Recurrent Pneumothoraces in a Patient With Anti-Sjögren’s Syndrome A Antibody-Positive Cystic Lung Disease: An Atypical Presentation of Sjögren’s Disease
Year: 2025
Type: article
Study design: Case Report
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.
Source: Cureus
DOI: 10.7759/cureus.81722
Authors: Timothy N Holbrook, Lavanya Srinivasan, Stephen E Baker
Institution: Baylor Scott & White All Saints Medical Center
Language: English
Subfield: Physiology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4409152177