Idiopathic pulmonary fibrosis in infants: good prognosis with conservativemanagement

Background-Pulmonary interstitial fibrosis in children is a disease of unkn own aetiology, usually associated with a poor prognosis. Methods-In this case series we describe 11 children presenting over a 10 ye ar period, managed conservatively and associated with a good prognosis. Results-In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and per cutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloro quine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recent ly diagnosed children have significant symptoms, seven have dyspnoea on exe rcise, and two are symptom free. Conclusion-The good prognosis seen in these patients is different to previo us case reports, indicating a greater than 50% mortality.