Constrictive (obliterative) bronchiolitis as presenting manifestation of connective tissue disease

Abstract

Background and objectives: Several studies reported an association between constrictive bronchiolitis (CB) and connective tissue disease (CTD) but there are no studies evaluating whether CB can be the presenting clinical manifestation of an evolving CTD.

Methods: Retrospective analysis of 44 patients with initial diagnosis of cryptogenic CB to assess for evidence of CTD at presentation or follow-up. Diagnosis of CB was based on respiratory symptoms and characteristic HRCT findings.

Results: The mean age at diagnosis was 60.5 years (SD 13.8), 38 (86%) were women, and 32 (73%) never smokers. Most frequent symptoms were dyspnea (100%) and cough (61%). An obstructive pattern on PFT was demonstrated in 90%.

Most frequent HRCT findings were mosaic attenuation pattern (100%) and air trapping (97%). Surgical lung biopsy, performed in 6 (13%) patients, yielded evidence of CB in 5 (83%) of them.

Serology testing for CTD was positive in 19 (43%) patients, of which 8 had positive RF, 9 anti-CCP, 9 ANA, 3 ENA, 3 ANCA, and 1 anti-DS-DNA.

Clinical follow-up was available in 30 patients (68%) among whom 7 (23%) were subsequently diagnosed to CTD including rheumatoid arthritis in 4 patients, Sjögren's syndrome in 2, and undifferentiated CTD in 1. Median duration of follow-up was 43 months (range, 2-384).The onset of respiratory symptoms preceded the diagnosis of CTD by a median of 24 months (range, 6-36).

Conclusions: Nearly one-half of patients with cryptogenic CB manifest positive CTD serologies and some of these patients subsequently develop clinical evidence CTD not previously diagnosed, demonstrating that CB can be the presenting manifestation of an underlying CTD.