Chronic interstitial lung disease in children: Response to high-dose intravenous methylprednisolone pulses

The prognosis for children with chronic interstitial lung disease is poor a nd the mortality rate is high, especially in infants. This explains the man y therapeutical protocols which have been proposed and investigated by seve ral authors. In the present work, we evaluated the response of three infant s with idiopathic pulmonary fibrosis to high-dose intravenous prednisolone pulses. The patients were referred to the department at the age of 4, 17, a nd 3 months, respectively. The diagnosis was confirmed by open lung biopsy and intravenous pulse methyl prednisolone therapy was started with the foll owing protocol: 300 mg/m(2) methylprednisolone daily for 3 days, repeated e very 4 to 6 weeks. Because of the extreme severity of the respiratory distr ess at the time of diagnosis, the intravenous pulse treatments were initial ly complemented by oral prednisone. Clinical improvement was noticed within 6 months with progressive correction of hypoxemia. After follow-up for 3.5 to 4 years, with a total number of pulses of 37, 26, and 32, respectively, the children are symptom-free and do not require oxygen supplementation. D uring this period, no side effects and no adrenal insufficiency could be do cumented. Based on current knowledge of steroid action, it can be speculate d that the response to intermittent high-dose intravenous methylprednisolon e may explain the ability of this mode of hormone administration to maintai n an adequate level of glucocorticoid receptor expression. More information and trials through multicenter collaborations are needed to assess therape utical protocols of repeated high-dose intravenous steroid treatment. Pedia tr Pulmonol. 1998; 26:332-338. (C) 1998 Wiley-Liss, Inc.