Holbrook Timothy N, Srinivasan Lavanya, Baker Stephen E
Internal Medicine, Baylor Scott & White All Saints Medical Center, Fort Worth, USA.
Pulmonology and Critical Care, Baylor Scott & White All Saints Medical Center, Fort Worth, USA.
Cureus. 2025 Apr 4;17(4):e81722. doi: 10.7759/cureus.81722. eCollection 2025 Apr.
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.
干燥综合征（SD）是一种主要以干燥症状为特征的全身性自身免疫性疾病。然而，肺部表现也可能出现，并且可能先于腺体受累。自发性气胸作为SD的首发表现极为罕见，此前尚无男性患者的相关报道。我们报告一例54岁男性患者，该患者反复出现自发性气胸。影像学检查显示为囊性肺病，组织病理学表现为淋巴样间质性肺炎和缩窄性细支气管炎。全面的自身免疫检查发现SSA - 52（Ro52）抗体呈阳性，尽管该患者没有干燥症状，但仍诊断为肺部SD。在接受双侧机器人辅助肺大疱切除术和胸膜固定术后，患者康复，并计划继续接受肺部和风湿病学监测。该病例突出了SD可能表现为以肺部为主的疾病而无典型干燥症状的可能性，这可能导致诊断延迟。在其他方面健康的患者中出现反复气胸和囊性肺病应促使考虑自身免疫病因，包括SD。
