Beneficial clinical responses were reported when treating acute exacerbations of idiopathic pulmonary fibrosis (IPF) with autoantibody reduction therapy, according to a recent study published in PLOS One.

Patients (n=10 who survived for at least 1 year) experienced a rapid reduction in the need for supplemental oxygen flow to maintain a resting arterial oxygen saturation >92% and/or an improvement in the ability to ambulate after treatment.

In this study, the authors used a triple-modality autoantibody reduction regimen consisting of 9 therapeutic plasma exchanges, 2 doses of rituximab, and 4 intravenous immunoglobulin infusions. The therapeutic regimen was well-tolerated by patients with IPF with acute exacerbations.

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“Incremental trials could further refine and optimize autoantibody reduction regimens, validate measures to facilitate the personalized applications of these treatments, and potentially illuminate other important upstream disease mechanisms aside from or in addition to humoral autoimmunity,” the authors said.

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Kulkarni et al found that survivors had nearly 3-fold higher plasma anti-HEp-2 autoantibody titers than nonsurvivors (P <.02). Most (75%) of the evaluated 1-year survivors presented with anti-HEp-2 titers >1:160, compared to 29% of nonsurvivors.

Moreover, 83% of patients with anti-HEP-2 titers <1:160 died during the observation period (hazard ratio, 3.3, 95% CI, 1.02-10.6, P =.047).

“Although this study does not have a control cohort, the rapid response to these therapies, and cumulative one-year survival (42%) appears to be an improvement compared to published series of responses and outcomes for conventionally-treated [acute exacerbations of IPF] patients, including our previous compilation of comparable [acute exacerbations of IPF] historical controls, in whom one-year survival was zero,” the authors highlighted.

It is estimated that 5%-10% of patients with IPF develop fulminant exacerbations of their lung disease annually. These episodes can lead to respiratory failure within days and account for nearly half of all deaths among patients with IPF.

The short-term mortality of acute exacerbations in patients with IPF may be as great as 90% or even more.


Kulkarni T, Valentine VG, Fei F, et al. Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations. PLOS One. 2021;16(11):e0260345. doi:10.1371/journal.pone.0260345