Constrictive Bronchiolitis in Patients with Primary Sjögren Syndrome

Elizabeth C. Wight, Misbah Baqir, Jay H Ryu

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Abstract

Objective Constrictive bronchiolitis (CB) is a poorly understood pulmonary manifestation of primary Sjögren syndrome (pSS). We aimed to clarify the presenting clinicoradiologic features and clinical course of pSS-associated CB through a retrospective cohort study. Methods We retrospectively identified 11 patients with pSS and CB (defined by mosaic pattern with air trapping on computed tomography) encountered at our institution over 9 years from 2007 to 2015. Presenting clinical features, laboratory results, radiologic findings, and clinical course were analyzed. Results Our patients were mostly women (91%), with a median age 53 years (range, 31-76 years) at the time of pulmonary symptom (dyspnea or cough) onset. Most (64%) were nonsmokers. Average interval between diagnosis of pSS and the onset of pulmonary symptoms was 4.4 years; in 4 patients (36%), CB was the presenting manifestation of pSS. Chest radiographs were normal (67%) or demonstrated hyperinflation (33%). Pulmonary function testing demonstrated airflow obstruction in 7 patients (63%), and 2 patients (18%) had an isolated reduction in diffusing capacity, whereas the remaining 2 patients manifested mixed physiology or normal results. Surgical lung biopsy was performed in 2 patients and confirmed the diagnosis of CB in both. Treatment was variable among our patients included glucocorticoids, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide. Among 6 patients with follow-up computed tomography and pulmonary function, the results remained stable or worsened. Conclusions Constrictive bronchiolitis is a rare extraglandular manifestation and can sometimes be the presenting manifestation of pSS. This pulmonary manifestation is associated with an indolent clinical course and does not respond well to immunosuppressive therapy.

Original languageEnglish (US)
Pages (from-to)74-77
Number of pages4
JournalJournal of Clinical Rheumatology
Volume25
Issue number2
DOIs
StatePublished - Mar 1 2019

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Keywords

  • bronchiolitis obliterans
  • constrictive bronchiolitis
  • obstructive lung diseases
  • Sjögren syndrome

ASJC Scopus subject areas

  • Rheumatology

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