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Lung sarcoidosis in children: update on disease expression and management
  1. Nadia Nathan1,2,3,
  2. Pierre Marcelo4,
  3. Véronique Houdouin5,
  4. Ralph Epaud6,7,8,
  5. Jacques de Blic9,10,
  6. Dominique Valeyre11,
  7. Anne Houzel12,
  8. Pierre-François Busson13,
  9. Harriet Corvol1,2,3,
  10. Antoine Deschildre4,
  11. Annick Clement1,2,3
  12. for the RespiRare and the French Sarcoidosis groups
  1. 1Pediatric Pulmonary Department, AP-HP, Hôpital Trousseau, Paris, France
  2. 2Université Pierre et Marie Curie-Paris6, Paris, France
  3. 3UMR S-U933, Inserm, Paris, France
  4. 4Pediatric Department, Centre Hospitalier Universitaire de Lille, Lille, France
  5. 5Pediatric Pulmonary Department, AP-HP, Hôpital Robert Debré, Paris, France
  6. 6Pediatric Department, Centre Hospitalier Intercommunal de Créteil, Créteil, France
  7. 7U955, Equipe 11, Inserm, Créteil, France
  8. 8Faculté de Médecine, Université Paris-Est, Créteil, France
  9. 9Pediatric Pulmonary Department, AP-HP, Hôpital Necker Enfants Malades, Paris, France
  10. 10Université Paris Descartes-Paris5, Paris, France
  11. 11AP–HP, Hôpital Avicenne, Pulmonary Department and l'Université Paris 13, COMUE Sorbonne Paris Cité, EA 2363, Bobigny, France
  12. 12Pediatric Department, Centre Hospitalier Universitaire de Dijon, Dijon, France
  13. 13UMR S-707, Inserm, Paris, France
  1. Correspondence to Dr Nadia Nathan, Pediatric Pulmonary Department, Hôpital Armand Trousseau, 26 avenue du Dr Arnold Netter, Paris 75012, France; nadia.nathan{at}trs.aphp.fr

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.

Discussion 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.

  • Sarcoidosis
  • Paediatric interstitial lung disease
  • Paediatric Lung Disaese
  • Rare lung diseases

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