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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Public Library of Science (PLoS)
2021-01-01
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| 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 |
| collection | DOAJ |
| 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 |
| id | doaj-art-85f900e1784f4301bb0c8b174ccf412b |
| institution | DOAJ |
| issn | 1932-6203 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Public Library of Science (PLoS) |
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| spelling | doaj-art-85f900e1784f4301bb0c8b174ccf412b2025-08-20T02:54:29ZengPublic Library of Science (PLoS)PLoS ONE1932-62032021-01-011611e026034510.1371/journal.pone.0260345Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.Tejaswini KulkarniVincent G ValentineFei FeiThi K Tran-NguyenLuisa D Quesada-AriasTakudzwa MkorombindoHuy P PhamSierra C SimmonsKevin G DsouzaTracy LuckhardtSteven R Duncan<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.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0260345&type=printable |
| spellingShingle | 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. PLoS ONE |
| title | 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_short | Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations. |
| title_sort | correlates of survival after autoantibody reduction therapy for acute ipf exacerbations |
| url | https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0260345&type=printable |
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