IDIOPATHIC PULMONARY FIBROSIS IN INFANTS

Idiopathic pulmonary fibrosis is a poorly characterized disease in inf ants. In the present report, we reviewed our experience with 10 infant s during a 10-year period. In 9 patients, onset of symptoms occurred b efore the age of 2 months and included tachypnea, cough, and inadequat e weight gain. However, despite the presence of these symptoms, diagno sis was delayed for 3 months at which time the infants were referred t o the pediatric pulmonary department, when the diagnosis was confirmed by open lung biopsy. At the time of admission, bronchoscopy with alve olar lavage was performed in 9 children and showed severe alveolitis w ith an increase in the neutrophil count. Nine inf ants were treated wi th prednisone alone or in combination with chloroquine, colchicine, or cyclophosphamide; all these patients died despite treatment. One infa nt was treated with pulses of methylprednisolone because of a failure in response to oral prednisone. This girl who displayed similar clinic al, radiological, and histological abnormalities as the other children at the time of diagnosis is the only child still alive after 3 years of follow-up. She is now free of respiratory symptoms and has a normal growth curve. The present report raised two important points: (1) a t horough evaluation of characteristic symptoms should lead to an early diagnosis of pulmonary fibrosis in infants; and (2) administration of pulse therapy using corticosteroids has been helpful and needs to be e valuated further. (C) 1997 Wiley-Liss, Inc.