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Childhood sarcoidosis, a rare granulomatous disease, is endemic in the southeastern and south central US. This study details its manifestations, prognosis, and suggests an inhaled antigen etiology in affected children.
Area of Science:
- Pediatric Pulmonology
- Immunology
- Rheumatology
Background:
- Sarcoidosis is a multisystem granulomatous disease of unknown cause, typically affecting young adults.
- Childhood sarcoidosis is less common, with previous reports primarily from specific US regions.
Purpose of the Study:
- To characterize childhood sarcoidosis cases in Arkansas.
- To redefine the endemic geographic area for pediatric sarcoidosis.
- To analyze clinical manifestations, treatment, and prognosis in children.
Main Methods:
- Retrospective review of 30 pediatric sarcoidosis cases diagnosed between 1957 and 1982.
- Analysis of demographic data, clinical symptoms, diagnostic findings, and treatment outcomes.
Main Results:
- The study identified 30 cases in Arkansas, expanding the known endemic area to the south central US.
- The median age was 11 years, with 90% of patients symptomatic, frequently presenting with respiratory and systemic symptoms.
- Common manifestations included abnormal chest X-rays, restrictive lung function, lymphadenopathy, splenomegaly, skin lesions, uveitis, hyperglobulinemia, and hypercalcemia.
Conclusions:
- Childhood sarcoidosis in the US is likely endemic to the southeastern and south central regions.
- The disease course and prognosis in children resemble those in adults, with potential for severe ocular, pulmonary, and systemic complications.
- Sarcoidosis is hypothesized to be an immune response to an inhaled antigen prevalent in these endemic areas.
Abstract:
Sarcoidosis, a granulomatous disease of unknown etiology, is most often seen in young adults. Childhood cases have been reported primarily from Virginia, North Carolina, and South Carolina. Thirty children have been seen in Arkansas between 1957 and 1982, which suggests that the endemic area for childhood sarcoidosis should include both the south central and southeastern United States. The median age was 11 years; 29/30 were black; and 90% were symptomatic, 60% with systemic symptoms. Manifestations included abnormal chest roentgenograms (100%), restrictive pulmonary functions (79%), lymphadenopathy (63%), splenomegaly (40%), skin lesions (30%), granulomatous uveitis (27%), hyperglobulinemia (72%), and hypercalcemia (30%). Course and prognosis were similar to those in adults at follow-up of two to 11 years. Four with uveitis had serious residua in the eyes, three had crippling restrictive lung disease, and two died of respiratory failure. Sarcoidosis seems to be an immunologic response to an unknown inhaled antigen, probably present in the southeastern and south central US.