Clinical Spectrum and Lung Pathology in Children with Interstitial Lung Disease

ABSTRACT

PURPOSE: Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. METHODS: Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings, lung pathology and clinical responses after steroid therapy and prognosis. Fifteen patients were performed open lung biopsy, but six patients were diagnosed bronchiolitis obliterans by HRCT. RESULTS: The median ages at onset of ILD was 1 year 5 month old. Common clinical findings were tachypnea(90.0%), hypoxemia(90.0%). 14 patients among 15 patients were diagnosed specifically after open lung biopsy. Interstitial pneumonitis was 6 cases, including 3 nonspecific interstitial pneumonitis, 2 cases of desquamative interstitial pneumonitis and one usual interstitial pneumonitis. Other diagnosis included idiopathic pulmonary fibrosis, cytomegalovirus pneumonitis, diffuse aspiration bronchiolitis, pulmonary lymphangiomatosis, BOOP(bronchiolotis obliterans organizing pneumonia) and pulmonary histiocytosis. Six patients showed clinical and HRCT findings consistent with bronchilitis obliterans. Common radiologic findings were mosaic perfusion(12/21), bronchial wall thickening(9/21). 13 patients among 15 patients with methylprednisolone pulse therapy showed improvement of clinical symptoms. The severity-of-illness score was improved significantly after methylprednisolone pulse therapy. CONCLUSION: Pediatric ILD includes heterogeneous disorders. Open lung biopsy was helpful to make early diagnosis. Severity-of-illness score is a noninvasive and useful measure of disease progression or response to therapy. Methylprednisolone pulse therapy may be effective to treat ILD.