Pulmonary involvement in inflammatory bowel diseases: report of a national cohort over the past 5 years

Background Chronic inflammatory bowel disease (IBD) is a common illness, with a number of cases constantly rising. Pulmonary involvement is rarely described in children with IBD. This national survey aims to describe the clinical, biological and therapeutic data of IBD children with pulmonary involvement followed from January 2017 to April 2023 in a rare respiratory diseases centre. Methods This was a retrospective, observational, descriptive, multicentre study conducted within the French RespiRare network. Socio-demographic characteristics, previous events, clinical and paraclinical data, treatment and evolution were collected via an anonymous secure questionnaire. Results Seven IBD children with pulmonary involvement were reported (sex ratio 0.75, median age 10 years, 4 with Crohn disease and 3 with ulcerative colitis). For 2 patients, the discovery of pulmonary involvement was concomitant with the diagnosis of Crohn's disease. Pulmonary symptoms were non-specific or absent. Chest computed tomography scans revealed condensation (n = 5), mosaic appearance (n = 2), excavated (n = 2) or non-excavated (n = 3) nodules or micronodules. Bronchoalveolar lavage revealed hypercellularity with hyperlymphocytosis in 4 patients among 6. Biopsies (n = 5) showed inflammation or granulomas. Organized pneumonia was diagnosed in 5 patients, granulomatosis in 1 and necrotizing pneumonia in 1. Most patients were treated with oral corticosteroids, with a favourable outcome. Conclusions This is the first national reported cohort of IBD children with pulmonary involvement. They are discussed in regard to the 29 previous published cases we found in the same period.