Constrictive Bronchiolitis in Patients With Primary Sjögren Syndrome

Area of Science:

• Pulmonary Medicine
• Rheumatology
• Autoimmune Diseases

Background:

• Constrictive bronchiolitis (CB) is an uncommon pulmonary complication associated with primary Sjögren syndrome (pSS).
• Understanding the clinicoradiologic features and disease course of pSS-associated CB is crucial for patient management.

Purpose of the Study:

• To investigate the presenting clinical and radiologic characteristics of constrictive bronchiolitis in patients with primary Sjögren syndrome.
• To analyze the clinical course and treatment outcomes of pSS-associated CB.

Main Methods:

• Retrospective cohort study of 11 patients diagnosed with pSS and CB between 2007 and 2015.
• Analysis of clinical data, laboratory results, imaging findings (CT scans), pulmonary function tests, and treatment responses.

Main Results:

• The study cohort comprised predominantly women (91%) with a median age of 53 years at symptom onset.
• Constrictive bronchiolitis was the initial manifestation of pSS in 36% of cases. Chest imaging often showed normal or hyperinflated lungs, while pulmonary function tests revealed airflow obstruction.
• Follow-up imaging and pulmonary function tests indicated stable or worsening disease, with limited response to varied immunosuppressive therapies.

Conclusions:

• Constrictive bronchiolitis represents a rare extraglandular manifestation of pSS, sometimes serving as its presenting feature.
• This pulmonary condition follows an indolent course and demonstrates poor responsiveness to immunosuppressive therapies, highlighting the need for further research into effective treatments.