Childhood sarcoidosis: long-term follow-up.
The aim of the present study was to describe clinical features and long-term survival in childhood sarcoidosis. In total, 46 ethnic Caucasian Danish children (aged <16 yrs, 24 males) with sarcoidosis were identified in 1979-1994. In 33 (72%) children, diagnosis was verified by histology and, in the remaining 13, by clinical and radiological findings. In total, 37 subjects had a follow-up examination. Median (range) age at onset of disease was 14 (0.7-15.8) yrs and median (range) clinical follow-up was 15 (3-23) yrs after onset of disease. The median (range) age at clinical follow-up was 28 (17-30) yrs. At follow-up: 36 (78%) children recovered completely; 30 (65%) showed complete clinical regression at a median (range) 0.7 (0.6-5.9) yrs after onset of disease; two (4%) recovered with organ damage (unilateral loss of vision, abnormal chest radiograph); five (11%) still had chronic active disease with multiorgan involvement and impaired lung function; and three (7%) were deceased, due to central nervous system sarcoidosis and acute myeloid leukaemia probably caused by cytostatics. In Danish children, sarcoidosis had a favourable prognosis; the majority recovered <6 yrs after onset of disease. Some developed chronic active disease and impairment of pulmonary function, demanding continuous medical treatment. Prognosis was not related to the age at onset of disease. Erythema nodosum was associated with a good prognosis, and central nervous system involvement with a poor prognosis.
The Heart Centre, Division of Lung Transplantation, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. nils.mil@dadlnet.dk
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Milman
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Hoffmann
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003718:Denmark / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D010993:Plethysmography, Whole Body; D020127:Recovery of Function; D012042:Registries; D012129:Respiratory Function Tests; D017565:Sarcoidosis, Pulmonary / Q000150:complications / Q000401:mortality / Q000473:pathology* / Q000534:rehabilitation*