SYSTEMIC-SCLEROSIS - USING HIGH-RESOLUTION CT TO DETECT LUNG-DISEASE IN CHILDREN

OBJECTIVE. The purpose of this study was to determine the prevalence o f interstitial lung disease and the severity of disease in children wi th systemic sclerosis using high-resolution CT (HRCT). SUBJECTS AND ME THODS. Eleven children (mean age, 11 years) with scleroderma underwent HRCT, chest radiography, and pulmonary function testing. Eight of the se 11 patients also underwent follow-up HRCT. HRCT studies were assess ed by two observers for ground-glass attenuation, honeycombing, and ot her abnormalities. Profusion scores for ground-glass attenuation and h oneycombing were determined by multiplying severity of disease by the percentage of lung involvement. RESULTS. Chest radiographs predicted i nterstitial lung disease in only two patients, whereas HRCT showed int erstitial lung disease in eight patients (p=.05). On HRCT, ground-glas s attenuation was found in eight patients (73%), honeycombing in five patients (45%), linear opacities in six patients (55%), and subpleural micronodules in seven patients (64%). By the end of the study, 10 pat ients (91%) had evidence of interstitial lung disease on HRCT. Overall , profusion scores for these 10 patients showed four patients with mil d, one with moderate, and five with severe disease. Also, HRCT reveale d worsening disease in three of eight patients. We found no correlatio n between duration of scleroderma and severity of interstitial lung di sease (p>.02). Seven patients with evidence of lung disease on HRCT ha d abnormal results on pulmonary function tests; patients with the high est scores for ground-glass attenuation had the most abnormal results on pulmonary function tests (p<.01). CONCLUSION. HRCT shows significan t pulmonary disease in children with systemic sclerosis, revealing abn ormalities in 91% of our patients. Pulmonary disease should be suspect ed in children with scleroderma, even if the chest radiograph has norm al findings.