Systemic glucocorticoids plus cyclophosphamide for acute exacerbation of idiopathic pulmonary fibrosis: a retrospective nationwide study.

Abstract

PURPOSE:

Mortality of acute exacerbation of idiopathic pulmonary fibrosis is high, and it remains unknown whether cyclophosphamide is an effective treatment for this condition.

OBJECTIVES:

This study compared the effects of cyclophosphamide combined with systemic glucocorticoids with those of systemic glucocorticoids alone.

METHODS:

Using the Diagnosis Procedure Combination database in Japan, adult patients with idiopathic pulmonary fibrosis who had received high-dose methylprednisolone and mechanical ventilation at admission from July 1, 2010, to March 31, 2014, were identified. Instrumental variable analyses based on a hospital preference for cyclophosphamide were performed to compare in-hospital outcomes.

RESULTS:

Eligible patients (n=1847) were divided into the methylprednisolone plus cyclophosphamide group (n=104) and the methylprednisolone alone group (n=1743). The results of an instrumental variable analysis detected no significant differences between the groups with respect to in-hospital mortality (odds ratio, 1.11; 95% confidence interval, 0.19-6.43), ventilator-free days (difference, 2.2; 95% confidence interval, -2.6 to 7.0).

CONCLUSIONS:

In a Japanese inpatient database study analyzing outcomes from patients with acute exacerbation idiopathic pulmonary fibrosis receiving systemic glucocorticoids, the addition of cyclophosphamide was not associated with improved in-hospital mortality and ventilator-free days.

• Authors: Shotaro Aso, Hiroki Matsui, Kiyohide Fushimi, Hideo Yasunaga
• Journal: Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG (Sarcoidosis Vasc Diffuse Lung Dis) Vol. 36 Issue 2 Pg. 116-123 ( 2019) ISSN: 2532-179X [Electronic] Italy
• PMID: 32476944 (Publication Type: Comparative Study, Journal Article)
• Copyright: Copyright: © 2019.

Chemical References

• Glucocorticoids
• Immunosuppressive Agents
• Cyclophosphamide
• Methylprednisolone

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Cyclophosphamide (administration & dosage, adverse effects)
• Databases, Factual
• Disease Progression
• Drug Therapy, Combination
• Female
• Glucocorticoids (administration & dosage, adverse effects)
• Hospital Mortality
• Humans
• Idiopathic Pulmonary Fibrosis (diagnosis, drug therapy, mortality, physiopathology)
• Immunosuppressive Agents (administration & dosage, adverse effects)
• Japan
• Lung (drug effects, physiopathology)
• Male
• Methylprednisolone (administration & dosage, adverse effects)
• Middle Aged
• Respiration, Artificial
• Retrospective Studies
• Risk Factors
• Time Factors
• Treatment Outcome
• Young Adult