Tracheobronchial stenosis in patients with granulomatosis with polyangiitis: histopathological findings and long-term outcome.

Abstract

Objectives: Tracheobronchial stenosis (TBS) occurs in a minority of patients with granulomatosis with polyangiitis (GPA). We aimed to i) review the literature on histopathological findings in patients with GPA and TBS, and ii) describe histopathological findings and long-term outcomes in a local cohort.

Methods: PubMed was searched for articles including biopsy findings in patients with GPA and TBS until May 2023. Our local cohort included patients diagnosed with GPA and TBS during 1991-2022. Demographic and clinical characteristics, number of biopsies, histopathological findings (GPA-characteristic versus non-specific) and number of endoscopic dilations during follow-up were recorded.

Results: In the reviewed literature, 203 patients were identified; 128 (63%) had a total of 296 biopsies; 64 (22%) with GPA-characteristic findings. The local cohort comprised 27 patients, the median (range) age at GPA diagnosis was 31 (14-76) years, the follow-up time was 17 (0-31) years. TBS occurred 2 (0-21) years after the GPA diagnosis. In the local cohort, 67 biopsies from 26 patients were available. GPA-characteristic findings were seen in 15 (22%) of these. Twenty-three patients underwent an endoscopic dilation during follow-up, and the number of dilations was 5 (1-100). Eighteen patients had ≥2 dilations; the interval between dilations was 23 (2-103) months.

Conclusions: The majority of patients with GPA and TBS experience recurring airway stenoses. GPA-characteristic findings are seen in approximately one in five tracheobronchial biopsies obtained from GPA patients with TBS. A biopsy could be of clinical value in the diagnostic process or to exclude other causes of stenosis.