Constrictive bronchiolitis (CB) is a pulmonary symptom of primary Sjögren syndrome that is poorly understood (PSS). Through a retrospective cohort analysis, researchers wanted to elucidate the presenting clinical radiologic characteristics and clinical course of pSS-associated CB. From 2007 to 2015, they identified 11 patients with pSS and CB (characterized by a mosaic pattern with air trapping on computed tomography) who were seen at the hospital. The clinical aspects of the patient, laboratory data, radiologic findings, and clinical course were all examined.
The patients were predominantly women (91%), with a median age of 53 years (range, 31–76 years) at the time of the beginning of pulmonary symptom (dyspnea or cough). The majority (64%) were nonsmokers. The average time between pSS diagnosis and the emergence of pulmonary symptoms was 4.4 years; CB was the presenting sign of pSS in 4 individuals (36%). Chest radiographs were either normal (67%) or showed hyperinflation (33%). Pulmonary function tests revealed airflow obstruction in 7 individuals (63%), an isolated impairment in diffusing capacity in 2 patients (18%), and mixed physiology or normal findings in the other 2 patients. A surgical lung biopsy was conducted on two individuals, both of whom had CB. Glucocorticoids, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide were among the medications used by our patients. The outcomes of six individuals who had follow-up computed tomography and pulmonary function tests were steady or deteriorated.
Constrictive bronchiolitis is an uncommon extraglandular manifestation that might occasionally be the presenting symptom of pSS. This lung manifestation has a slow clinical course and does not react well to immunosuppressive treatment.
Reference:journals.lww.com/jclinrheum/Abstract/2019/03000/Constrictive_Bronchiolitis_in_Patients_With.4.aspx
