TOPLINE:

Patients with acute exacerbations of idiopathic pulmonary fibrosis (IPF) who used corticosteroids had a higher risk for mortality. Lower corticosteroid doses and background antifibrotic therapy were associated with improved short-term survival.

METHODOLOGY:

• Researchers conducted a systematic review and meta-analysis to quantify rates of short‑ and long‑term mortality after treatment with corticosteroids for acute exacerbations of IPF and to evaluate how steroid dose, background antifibrotic therapy, and concomitant immunosuppression affected the outcomes.
• The analysis included 33 studies (28 retrospective studies, two prospective studies, and three randomized controlled trials) comprising a range of 9-5616 patients with acute exacerbations of IPF (range of mean or median age, 64.3-78.0 years; 3.0%-95.4% men).
• All participants received various corticosteroid regimens, most commonly methylprednisolone pulses at 1000 mg/d for 3 days (40.6% of studies) or methylprednisolone at 500-1000 mg/d (10 mg/kg/d) for 3 days (31.3% of studies), with almost all participants receiving concomitant antibiotics and some receiving immunosuppressive therapy.
• The outcomes analyzed were 90-day, 1-year, and in-hospital mortality.

TAKEAWAY:

• Among participants treated with corticosteroids alone, the pooled rate of 90-day mortality was 42% (95% CI, 0.19%-0.67%; nine studies), and both the pooled rate of in-hospital mortality and overall rate of 1-year mortality were 43% (95% CI, 0.30%-0.56%; seven studies).
• The pooled rate of 90-day mortality among participants treated with 1000 mg/d methylprednisolone was 54% (95% CI, 0.11%-0.94%; three studies) compared with 39% (95% CI, 0.12%-0.71%; five studies) among those treated with 500-1000 mg/d methylprednisolone.
• Participants with background antifibrotic therapy had a lower pooled rate of 90-day mortality (39%; 95% CI, 0.25%-0.54%; five studies) than those without such therapy (49%; 95% CI, 0.10%-0.89%; four studies).
• The pooled rate of 90-day mortality for participants who received concomitant immunosuppressive therapy was 37% (95% CI, 0.29%-0.46%; four studies), and the rate of in-hospital mortality was 35% (95% CI, 0.12%-0.62%; five studies), both lower than for participants who received corticosteroids alone.

IN PRACTICE:

“Patients treated with immunosuppressants and corticosteroids show better in-hospital and 90-day survival than those with only steroids,” the authors of the study wrote.

SOURCE:

The study was led by Michele Mondoni, Università degli Studi di Milano, Milan, Italy. It was published online on April 9, 2026, in Respiratory Research. 

LIMITATIONS:

This analysis was limited by small observational studies, most of which were retrospective in nature. Variations in the definitions of IPF and acute exacerbations, steroid regimens, and background therapies across studies may have limited comparability. Several datasets lacked separable data on outcomes for subgroups (for example, patients who received immunosuppressants or antifibrotics), which prevented drawing definitive conclusions about dose-response or treatment interactions.

DISCLOSURES:

No funding information was provided for this study. One author reported being an associate editor of Respiratory Research. 

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.