Pulmonary Manifestations of Sjögren's Disease

Louise Byrne; Cormac McCarthy; Aurelie Fabre; Nishant Gupta

doi:10.1055/s-0044-1785675

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2024; 45(03): 397-410
DOI: 10.1055/s-0044-1785675

Review Article

Pulmonary Manifestations of Sjögren's Disease

Authors

Louise Byrne

¹Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland
Cormac McCarthy

¹Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland

²School of Medicine, University College Dublin, Dublin, Ireland
Aurelie Fabre

²School of Medicine, University College Dublin, Dublin, Ireland

³Department of Histopathology, St. Vincent's University Hospital, Dublin, Ireland
Nishant Gupta

⁴Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, Cincinnati, Ohio

Abstract

Sjögren's disease (SjD) is a chronic, progressive autoimmune condition of exocrine and extraglandular tissues. It can present with isolated disease characterized by lymphocytic infiltration of salivary or lacrimal glands, but in approximately one-third of the patients, lymphocytic infiltration extends beyond exocrine glands to involve extraglandular organs such as the lungs. Pulmonary complications have been reported to occur between 9 and 27% of patients with SjD across studies. Respiratory manifestations occur on a spectrum of severity and include airways disease, interstitial lung disease, cystic lung disease, and lymphoma. Lung involvement can greatly affect patients' quality of life, has a major impact on the overall prognosis, and frequently leads to alteration in the treatment plans, highlighting the importance of maintaining a high index of clinical suspicion and taking appropriate steps to facilitate early recognition and intervention.

Keywords

SjD - ILD - connective tissue disease - cystic lung disease

Full Text

References

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