Objectives: The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases involving multiple systems, and kidney is one of the most commonly involved target organs. Some patients may rapidly progress to end-stage renal disease in a short time. Whereas some patients have poor remission and renal prognosis after standard induction therapy. As a selective blood purification therapy, protein a immunoadsorption (PAIA) has shown great advantages on treating of severe autoimmune diseases. This study aims to evaluate the short-term efficacy of PAIA therapy combined with glucocorticoids and immunosuppressants on treating of severe AAV renal injury.
Methods: A total of 10 AAV cases with severe kidney involvement in the Second Xiangya Hospital of Central South University from 2019 to 2020 were selected for this retrospective study. During the induction remission stage, each patient was treated with PAIA on the basis of standard therapy of glucocorticoids and immunosuppressive treatment. Before and after the initial treatment, 1 month and 3 months after treatment, clinical data including demographic characteristics, immunological indicators, and Birmingham Vasculitis Activity Score (BVAS) were compared. The related adverse reactions during treatment were recorded to evaluate the short-term efficacy.
Results: In this study, all 10 patients were MPO positive, and 2 patients were PR3 positive (≥2.3 U/mL). There are 6 males and 4 females at (61.5±11.4) years old, with the median time from onset to admission to hospital at (2.8±1.8) months. Multisystem damage, especially kidney damage, can be seen with eGFR lower than 30 mL/(min·1.73 m2). During the 3-month follow-up, BVAS, erythrocyte sedimentation rate, and hemoglobin of 10 patients showed continuous improvement compared with the initial admission levels (all P<0.05). ANCA titer, serum creatinine and urine red blood cell were all decreased and eGFR levels were increased in 3 months after treatment (all P<0.05). Serum albumin, urinary protein, C-reactive protein and complement levels showed no significant changes after treatment (all P>0.05). One patient who had received renal replacement therapy was still dialysis dependent after PAIA treatment. One patient who had transient hypotension was corrected by routine treatment. The rest of the patients were all tolerant with PAIA during their treatments.
Conclusions: Treatment with PAIA combined with glucocorticoids and cyclophosphamide can rapidly lower serum ANCA level and improve disease activity in patients with AAV complicated with severe kidney damage, suggesting a good short-term renal prognosis and good overall safety.
目的: 抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody，ANCA)相关性血管炎(ANCA-associated vasculitis，AAV)是一组累及多系统及器官的疾病，肾是最常受累的靶器官之一，部分患者短期内迅速进展至终末期肾病。既往标准诱导缓解方案治疗后仍有部分患者的缓解情况及肾脏预后较差。蛋白A免疫吸附(protein A immunoadsorption，PAIA)是一种选择性血液净化方式，在多种重症自身免疫系统疾病治疗方面表现出巨大的优势。本研究旨在探讨PAIA联合激素及免疫抑制剂在重症AAV肾损伤中的短期疗效。方法: 回顾性纳入中南大学湘雅二医院于2019至2020年间收治的10例重症AAV肾损伤患者。在诱导缓解期对患者在使用标准激素联合免疫抑制剂治疗的基础上联合PAIA治疗，于初次治疗前、PAIA治疗后、PAIA治疗后1个月及3个月时分别收集患者的人口学特征，比较治疗前后患者的免疫学指标、伯明翰血管炎活动性评分(Birmingham Vasculitis Aactivity Score，BVAS)及相关临床指标等变化，并记录其不良反应情况，评估该疗法的短期疗效。结果: 10例患者中男性6例，女性4例，年龄为(61.5±11.4)岁，从发病到确诊治疗的时长为(2.8±1.8)个月，均为抗髓过氧化物酶抗体阳性，2例合并抗蛋白酶３(anti-proteinase 3，PR3)抗体阳性(≥2.3 U/mL)。10例患者普遍表现为多器官受累，肾损伤严重，估算肾小球滤过率(estimated glomerular filtration rate，eGFR)均<30 mL/(min·1.73 m2)。随访3个月期间，10例患者BVAS、红细胞沉降率(erythrocyte sedimentation rate，ESR)及血红蛋白较治疗前的水平均可见持续改善(均P<0.05)。与治疗前相比，治疗3个月后患者的ANCA滴度、血清肌酐及尿红细胞水平均下降，eGFR水平回升(均P<0.05)；患者的血清白蛋白、C反应蛋白及补体水平在治疗前后的差异均无统计学意义(均P>0.05)。1例患者纳入时已进入肾脏替代治疗阶段的患者治疗后未脱离维持性透析；1例患者在治疗过程中出现一过性低血压，对症处理后好转；余患者治疗过程中耐受性良好。结论: PAIA联合激素和环磷酰胺治疗可快速降低血清ANCA水平，有效改善AAV合并肾损伤患者疾病活动情况，提示较好的短期肾脏预后，且整体安全性良好。.
Keywords: anti-neutrophil cytoplasmic antibody-associated vasculitis; curative effect; kidney injury; protein A immunoadsorption.