Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.

<h4>Background</h4>No medical treatment has proven efficacy for acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), and this syndrome has a very high mortality. Based on data indicating humoral autoimmune processes are involved in IPF pathogenesis, we treated AE-IPF patients w...

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Autores principales: Tejaswini Kulkarni, Vincent G Valentine, Fei Fei, Thi K Tran-Nguyen, Luisa D Quesada-Arias, Takudzwa Mkorombindo, Huy P Pham, Sierra C Simmons, Kevin G Dsouza, Tracy Luckhardt, Steven R Duncan
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spelling oai:doaj.org-article:85f900e1784f4301bb0c8b174ccf412b2021-12-02T20:16:13ZCorrelates of survival after autoantibody reduction therapy for acute IPF exacerbations.1932-620310.1371/journal.pone.0260345https://doaj.org/article/85f900e1784f4301bb0c8b174ccf412b2021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0260345https://doaj.org/toc/1932-6203<h4>Background</h4>No medical treatment has proven efficacy for acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), and this syndrome has a very high mortality. Based on data indicating humoral autoimmune processes are involved in IPF pathogenesis, we treated AE-IPF patients with an autoantibody reduction regimen of therapeutic plasma exchange, rituximab, and intravenous immunoglobulin. This study aimed to identify clinical and autoantibody determinants associated with survival after autoantibody reduction in AE-IPF.<h4>Methods</h4>Twenty-four(24) AE-IPF patients received the autoantibody reduction regimen. Plasma anti-epithelial autoantibody titers were determined by HEp-2 indirect immunofluorescence assays in 22 patients.<h4>Results</h4>Mean age of the patients was 70 + 7 years old, and 70% were male. Beneficial clinical responses that occurred early during therapy were a favorable prognostic indicator: supplemental O2 flows needed to maintain resting SaO2>92% significantly decreased and/or walk distances increased among all 10 patients who survived for at least one year. Plasma anti-HEp-2 autoantibody titers were ~-three-fold greater in survivors compared to non-survivors (p<0.02). Anti-HEp-2 titers >1:160 were present in 75% of the evaluable one-year survivors, compared to 29% of non-survivors, and 10 of 12 patients (83%) with anti-HEP-2 titers <1:160 died during the observation period (Hazard Ratio = 3.3, 95% Confidence Interval = 1.02-10.6, p = 0.047).<h4>Conclusions</h4>Autoantibody reduction therapy is associated with rapid reduction of supplemental oxygen requirements and/or improved ability to ambulate in many AE-IPF patients. Facile anti-epithelial autoantibody assays may help identify those most likely to benefit from these treatments.Tejaswini KulkarniVincent G ValentineFei FeiThi K Tran-NguyenLuisa D Quesada-AriasTakudzwa MkorombindoHuy P PhamSierra C SimmonsKevin G DsouzaTracy LuckhardtSteven R DuncanPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 11, p e0260345 (2021)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Tejaswini Kulkarni
Vincent G Valentine
Fei Fei
Thi K Tran-Nguyen
Luisa D Quesada-Arias
Takudzwa Mkorombindo
Huy P Pham
Sierra C Simmons
Kevin G Dsouza
Tracy Luckhardt
Steven R Duncan
Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
description <h4>Background</h4>No medical treatment has proven efficacy for acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), and this syndrome has a very high mortality. Based on data indicating humoral autoimmune processes are involved in IPF pathogenesis, we treated AE-IPF patients with an autoantibody reduction regimen of therapeutic plasma exchange, rituximab, and intravenous immunoglobulin. This study aimed to identify clinical and autoantibody determinants associated with survival after autoantibody reduction in AE-IPF.<h4>Methods</h4>Twenty-four(24) AE-IPF patients received the autoantibody reduction regimen. Plasma anti-epithelial autoantibody titers were determined by HEp-2 indirect immunofluorescence assays in 22 patients.<h4>Results</h4>Mean age of the patients was 70 + 7 years old, and 70% were male. Beneficial clinical responses that occurred early during therapy were a favorable prognostic indicator: supplemental O2 flows needed to maintain resting SaO2>92% significantly decreased and/or walk distances increased among all 10 patients who survived for at least one year. Plasma anti-HEp-2 autoantibody titers were ~-three-fold greater in survivors compared to non-survivors (p<0.02). Anti-HEp-2 titers >1:160 were present in 75% of the evaluable one-year survivors, compared to 29% of non-survivors, and 10 of 12 patients (83%) with anti-HEP-2 titers <1:160 died during the observation period (Hazard Ratio = 3.3, 95% Confidence Interval = 1.02-10.6, p = 0.047).<h4>Conclusions</h4>Autoantibody reduction therapy is associated with rapid reduction of supplemental oxygen requirements and/or improved ability to ambulate in many AE-IPF patients. Facile anti-epithelial autoantibody assays may help identify those most likely to benefit from these treatments.
format article
author Tejaswini Kulkarni
Vincent G Valentine
Fei Fei
Thi K Tran-Nguyen
Luisa D Quesada-Arias
Takudzwa Mkorombindo
Huy P Pham
Sierra C Simmons
Kevin G Dsouza
Tracy Luckhardt
Steven R Duncan
author_facet Tejaswini Kulkarni
Vincent G Valentine
Fei Fei
Thi K Tran-Nguyen
Luisa D Quesada-Arias
Takudzwa Mkorombindo
Huy P Pham
Sierra C Simmons
Kevin G Dsouza
Tracy Luckhardt
Steven R Duncan
author_sort Tejaswini Kulkarni
title Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
title_short Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
title_full Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
title_fullStr Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
title_full_unstemmed Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
title_sort correlates of survival after autoantibody reduction therapy for acute ipf exacerbations.
publisher Public Library of Science (PLoS)
publishDate 2021
url https://doaj.org/article/85f900e1784f4301bb0c8b174ccf412b
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