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

• Published in: Pediatric pulmonology Vol. 26; no. 5; pp. 332 - 338
• Main Authors: Desmarquest, Pascale, Tamalet, Aline, Fauroux, Brigitte, Boule, Michele, Boccon-Gibod, Liliane, Tournier, Guy, Clement, Annick
• Format: Journal Article
• Language: English
• Published: New York Wiley Subscription Services, Inc., A Wiley Company 01-11-1998; Wiley-Liss
• Subjects: Adolescent; Anti-Inflammatory Agents - administration & dosage; Biological and medical sciences; Biopsy; Bronchoalveolar Lavage Fluid - cytology; Bronchoalveolar Lavage Fluid - microbiology; Bronchoscopy; Child, Preschool; children; Chronic Disease; Dose-Response Relationship, Drug; Drug Evaluation; Female; Humans; Infant; Injections, Intravenous; interstitial lung disease; Lung - pathology; Lung Diseases, Interstitial - blood; Lung Diseases, Interstitial - diagnosis; Lung Diseases, Interstitial - drug therapy; Male; Medical sciences; methyl prednisolone; Methylprednisolone - administration & dosage; Pharmacology. Drug treatments; Prospective Studies; pulmonary fibrosis; pulse therapy; Respiratory system; Treatment Outcome; Human; Corticosteroid; Lung disease; Intravenous administration; Respiratory disease; Treatment efficiency; Idiopathic; Infant; Methylprednisolone; Case study; Chemotherapy; Chronic; Treatment; Follow up study; Interstitial pneumonitis; High dose
• ISSN: 8755-6863; 1099-0496
• DOI: 10.1002/(SICI)1099-0496(199811)26:5<332::AID-PPUL5>3.0.CO;2-Q

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. Pediatr Pulmonol. 1998; 26:332–338. © 1998 Wiley‐Liss, Inc.