Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort.
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Fletcher Camille, Hadchouel Alice, Thumerelle Caroline, Mazenq Julie, Fleury Manon, Corvol Harriet, Jedidi Nouha, Benhamida Myriam, Bessaci Katia, Bilhouee Tiphaine, Borie Raphael, Brouard Jacques, Cantais Aurélie, Clement Annick, Coutier Laurianne, Cisterne Camille, Cros Pierrick, Dalphin Marie-Laure, Delacourt Christophe, Deneuville Eric, Dubus Jean-Christophe, Egron Carole, Epaud Ralph, Fayon Michael, Forgeron Aude, Gachelin Elsa, Galode François, Gertini Isabelle, Giovannini-Chami Lisa, Gourdan Pierre, Guiddir Tamazoust, Herzog Audrey, Houdouin Véronique, Hullo Églantine, Jarreau Pierre-Henri, Labbé Guillame, Labouret Géraldine, Ladaurade Alice, Le Clainche Viala Laurence, Marguet Christophe, Masson-Rouchaud Alexandra, Perisson Caroline, Rames Cinthia, Reix Philippe, Renoux Marie-Catherine, Roditis Léa, Schweitzer Cyril, Tatopoulos Aurélie, Trioche-Eberschweiler Pascale, Troussier Françoise, Vigier Clémentine, Weiss Laurence, Legendre Marie, Louvrier Camille, de Becdelievre Alix, Coulomb Aurore, Sileo Chiara, Ducou le Pointe Hubert, Berteloot Laureline, Delestrain Céline, Nathan Nadia
Аннотация
METHODS: We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD's incidence between February 2022 and 2023.
RESULTS: chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years.
CONCLUSION: This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.