Constrictive (Obliterative) Bronchiolitis as Presenting Manifestation of Connective Tissue Diseases

Antonella Arcadu, MD; Jay H. Ryu, MD


J Clin Rheumatol. 2020;26(5):176-180. 

In This Article


Subject Selection

We used a computer-assisted search of medical records to identify patients with CB encountered at Mayo Clinic, Rochester, MN, between January 1, 1997, and December 31, 2013. We searched for terms including constrictive bronchiolitis, obliterative bronchiolitis, bronchiolitis obliterans, or unspecified bronchiolitis. The study was approved by the Mayo Clinic Institutional Review Board (IRB # 14–000932).

Constrictive bronchiolitis was defined by the presence of (1) respiratory manifestations including dyspnea, wheezing, cough, and/or chest tightness; (2) presence of mosaic attenuation pattern with signs of air trapping on expiratory images on HRCT (Figure); (3) absence of parenchymal findings on HRCT to suggest interstitial lung disease, including diffuse micronodules or patchy ground-glass opacities. One patient (2%) did not exhibit air trapping on HRCT but had histopathologic confirmation of CB on surgical lung biopsy. We excluded patients with CB who were recipients of solid organ or hematopoietic stem cell transplant or carried a known diagnosis of CTD or other known causes, including inhalational injuries and respiratory infections, at initial presentation.


A and B, High-resolution computed tomography of the chest demonstrating mosaic attenuation (A) and air-trapping on expiratory view (B).

Pulmonary function testing (PFT) was performed with body plethysmography and single-breath diffusing capacity for carbon monoxide using the American Thoracic Society/European Respiratory Society standards.[6–9] The presence of obstructive/restrictive pattern was determined using the same guidelines; positive bronchodilator response was defined by an increase in baseline forced expiratory volume in the first second (FEV1) of 12% or 200 mL.

The diagnosis of specific CTD was confirmed by a rheumatologist, according to the criteria of the American College of Rheumatology[10] and based on clinical manifestations and associated laboratory results.

Data Collection

Medical records were abstracted for clinical data including age, gender, smoking history, symptoms, laboratory and pulmonary function results, radiologic findings, lung biopsy results, treatments, and follow-up including vital status. Laboratory testing data were abstracted for autoimmune serologic results including rheumatoid factor, anti-cyclic citrullinated peptide (CCP) antibodies, antinuclear antibodies, and antibodies to extractable nuclear antigens, including SS-A, SS-B, Jo1, anti-scl70, and anti-double-stranded DNA.

All chest radiographs and computed tomography scans were reviewed to address the presence of hyperinflation, reticular opacities, mosaic attenuation pattern, air trapping, bronchiectasis, centrilobular nodules, bronchial wall thickening, and ground-glass opacities. Therapeutic management including data on the use of oral corticosteroids, inhaled corticosteroids/bronchodilators, macrolides, steroid-sparing immunosuppressive agents, and oxygen therapy were collected. Follow-up data were reviewed to determine whether the patients received a CTD diagnosis during the subsequent clinical course. Vital status was obtained using the US Social Security Death Index (search date, June 30, 2016).

Statistical Analysis

Clinical data were expressed as median (range) or mean (SD) for quantitative variables; frequencies and percentages were used for the qualitative variables. Results of PFT and diffusing capacity for carbon monoxide were expressed as a percentage of normal predicted values. χ 2 and Fisher exact tests were used for comparing categorical data, with t test and Mann-Whitney U test for continuous data. p < 0.05 was considered statistically significant. Time-to-event variable (overall survival) was calculated from the date of diagnosis to date of death or censored (months). Statistical analysis was performed using JMP Statistics Software (version 10.0; SAS Institute Inc., Cary, NC).