Patients with idiopathic pulmonary fibrosis (IPF) who develop acute exacerbations have a higher mortality rate than patients with other interstitial lung diseases (ILDs) who experience similar respiratory deterioration (43% vs 19%), according to a new study published in the journal Diagnostics.

This conclusion is from a retrospective study that engaged 158 ILD patients (IPF, 38%, non-IPF, 62%) who were hospitalized due to acute exacerbations in 2 Italian ILD referral centers, the University Hospitals of Monza and Modena.

The analysis performed by Faverio et al identified increased inflammatory markers, such as neutrophilia
on the differential blood cell count (hazard ratio [HR], 1.02, CI, 1.01-1.04), pulmonary hypertension (HR, 1.85, CI, 1.17-2.92), and the diagnosis of IPF (HR, 2.31, CI, 1.55-3.46) as negative prognostic factors.

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“There are no evidence-based therapeutic strategies for the management of [acute exacerbations], and the treatment does not differ between the two groups; all patients were treated with high flow oxygen, a high dose of intravenous corticosteroids, and broad-spectrum antibiotics. Immunosuppressant therapy was administered only in 7 patients in the IPF and in 16 in the non-IPF group,” the authors explained.

The estimated annual incidence of acute exacerbations (ie, acute respiratory deterioration) in IPF patients is 5%-19%. Patients have a poor prognosis, with a median survival inferior to 1 year.

Most patients experienced acute exacerbations before the inclusion of antifibrotic drugs (pirfenidone or nintedanib) in their treatment regimen. Given the effect of these drugs on controlling disease progression, it is expected that they might have a protective role against acute exacerbations. Therefore, it is reasonable to expect a decrease in the incidence of acute exacerbations with the widespread use of antifibrotic drugs, according to the authors.

Acute exacerbations of ILDs are events that are difficult to predict and associated with high mortality, the authors said. As a result, future prospective, large-scale, and real-world data are essential to improve current knowledge.


Faverio P, Stainer A, Conti S, et al. Differences between acute exacerbations of idiopathic pulmonary fibrosis and other interstitial lung diseases. Diagnostics. 2021;11(9). doi:10.3390/diagnostics11091623