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Child-Adult Transition in Sarcoidosis: A Series of 52 Patients
Simon Chauveau1,2, Florence Jeny1,2, Marie-Emeline Montagne3
1AP-HP Pulmonology Department, Avicenne Hospital, 93000 Bobigny, France.
Insights
Pediatric sarcoidosis often requires long-term treatment, with nearly half of patients needing it into adulthood. Approximately one-fifth of these individuals experience severe sarcoidosis complications later in life.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Immunology
Background:
- Pediatric sarcoidosis is a rare, often severe condition with limited long-term follow-up data.
- Understanding the adult outcomes of childhood-onset sarcoidosis is crucial for effective management.
Purpose of the Study:
- To evaluate the long-term evolution and adult outcomes of sarcoidosis that began in childhood.
- To identify factors influencing disease progression and treatment response in pediatric sarcoidosis survivors.
Main Methods:
- Retrospective and prospective study of 52 patients with pediatric-onset sarcoidosis (diagnosis ≤15 years old) followed for at least three years.
- Analysis of clinical data at presentation and long-term outcomes in adulthood, including disease severity, relapses, and treatment adverse events.
Main Results:
- A median follow-up of 11.5 years revealed that 21.2% of patients had severe sarcoidosis in adulthood.
- Relapses predominantly occurred during treatment tapering (84.5%).
- Long-term corticosteroid therapy (median 5 years) led to mostly mild adverse events (35.3%), with severe events in 3.8%.
Conclusions:
- Pediatric-onset sarcoidosis frequently necessitates prolonged treatment, impacting nearly half of patients into adulthood.
- A significant proportion of individuals with childhood-onset sarcoidosis face severe disease consequences in adulthood, highlighting the need for ongoing monitoring and management.
Abstract:
(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis (≤15-year-old) who completed at least a three-year follow-up in French expert centers were included. Clinical information at presentation and outcome in adulthood were studied. (3) Results: A total of 52 patients were included (34 prospectively in childhood and 18 retrospectively in adulthood), with a mean age of 12 (±2.7) at diagnosis. The median duration time of follow-up was 11.5 years (range 3-44.5). Relapses mostly occurred during treatment decrease (84.5%), others within the three years after treatment interruption (9.1%), and rarely when the disease was stable for more than three years (6.4%). Sarcoidosis was severe in 11 (21.2%) in adulthood. Patients received a high corticosteroid cumulative dose (median 17,900 mg) for a median duration of five years (range 0-32), resulting in mostly mild (18; 35.3%) and rarely severe (2; 3.8%) adverse events. (4) Conclusions: Pediatric-onset sarcoidosis needed a long-term treatment in almost half of the patients. Around one fifth of pediatric-onset sarcoidosis patients had severe sarcoidosis consequences in adulthood.
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