Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort

被引：4

作者：

Fletcher, Camille ^{[1
,2
,3
]}

Hadchouel, Alice ^{[4
,5
,6
]}

Thumerelle, Caroline ^{[7
]}

Mazenq, Julie ^{[8
,9
,10
]}

Fleury, Manon ^{[11
,12
]}

Corvol, Harriet ^{[13
]}

Jedidi, Nouha ^{[1
]}

Benhamida, Myriam ^{[14
]}

Bessaci, Katia ^{[15
]}

Bilhouee, Tiphaine ^{[14
]}

Borie, Raphael ^{[16
,17
]}

Brouard, Jacques ^{[18
]}

Cantais, Aurelie ^{[19
]}

Clement, Annick ^{[20
]}

Coutier, Laurianne ^{[21
]}

Cisterne, Camille ^{[7
]}

Cros, Pierrick ^{[22
]}

Dalphin, Marie-Laure ^{[23
]}

Delacourt, Christophe ^{[4
,5
,6
]}

Deneuville, Eric ^{[24
]}

Dubus, Jean-Christophe ^{[8
,9
,25
]}

Egron, Carole ^{[26
]}

Epaud, Ralph ^{[27
,28
,29
]}

Fayon, Michael ^{[30
,31
,32
]}

Forgeron, Aude ^{[33
]}

Gachelin, Elsa ^{[34
]}

Galode, Francois ^{[30
]}

Gertini, Isabelle ^{[35
]}

Giovannini-Chami, Lisa ^{[36
]}

Gourdan, Pierre ^{[36
]}

Guiddir, Tamazoust ^{[37
]}

Herzog, Audrey ^{[38
]}

Houdouin, Veronique ^{[39
]}

Hullo, Eglantine ^{[40
]}

Jarreau, Pierre-Henri ^{[41
]}

Labbe, Guillame ^{[26
]}

Labouret, Geraldine ^{[42
]}

Ladaurade, Alice ^{[23
]}

Viala, Laurence Le Clainche ^{[39
]}

Marguet, Christophe ^{[43
]}

Masson-Rouchaud, Alexandra ^{[44
]}

Perisson, Caroline ^{[45
]}

Rames, Cinthia ^{[46
]}

Reix, Philippe ^{[21
]}

Renoux, Marie-Catherine ^{[47
]}

Roditis, Lea ^{[42
]}

Schweitzer, Cyril ^{[48
]}

Tatopoulos, Aurelie ^{[48
]}

Trioche-Eberschweiler, Pascale ^{[49
]}

Troussier, Francoise ^{[50
]}

机构：

[1] Sorbonne Univ, Armand Trousseau Hosp, AP HP, Paediat Pulmonol Dept, F-75012 Paris, France

[2] Sorbonne Univ, Armand Trousseau Hosp, AP HP, RespiRare,Reference Ctr Rare Lung Dis, Paris, France

[3] Sorbonne Univ, Armand Trousseau Hosp, Lab Childhood Genet Dis, INSERM,UMR S933, Paris, France

[4] Necker Enfants Malad Hosp, AP HP, Serv Pneumol Pediat, Paris, France

[5] Necker Enfants Malad Hosp, Reference Ctr rare lung Dis RespiRare, Paris, France

[6] Univ Paris Cite, INSERM, U1151, INEM, Paris, France

[7] Lille Univ Hosp, Pediat Pulmonol Dept, Lille, France

[8] Aix Marseille Univ, Pediat Pulmonol Dept, AP HM, Marseille, France

[9] Aix Marseille Univ, Reference Ctr Rare Lung Dis RespiRare, AP HM, Marseille, France

[10] INRAE, C2VN, INSERM, Marseille, France

[11] Sorbonne Univ, Armand Trousseau Hosp, APHP, Pediat Pulmonol Dept, Paris, France

[12] Sorbonne Univ, Reference Ctr Rare Lung Dis RespiRare, Paris, France

[13] CDR St Antoine, Paris, France

[14] Univ Hosp Ctr Nantes, Pediat Dept, Nantes, France

[15] Univ Hosp Ctr Reims, Pediat Pulmonol Dept, Reims, France

[16] Univ Paris Cite, Bichat Hosp, APHP, Pulmonol Dept A, Paris, France

[17] INSERM, Inserm UMR S PHERE 1152, Paris, France

[18] Univ Hosp Ctr Caen, Med Pediat Dept, Inserm UMRS 1311, DYNAMICURE,UNICAEN, Caen, France

[19] St Etienne Univ Hosp, Pediat Dept, St Etienne, France

[20] Sorbonne Univ, Plateforme Expertise Malad Rares, AP HP, Paris, France

[21] Univ Hosp Lyon, Pediat Pulmonol Dept, Lyon, France

[22] Ctr Hosp Univ Brest, Pediat Dept, Brest, France

[23] Ctr Hosp Univ Besancon, Pediat Pulmonol Dept, Besancon, France

[24] Univ Hosp Ctr Rennes, Pediat Pulmonol Dept, Rennes, France

[25] Aix Marseille Univ, IRD, MEPHI, IHU Mediterranee Infect, Marseille, France

[26] Univ Hosp Ctr Clermont Ferrand, Clermont Ferrand, France

[27] Ctr Hosp Intercommunal Creteil, Pediat, Creteil, France

[28] FHU SENEC, Creteil, France

[29] Univ Paris Est Creteil, INSERM, IMRB, Creteil, France

[30] Ctr Hosp Univ Bordeaux, Pediat Pulmonol Dept, Bordeaux, France

[31] Clin Invest Ctr CIC 1401, Bordeaux, France

[32] Bordeaux Univ, Cardiothorac Res Ctr Bordeaux, Inserm, U1045, Bordeaux, France

[33] Hosp Ctr Le Mans, Pediat Pulmonol Dept, Le Mans, France

[34] CHU Nord Reunion, Pediat Pulmonol Dept, St Denis, France

[35] Tours Univ Hosp, Pediat Pulmonol Dept, Tours, France

[36] Hop Pediat Nice CHU LENVAL, Pediat Pulmonol Dept, Nice, France

[37] Univ Paris Saclay, Hosp Bicetre, Pediat Pulmonol Dept, AP HP, Le Kremlin Bicetre, France

[38] CHU Strasbourg, Pediat Pulmonol Dept, Strasbourg, France

[39] Paris Univ, Robert Debre Hosp, AP HP, Pediat Pulmonol Dept, Paris, France

[40] Univ Grenoble Alpes, Pediat Pulmonol Dept, CHU Grenoble Alpes, Grenoble, France

[41] Univ Paris Cite, Cochin Hosp, Neonatal intens care unit, Paris, France

[42] CHU Toulouse, Pediat Pulmonol Dept, Toulouse, France

[43] CHU Rouen, Pediat Pulmonol Dept, Rouen, France

[44] CHU Limoges, Pediat Pulmonol Dept, Limoges, France

[45] CHU La Reunion Sites Sud, Pediat Pulmonol Dept, St Pierre, Reunion, France

[46] CHU Amiens Picardie, Pediat Dept, Amiens, France

[47] CHRU Montpellier, Pediat Pulmonol Dept, Montpellier, France

[48] CHU Nancy, Pediat Pulmonol Dept, Nancy, France

[49] Paris Saclay Univ, Antoine Beclere Hosp, APHP, Pediat Dept, Clamart, France

[50] CHU Angers, Pediat Dept, Angers, France

来源：

THORAX | 2024年 / 79卷 / 09期

关键词：

Child; Clinical Epidemiology; Rare lung diseases; Paediatric Lung Disaese; Paediatric interstitial lung disease; UNITED-KINGDOM; CHILDREN; CLASSIFICATION; MANAGEMENT;

D O I：

10.1136/thorax-2023-221325

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Introduction Interstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was to assess the prevalence and incidence of chILD in France. 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.

引用

页码：842 / 852

页数：11

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