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07-03-2019 | Respiratory | News | Article

No survival benefit of add-on cyclophosphamide following IPF exacerbation

medwireNews: The addition of intravenous cyclophosphamide to corticosteroid treatment is not associated with significantly improved survival among patients with an acute exacerbation of idiopathic pulmonary fibrosis (IPF), researchers report.

The retrospective study included 102 IPF patients diagnosed with a first idiopathic acute exacerbation at one of three Japanese centres between 2004 and 2017 and followed up for more than 1 year or until death. A total of 85 (83.3%) patients died during the study, most commonly due to exacerbation-related respiratory failure (50.0%), followed by chronic respiratory failure (18.6%), other causes (7.8%) and infection (6.9%).

As reported in Respirology, Hironao Hozumi (Hamamatsu University School of Medicine, Japan) and co-authors used a propensity score matching approach to compare 26 patients treated with intravenous cyclophosphamide plus corticosteroids with 26 patients given corticosteroids alone, finding no significant differences in survival between the groups.

Specifically, 76.9% of patients in the combination therapy group were alive 90 days after experiencing an acute exacerbation, compared with 84.6% of those treated with corticosteroids only. Moreover, there was no significant difference in survival probability at 180 and 360 days, with rates of 69.2% versus 69.2% and 61.5% versus 53.9%, respectively.

The proportion of patients experiencing adverse events of grade 3 or higher was also comparable between the combination and corticosteroid-only groups (65.4 vs 61.5%, respectively), including pneumothorax (19.2 vs 23.1%), infection (30.8 vs 19.3%) and hyperglycaemia (42.3 vs 30.8%).

“These findings suggest that the concurrent use of [intravenous cyclophosphamide] with [corticosteroids] has no additional benefit in terms of short-term survival”, and thus “the routine use of this combination therapy for patients with IPF with a first episode of idiopathic [acute exacerbation] might not be recommended”, write Hozumi and colleagues.

They note, however, that their study was not able to evaluate the impact of intravenous cyclophosphamide on longer-term survival, nor “its steroid sparing effect or its potential as salvage therapy against initial treatment-refractory cases.”

They conclude that their findings “could inform the design of future studies to establish optimal therapeutic strategies” for patients with acute exacerbations of IPF.

By Claire Barnard

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