Pulmonary sarcoidosis in children: a follow-up study

Progression of pulmonary sarcoidosis in children remains poorly documented. The aim of this work was to gather follow-up information on pulmonary outc omes in children with sarcoidosis and to obtain data of relevance to a disc ussion df the optimal length and regimen of glucocorticoid therapy, In the present study, the authors experience of pulmonary sarcoidosis in 21 children referred to the paediatric pulmonary department over a 10-yr peri od is reported with a documented follow-up of at least 4 yr. Evaluation of the disease during the follow-up included analysis of clinical manifestatio ns, chest radiographs, pulmonary function tests with measurements of the vi tal capacity (VC), dynamic lung compliance (Ct,dyn), lung transfer for CO ( TL,CO), and arterial blood gases, as well as bronchoalveolar lavage (BAL) w ith determination of total and differential cell counts. After initial evaluation the decision was a careful observation of four chi ldren without therapy. Corticosteroid treatment was initiated in 17 childre n, Analysis of results; indicated that after 6-12 months of treatment most clinical manifestations of the disease and chest radiograph abnormalities d isappeared, and beneficial effects on VC and TL,CO were apparent. After 18 months of steroids no benefit on pulmonary function tests could be noticed, with mainly persistence of alterations of CL,dyn. Results of BAL studies d ocumented the presence of an alveolitis with increased lymphocyte populatio ns throughout the follow-up, Relapses mere observed in four children during tapering of prednisone; they were not reported after discontinuation of st eroid therapy, Taken together data obtained in the presented population can lead to the fo llowing suggestions for the management of pulmonary sarcoidosis in children . BAL should be performed at the initial evaluation to document alveolitis; however, nothing stems to be gained from repeating this investigation duri ng follow-up in the absence of specific reasons. Once the decision to initi ate glucocorticoid therapy is made, 18 months mag be a reasonable treatment duration, Discontinuation of therapy can be decided even if the pulmonary function tests remain abnormal, but the child should then be carefully moni tored for a relapse.