J Korean Pediatr Soc.  2002 Jan;45(1):79-87.

Clinical Spectrum and Lung Pathology in Children with Interstitial Lung Disease

• Chung JH
• Ha SJ
• Kim BS
• Hong SJ

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.

Keyword

Interstitial lung disease; Open lung biopsy; Methylprednisolone therapy

MeSH Terms

Biopsy
Bronchiolitis
Bronchiolitis Obliterans
Child*
Chungcheongnam-do
Cytomegalovirus
Diagnosis
Disease Progression
Early Diagnosis
Histiocytosis
Humans
Idiopathic Pulmonary Fibrosis
Infant
Lung Diseases, Interstitial*
Lung*
Methylprednisolone
Pathology*
Pneumonia
Prognosis
Retrospective Studies
Methylprednisolone