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Lung sarcoidosis in children: update on disease expression and management
Nadia Nathan1, Pierre Marcelo2, Véronique Houdouin3
1Pediatric Pulmonary Department, AP-HP, Hôpital Trousseau, Paris, France Université Pierre et Marie Curie-Paris6, Paris, France UMR S-U933, Inserm, Paris, France.
Insights
Pediatric sarcoidosis, a rare lung disease, often presents as multi-organ involvement. Younger children diagnosed with sarcoidosis showed better recovery rates and fewer relapses compared to older children.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Immunology
Background:
- Sarcoidosis is a rare granulomatous disease affecting multiple organs, with a significant impact on pediatric respiratory health.
- Understanding the specific characteristics of childhood sarcoidosis is crucial for effective management and improved patient outcomes.
Purpose of the Study:
- To update information on the presentation, progression, and management of pediatric lung sarcoidosis.
- To identify prognostic factors influencing outcomes in children diagnosed with sarcoidosis.
Main Methods:
- A large cohort of pediatric thoracic sarcoidosis patients was analyzed through the French Reference Centre for Rare Lung Diseases (RespiRare) network.
- Data collected included disease presentation, treatment strategies, and patient outcomes over time.
Main Results:
- Forty-one patients, primarily of Afro-Caribbean and Sub-Saharan origin, were included, with a median age of 11.8 years at diagnosis.
- Eighty-five percent presented with multi-organ involvement; corticosteroids were the primary treatment.
- Children diagnosed before 10 years old demonstrated higher recovery rates (50% vs. 29%) and fewer relapses (29% vs. 58%) within 18 months.
Conclusions:
- Pediatric sarcoidosis is often severe, characterized by multi-organ involvement and general symptoms, particularly in children of specific ethnic backgrounds.
- Traditional prognostic factors may not be applicable to pediatric cases; younger age at diagnosis does not necessarily predict a worse outcome.
- Further long-term follow-up studies are needed to fully understand the trajectory of pediatric sarcoidosis.
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.
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