Arcadu, Antonella

Background Constrictive (obliterative) bronchiolitis (CB) is an uncommon form of obstructive lung disease that can occur in patients with identifiable causes including connective tissue diseases (CTDs) as a form of lung involvement. We explored whether CB can be the presenting manifestation of CTD. Methods We identified 44 patients with cryptogenic CB and examined the presenting clinical, laboratory, and radiologic features, as well as their clinical course. Results The mean age at presentation was 60.5 (SD, 13.8) years and included 38 women (86%); 32 (73%) were never smokers. All patients presented for evaluation of dyspnea, commonly associated with cough. An obstructive pattern on pulmonary function testing was demonstrated in 86% of patients. On chest high-resolution computed tomography, nearly all patients manifested a mosaic attenuation pattern with air trapping on expiratory views, characteristic of CB. Bronchoscopic lung biopsy (n = 10) was usually nondiagnostic (90%), whereas all 5 surgical lung biopsies yielded evidence of CB. Serologic testing for CTD was positive in 19 patients (43%) and most commonly included antinuclear antibody, rheumatoid factor, and anti–cyclic citrullinated antibodies. Seven of these patients with positive serologic results were eventually diagnosed to have CTD. Connective tissue diseases included rheumatoid arthritis in 4 patients, Sjögren syndrome in 2, and undifferentiated CTD in 1 patient. Conclusions Nearly one-half of patients with cryptogenic CB manifest positive CTD serology, and some of these patients have CTD not previously diagnosed. These results suggest that CB can be the presenting manifestation of a CTD.

ABSTRACT Background and objectiveAcute respiratory failure (ARF) in patients with interstitial lung disease (ILD) is associated with significant morbidity and mortality. Recommended assessment for acute exacerbation (AE) of ILD includes exclusion of secondary causes via fibreoptic bronchoscopy. Our aim is to assess the role of bronchoscopy during ARF‐ILD.MethodsConsecutive ILD patients (2002–2014) hospitalized with ARF who underwent bronchoscopy were included. Baseline demographics, underlying ILD diagnoses and presenting clinical features were reviewed. Characteristics of bronchoscopy including diagnostic findings, management and complications were collated.ResultsOne hundred and six patients accounted for 119 unique bronchoscopies. Sixteen (13%) were abnormal (12 infections and 4 haemorrhages). Baseline presenting clinical features did not differ between those with and without abnormal findings. About half were performed in an intensive care unit (ICU) (53%), with 25% of bronchoscopies performed in a general floor setting resulting in ICU transfer; 71% of whom resulted in immediate mechanical ventilation. Overall management of ARF in those with positive bronchoscopy findings was similar to those without, resulting in similar in‐hospital mortality.ConclusionBronchoscopy in the clinical assessment of ARF‐ILD is often performed with only a 13% yield in this large retrospective cohort. As management and in‐hospital mortality were similar, routine diagnostic bronchoscopy in ARF‐ILD should be further studied given its low yield and high procedural risk.