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

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.