Chaleshtori Maryam Tavakoli, Farajzadegan Ziba, Salesi Mansour
Department of Internal Medicine, Isfahan University of Medical Sciences, School of Medicine, Isfahan, Iran.
Department of Community and Family Medicine, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Eur J Rheumatol. 2022 Apr;9(2):88-92. doi: 10.5152/eurjrheum.2022.21063.
Granulomatosis with polyangiitis (GPA), formerly known as Wegner's granulomatosis, is a rare vasculitic syndrome classified under Anti-Neutrophilic Cytoplasmic Autoantibody (ANCA)-associ- ated vasculitides, which is fatal if untreated. The mainstay of treatment consists of immunosuppression using a combination of corticosteroids with either rituximab (RTX) or cyclophosphamide (CYC). We aimed to compare the 4-year clinical outcomes between patients with GPA receiving CYC and RTX as remission induction.
In this retrospective cohort, we used patient data from 92 patients with GPA at two large teaching hospitals and a private clinic in Isfahan, Iran. The patients were classified based on the medi- cation they received for remission induction into RTX and CYC groups. The main outcomes were rate of death and relapse, disease activity assessed based on the Birmingham Vasculitis Activity Score (BVAS), disease-related complications, laboratory markers, and adverse-drug-reactions.
Fifty-three (57.6%) patients received CYC, whereas 39 (42.4%) received RTX. The mean duration of follow-up was 3.6 (62) years. Most of patients (70%) had a successful remission, while 20.7% experi- enced a relapse and 8.7% of patients died. The rate of death and relapse did not differ between the RTX and CYC groups. Disease-related complications involved an insignificantly higher proportion of patients in the CYC (12/53) group than the RTX (4/39) group. Patients in both groups showed a signifi- cant decrease in BVAS during follow-ups irrespective of the medication exposure. The rate of adverse events was similarly low (n 1⁄4 1) in both groups.
RTX and CYC were similar in inducing remission and reducing adverse clinical outcomes among patients with GPA with acceptable side effect profiles.
肉芽肿性多血管炎(GPA),以前称为韦格纳肉芽肿,是一种罕见的血管炎综合征,归类于抗中性粒细胞胞浆自身抗体(ANCA)相关血管炎,若不治疗则会致命。治疗的主要方法是使用皮质类固醇与利妥昔单抗(RTX)或环磷酰胺(CYC)联合进行免疫抑制。我们旨在比较接受CYC和RTX作为缓解诱导治疗的GPA患者的4年临床结局。
在这项回顾性队列研究中,我们使用了伊朗伊斯法罕两家大型教学医院和一家私人诊所92例GPA患者的数据。根据患者接受的缓解诱导药物将其分为RTX组和CYC组。主要结局包括死亡率和复发率、根据伯明翰血管炎活动评分(BVAS)评估的疾病活动度、疾病相关并发症、实验室指标和药物不良反应。
53例(57.6%)患者接受CYC治疗,而39例(42.4%)接受RTX治疗。平均随访时间为3.6(62)年。大多数患者(70%)成功缓解,20.7%的患者复发,8.7%的患者死亡。RTX组和CYC组的死亡率和复发率无差异。疾病相关并发症在CYC组(12/53)中的患者比例略高于RTX组(4/39)。两组患者在随访期间BVAS均显著下降,与用药情况无关。两组的不良事件发生率同样较低(n = 1)。
在诱导缓解和降低GPA患者不良临床结局方面,RTX和CYC相似,且副作用可接受。