Childhood sarcoidosis in Denmark 1979–1994: long-term follow-up of 46 children

¹ The Heart Centre, Division of Lung Transplantation, Rigshospitalet, University of Copenhagen E-mail: nils.mil@dadlnet.dk and
² Dept of Paediatrics, Næstved Hospital, Næstved, sulihof@stam.dk, Denmark

^* To whom correspondence should be addressed. E-mail: nils.mil{at}dadlnet.dk,.

	Abstract

To describe clinical features and long-term survival of childhood sarcoidosis. Forty-six children, ethnic Caucasian Danes, (24 males) <16 years of age with sarcoidosis were identified in 1979–1994. In 33/46 (72%) children, diagnosis was verified by histology, in 13 by clinical and radiological findings. 37 subjects had a follow-up examination. Median age at onset of disease was 14 years (range 0.7–15.8). Clinical follow-up was median 15 years (range 3–23) after onset of disease. Median age at clinical follow-up was 28 years (range 17–30). 36/46 children (78%) recovered completely; 30/46 (65%) showed complete clinical regression at median 0.7 years (range 0.6–5.9) after onset of disease; 2/46 (4%) recovered with organ damage (unilateral loss of vision, abnormal chest X-ray); 5/46 (11%) still have chronic active disease with multiorgan involvement and impaired lung function. Three subjects (7%) were deceased, due to central nervous system (CNS) sarcoidosis and acute myeloid leukaemia probably caused by cytostatics. In Danish children, sarcoidosis had a favourable prognosis; the majority recovered within 6 years after onset of disease. Some developed chronic active disease and impairment of pulmonary function demanding continuing medical treatment. Prognosis was not related to the age at onset of disease. Erythema nodosum was associated with a good prognosis and CNS involvement with a poor prognosis.

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