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接受治疗性血浆置换的抗体阳性系统性血管炎患者的预后

Patient outcome in antibody-positive systemic vasculitis treated with therapeutic plasma exchange.

作者信息

Tiwari Aseem Kumar, Setya Divya, Tanna Dhaval, Arora Dinesh, Aggarwal Geet, Gupta Rajiva, Bansal Shyam Bihari, Sethi Sidharth Kumar

机构信息

Department of Transfusion Medicine, Medanta - The Medicity, Gurgaon, Haryana, India.

Department of Rheumatology and Clinical Immunology, Medanta - The Medicity, Gurgaon, Haryana, India.

出版信息

Asian J Transfus Sci. 2023 Jan-Jun;17(1):79-84. doi: 10.4103/ajts.AJTS_50_21. Epub 2023 Mar 1.

Abstract

BACKGROUND

Therapeutic plasma exchange (TPE) has been advocated as an adjunct to steroids and cytotoxic drugs in treating patients suffering from vasculitis and presenting with active disease, but we still have insufficient evidence on its effectiveness in improving the clinical response, especially in India. This study was planned to study the clinical outcome in severe vasculitic presentations treated with TPE as an adjunctive therapy.

MATERIALS AND METHODS

A retrospective analysis of TPE procedures performed from July 2013 to July 2017 in the department of transfusion medicine at a large tertiary care hospital was done. All consecutive patients admitted with new diagnosis of systemic vasculitis presenting with active disease and severe presentations such as advanced renal failure or severe respiratory abnormalities or life-threatening vasculitis affecting the gastrointestinal tract, neurological and musculoskeletal system; who needed TPE for removal of preformed antibodies, were included in the study.

RESULTS

There were a total of 31 patients in whom TPE was performed for severe systemic vasculitis; 26 adults and five pediatric. Six patients tested positive for perinuclear fluorescence, 13 for cytoplasmic fluorescence (cANCA), two for atypical antineutrophil cytoplasmic autoantibody, seven for anti-glomerular basement membrane antibodies, two for antinuclear antibodies (ANA), and one patient tested positive for ANA as well as cANCA before the augmentation of TPE. Out of 31, seven patients showed no clinical improvement and succumbed to the disease. At the end of desired number of procedures, 19 tested negative and five tested weak positive for their respective antibodies.

CONCLUSION

Favorable clinical outcomes were observed with TPE in patients with antibody-positive systemic vasculitis.

摘要

背景

治疗性血浆置换(TPE)已被提倡作为类固醇和细胞毒性药物的辅助手段,用于治疗患有血管炎且处于疾病活动期的患者,但我们仍缺乏其改善临床反应有效性的充分证据,尤其是在印度。本研究旨在探讨TPE作为辅助治疗手段对重症血管炎患者的临床疗效。

材料与方法

对一家大型三级医疗中心输血医学科2013年7月至2017年7月期间进行的TPE操作进行回顾性分析。纳入所有新诊断为系统性血管炎且处于疾病活动期、伴有严重表现(如晚期肾衰竭、严重呼吸异常或危及生命的影响胃肠道、神经和肌肉骨骼系统的血管炎)、需要进行TPE以清除预先形成抗体的连续患者。

结果

共有31例患者因重症系统性血管炎接受了TPE治疗,其中26例为成人,5例为儿童。6例患者核周荧光检测呈阳性,13例胞质荧光(cANCA)检测呈阳性,2例非典型抗中性粒细胞胞浆自身抗体检测呈阳性,7例抗肾小球基底膜抗体检测呈阳性,2例抗核抗体(ANA)检测呈阳性,1例患者在增加TPE治疗前ANA和cANCA检测均呈阳性。31例患者中,7例临床症状未改善并最终死亡。在完成预期治疗次数后,19例患者各自的抗体检测呈阴性,5例呈弱阳性。

结论

抗体阳性的系统性血管炎患者接受TPE治疗后观察到了良好的临床疗效。

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