title: Lung sarcoidosis in children: update on disease expression and management.
abstract: BACKGROUND
  Sarcoidosis is a rare lung disease in children. The aim of the present study was to provide update information on disease presentation and progression, patient management and prognosis factors in a cohort of children with lung sarcoidosis.
  
  
  METHODS
  With the network of the French Reference Centre for Rare Lung Diseases (RespiRare), we collected information on a large cohort of paediatric thoracic sarcoidosis to provide information on disease presentation, management and outcome.
  
  
  RESULTS
  Forty-one patients were included with a median age at diagnosis of 11.8  years (1.1-15.8), mostly from Afro-Caribbean and Sub-Saharan origin. At diagnosis, 85% presented with a multi-organic disease, and no major differences were found regarding disease severity between the patients diagnosed before or after 10 years old. Corticosteroids were the most used treatment, with more intravenous pulses in the youngest patients. The 18-month outcome showed that patients diagnosed before 10 years old were more likely to recover (50% vs 29%), and presented fewer relapses (29% vs 58%). At 4-5 years of follow-up, relapses were mostly observed for patients diagnosed after 10 years old.
  
  
  CONCLUSIONS
  In the included children, mostly of Afro-Caribbean and Sub-Saharan origin, sarcoidosis seems severe, with multi-organic involvement and foreground general symptoms. Common prognosis factors are not suitable in paediatric patients, and a young age at diagnosis does not seem to be associated with a poorer prognosis. The study is ongoing to provide further information on the very-long-term follow-up of paediatric sarcoidosis.
authors: Pediatric Pulmonary Department, AP-HP, Hôpital Trousseau, Paris, France Université Pierre et Marie Curie-Paris6, Paris, France UMR S-U933, Inserm, Paris, France.
authors: Nadia
authors: N
authors: Nathan
authors: Pediatric Department, Centre Hospitalier Universitaire de Lille, Lille, France.
authors: Pierre
authors: P
authors: Marcelo
authors: Pediatric Pulmonary Department, AP-HP, Hôpital Robert Debré, Paris, France.
authors: Véronique
authors: V
authors: Houdouin
authors: Pediatric Department, Centre Hospitalier Intercommunal de Créteil, Créteil, France U955, Equipe 11, Inserm, Créteil, France Faculté de Médecine, Université Paris-Est, Créteil, France.
authors: Ralph
authors: R
authors: Epaud
authors: Pediatric Pulmonary Department, AP-HP, Hôpital Necker Enfants Malades, Paris, France Université Paris Descartes-Paris5, Paris, France.
authors: Jacques
authors: J
authors: de Blic
authors: AP-HP, Hôpital Avicenne, Pulmonary Department and l'Université Paris 13, COMUE Sorbonne Paris Cité, EA 2363, Bobigny, France.
authors: Dominique
authors: D
authors: Valeyre
authors: Pediatric Department, Centre Hospitalier Universitaire de Dijon, Dijon, France.
authors: Anne
authors: A
authors: Houzel
authors: UMR S-707, Inserm, Paris, France.
authors: Pierre-François
authors: PF
authors: Busson
authors: Pediatric Pulmonary Department, AP-HP, Hôpital Trousseau, Paris, France Université Pierre et Marie Curie-Paris6, Paris, France UMR S-U933, Inserm, Paris, France.
authors: Harriet
authors: H
authors: Corvol
authors: Pediatric Department, Centre Hospitalier Universitaire de Lille, Lille, France.
authors: Antoine
authors: A
authors: Deschildre
authors: Pediatric Pulmonary Department, AP-HP, Hôpital Trousseau, Paris, France Université Pierre et Marie Curie-Paris6, Paris, France UMR S-U933, Inserm, Paris, France.
authors: Annick
authors: A
authors: Clement
mesh_terms: D000293:Adolescent; D017954:Africa South of the Sahara / Q000208:ethnology; D044383:Black People / Q000706:statistics & numerical data; D017691:Caribbean Region / Q000208:ethnology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007262:Infusions, Intravenous; D008297:Male; D035583:Rare Diseases; D012008:Recurrence; D017565:Sarcoidosis, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000208:ethnology*; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids
keywords: Paediatric Lung Disaese; Paediatric interstitial lung disease; Rare lung diseases; Sarcoidosis