Pulmonary sarcoidosis in children: a follow-up study

Abstract

Progression of pulmonary sarcoidosis in children remains poorly documented. The aim of this work was to gather follow-up information on pulmonary outcomes in children with sarcoidosis and to obtain data of relevance to a discussion of 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 period is reported with a documented follow-up of at least 4 yr. Evaluation of the disease during the follow-up included analysis of clinical manifestations, chest radiographs, pulmonary function tests with measurements of the vital capacity (VC), dynamic lung compliance (CL,dyn), lung transfer for CO (TL,CO), and arterial blood gases, as well as bronchoalveolar lavage (BAL) with determination of total and differential cell counts.After initial evaluation the decision was a careful observation of four children without therapy. Corticosteroid treatment was initiated in 17 children. Analysis of results indicated that after 6±12 months of treatment most clinical manifestations of the disease and chest radiograph abnormalities disappeared, and bene®cial effects on VC and TL,CO were apparent. After 18 months of steroids no bene®t on pulmonary function tests could be noticed, with mainly persistence of alterations of CL,dyn. Results of BAL studies documented the presence of an alveolitis with increased lymphocyte populations throughout the follow-up. Relapses were observed in four children during tapering of prednisone; they were not reported after discontinuation of steroid therapy.Taken together data obtained in the presented population can lead to the following suggestions for the management of pulmonary sarcoidosis in children. BAL should be performed at the initial evaluation to document alveolitis; however, nothing seems to be gained from repeating this investigation during follow-up in the absence of speci®c reasons. Once the decision to initiate glucocorticoid therapy is made, 18 months may 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 monitored for a relapse.

Sarcoidosis infantil. Un caso clínico infrecuente

“…En ese trabajo, 8,3% tenían compromiso pulmonar estadio III, todos ellos con afectación de órganos extrapulmonares. 3 En las otras series de casos no se comunicaron pacientes con compromiso pulmonar estadio III sin compromiso de otro órgano, como en la paciente presentada [6][7][8][9] (Tabla 1). Las manifestaciones oculares, cutáneas y musculoesqueléticas se observaron en 15% a 60% de los niños con sarcoidosis.…”

An unusual case of childhood sarcoidosis

“…In this report, although 8.3% had stage III lung involvement; all of them had extrapulmonary organ involvements as well. 3 In the other case series of sarcoidosis there was no patient reported as state III lung involvement without any other organ involvement like ours 6,7,8,9 (Table 1). Eye, skin, musculosketelal features of sarcoidosis has been reported in 15% to 60% of children with sarcoidosis.…”

Prognostic Factors in Sarcoidosis

“…Self-limiting disease with an acute clinical presentation more typical of younger patients may also be observed in patients over 60. On the other hand, sarcoidosis in children may be systemic, chronic, progressive and recurrent (Baculard et al, 2001;Kendig & Brummer, 1976). Lenner et al (2002) compared clinical features of patients with disease onset < 50 and > 50 year of age, and did not find significant differences.…”