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[Sarcoidosis in children. Clinical manifestations, epidemiology, treatment and prognosis]
1Børneafdelingen, Amtssygehuset i Gentofte.
Insights
Pediatric sarcoidosis, a rare granulomatous disease, affects Danish children with pulmonary involvement and hypercalcemia. Treatment with steroids requires careful consideration due to growth retardation risks.
Area of Science:
- Pediatric Pulmonology
- Rheumatology
- Immunology
Background:
- Sarcoidosis is a multisystem granulomatous disease of unknown etiology.
- Diagnosis relies on identifying epithelioid cell granulomas in affected organs.
Observation:
- The incidence in Danish children under 15 is low, approximately 0.22-0.27/100,000 children/year.
- Three pediatric sarcoidosis cases were observed in Copenhagen Country (1980-1992), all with pulmonary involvement.
- One case presented with severe hypercalcemia; younger children (<5 years) often show lung, lymph node, and eye involvement.
Findings:
- Pediatric sarcoidosis can be asymptomatic.
- Pulmonary involvement is common in affected children.
- Hypercalcemia is a potential complication, particularly in younger children.
Implications:
- Systemic steroids are the primary symptomatic treatment.
- Steroid use necessitates careful monitoring due to growth retardation risks.
- Long-term prognosis remains unclear due to limited follow-up data.
Abstract:
Sarcoidosis is a granulomatous disease of unknown aetiology, which may affect various organs. The diagnosis is obtained by the demonstration of epytheloid cell granulomas in an affected organ. The incidence of sarcoidosis in Danish children less than 15 years of age is 0.22-0.27/100.000 children/year, corresponding to approximately three new cases in Denmark each year. The disease often takes an asymptomatic course. During the period 1980-1992, three cases of paediatric sarcoidosis were observed in Copenhagen Country. All three had pulmonary involvement, and one had severe hypercalcaemia. In children less than five years of age, the disease is characterized by involvement of lungs, lymph nodes and eyes. Treatment, which is symptomatic, consists of systemic steroids. Due to the risk of growth retardation, the indication for treatment should be carefully considered and steroids administered at the lowest effective dose. Due to the lack of follow-up studies, the long term prognosis is unclarified.