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

Correlates of survival after autoantibody reduction therapy for acute IPF exacerbations.
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DOI:
10.1371/journal.pone.0260345
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发表时间:
2021
期刊:
影响因子:
3.7
通讯作者:
Duncan SR
Duncan SR
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Kulkarni T;Valentine VG;Fei F;Tran-Nguyen TK;Quesada-Arias LD;Mkorombindo T;Pham HP;Simmons SC;Dsouza KG;Luckhardt T;Duncan SR

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特发性肺纤维化急性加重期(AE-IPF)目前尚无有效的内科治疗方法,且病死率极高。基于体液自身免疫过程参与IPF发病机制的数据,我们采用治疗性血浆交换、利妥昔单抗和静脉免疫球蛋白的自身抗体减少方案治疗AE-IPF患者。这项研究的目的是确定临床和自身抗体决定因素与AE-IPF自身抗体减少后的生存相关。24例AE-IPF患者接受自身抗体减量方案治疗。用Hep-2间接免疫荧光法测定22例患者血浆抗上皮细胞自身抗体效价。患者平均年龄为70±7岁,其中70%为男性。治疗早期出现的有益的临床反应是一个有利的预后指标:在所有存活至少一年的10名患者中,维持静息SaO2和GT所需的补充O2流量显著减少和/或步行距离增加92%。存活患者的血浆抗Hep-2自身抗体效价是死亡患者的~-3倍(p<0.02)。在可评估的一年存活者中,75%的患者抗-HEP-2滴度和1:160值存在,而死亡患者的这一比例为29%。在观察期间,12名抗-HEP-2滴度和1:160值的患者中有10人(83%)死亡(危险比=3.395%可信区间=1.0210.6,p=0.047)。在许多AE-IPF患者中,自身抗体减少治疗与快速减少补充氧需求和/或改善行走能力有关。简便的抗上皮细胞自身抗体检测可能有助于识别那些最有可能从这些治疗中受益的人。
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. 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. 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). 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.
DOI: 10.1371/journal.pone.0154516
发表时间: 2016
期刊: PloS one
影响因子: 3.7
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DOI: 10.1164/rccm.201203-0506oc
发表时间: 2013-04-01
影响因子: 24.7
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DOI: 10.1016/j.jaut.2014.08.003
发表时间: 2015-01-01
影响因子: 12.8
作者:
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