Hemosiderosis pulmonar idiopática: Evolución de 5 niños

Abstract

Idiopathic pulmonary haemosiderosis (IPH) is a rare and serious disorder characterised by diffuse alveolar haemorrhage. Its aetiology is unknown but owing to its unpredictable prognosis long term treatment is justified. Objective: to describe the clinical and therapeutic characteristics of children with IPH during 5 years of follow up. Design: this prospective study reports our experience with 5 children (3 males) aged 9 to 21 years with IPH. In 4 the initial diagnosis was pneumonia, with an average delay in the diagnosis of IPH of 2.5 years. 3 children came from agricultural zones where pesticides are frequently used. All children were treated with a standard protocol of prednisone 2 mg/kg/day for 1 month followed by 1 mg/kg every other day, plus inhaled budesonide 800-2 400 ug/day and the addition of azathioprine 2 mg/kg/day or chloroquine 10 mg/kg/day. Secondary effects included osteoporosis in 5 children, Cushing syndrome in 3 and retrocapsular opacities in 2. After 5 years of treatment 2 children stopped having bleeding episodes, 3 had in general mild episodes in whom 2 had serious bleeding events, one needing mechanical ventilation. There were no deaths. One patient became pregnant without ill effects and delivered a healthy baby. Initial pulmonary function (PFT) tests were normal in 3, mildly restrictive in 1 and mildly obstructive in 1, however during the follow-up period there were changes in the PFT in 2 patients, from normal to mildly restrictive in 1 and from mildly to moderately obstructive in 1. Intial pulmonary CT were normal in 2, 2 had a pattern of alveolar infiltrates and one interstitial infiltrates, follow up CT scans remained normal in 2 patients and showed interstitial infiltrates in the rest. Conclusions: Using this protocol in a small series of IPH there were no deaths after five years of follow up. We speculate that the use of inhaled steroids can reduce the secondary effects of oral steroids in IPH.