilateral robotic-assisted bleb resection and pleurodesis, the patient recovered and was scheduled for continued pulmonary and rheumatologic monitoring. This case highlights the potential for SD to manifest as lung-predominant disease without classic sicca symptoms, which may result in diagnostic delays. The presence of recurrent pneumothorax and cystic lung disease in an otherwise healthy patient should prompt consideration of autoimmune etiologies, including SD.

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