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Pulmonary sarcoidosis in children: a follow-up study.
- Source :
-
The European respiratory journal [Eur Respir J] 2001 Apr; Vol. 17 (4), pp. 628-35. - Publication Year :
- 2001
-
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 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 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 specific 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.
- Subjects :
- Adolescent
Blood Gas Analysis
Bronchoalveolar Lavage
Cell Count
Child
Child, Preschool
Disease Progression
Female
Follow-Up Studies
Glucocorticoids administration & dosage
Humans
Lung Compliance
Male
Prednisone administration & dosage
Pulmonary Gas Exchange
Radiography, Thoracic
Sarcoidosis, Pulmonary physiopathology
Treatment Outcome
Vital Capacity
Glucocorticoids therapeutic use
Prednisone therapeutic use
Sarcoidosis, Pulmonary drug therapy
Subjects
Details
- Language :
- English
- ISSN :
- 0903-1936
- Volume :
- 17
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- The European respiratory journal
- Publication Type :
- Academic Journal
- Accession number :
- 11401056
- Full Text :
- https://doi.org/10.1183/09031936.01.17406280