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Chronic interstitial lung disease in children: response to high-dose intravenous methylprednisolone pulses
P Desmarquest1, A Tamalet, B Fauroux
1Department of Pediatric Pulmonology-INSERM U142, Hopital Trousseau, St Antoine Medical School, University of Paris, France.
Insights
High-dose intravenous methylprednisolone pulses show promise for treating infants with idiopathic pulmonary fibrosis, leading to symptom resolution and improved oxygenation without significant side effects.
Area of Science:
- Pediatric Pulmonology
- Interstitial Lung Disease
- Pharmacology
Background:
- Chronic interstitial lung disease (ILD) in children, particularly infants, carries a poor prognosis and high mortality rate.
- Existing therapeutic protocols for pediatric ILD have shown limited success, necessitating novel treatment strategies.
Purpose of the Study:
- To evaluate the efficacy and safety of high-dose intravenous methylprednisolone pulse therapy in infants diagnosed with idiopathic pulmonary fibrosis (IPF).
Main Methods:
- Three infants with IPF underwent high-dose intravenous methylprednisolone pulse therapy (300 mg/m2 daily for 3 days every 4-6 weeks).
- Initial treatment was supplemented with oral prednisone due to severe respiratory distress.
- Diagnosis was confirmed via open lung biopsy.
Main Results:
- All three patients demonstrated significant clinical improvement within six months, including progressive correction of hypoxemia.
- After 3.5 to 4 years of follow-up, children were symptom-free and independent of oxygen supplementation.
- No significant side effects or adrenal insufficiency were observed during the treatment period.
Conclusions:
- Intermittent high-dose intravenous methylprednisolone pulses may be an effective treatment for pediatric idiopathic pulmonary fibrosis.
- This therapeutic approach might maintain adequate glucocorticoid receptor expression, contributing to treatment success.
- Further multicenter trials are warranted to validate these findings and optimize steroid treatment protocols for pediatric ILD.
Abstract:
The prognosis for children with chronic interstitial lung disease is poor and the mortality rate is high, especially in infants. This explains the many therapeutical protocols which have been proposed and investigated by several authors. In the present work, we evaluated the response of three infants with idiopathic pulmonary fibrosis to high-dose intravenous prednisolone pulses. The patients were referred to the department at the age of 4, 17, and 3 months, respectively. The diagnosis was confirmed by open lung biopsy and intravenous pulse methyl prednisolone therapy was started with the following protocol: 300 mg/m2 methylprednisolone daily for 3 days, repeated every 4 to 6 weeks. Because of the extreme severity of the respiratory distress at the time of diagnosis, the intravenous pulse treatments were initially 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. During this period, no side effects and no adrenal insufficiency could be documented. Based on current knowledge of steroid action, it can be speculated that the response to intermittent high-dose intravenous methylprednisolone may explain the ability of this mode of hormone administration to maintain an adequate level of glucocorticoid receptor expression. More information and trials through multicenter collaborations are needed to assess therapeutical protocols of repeated high-dose intravenous steroid treatment.