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 center.

This was a retrospective, observational, descriptive, multicenter 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.

Seven IBD children with pulmonary involvement were reported (four girls and three boys, median age 10 years, 4 with Crohn’s 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 favorable outcome.

This is the first nationally reported cohort of IBD children with pulmonary involvement. They are discussed in regard to the 29 previously published cases we found in the same period.