National Referral Center for Rare Systemic Autoimmune Diseases, Université de Paris, Hôpital Cochin, AP-HP, Paris, France.
Hôpital Européen Georges Pompidou, AP-HP, Paris, France.
Arthritis Rheumatol. 2021 Apr;73(4):641-650. doi: 10.1002/art.41551. Epub 2021 Feb 28.
Data on sustained remission of granulomatosis with polyangiitis (GPA) after discontinuation of therapy (referred to as GPA with sustained remission off-therapy [SROT]) are scarce. In the present study, SROT among GPA patients from the French Vasculitis Study Group Registry was evaluated to identify factors associated with its occurrence and durability.
For inclusion of patients in the study, the diagnosis of GPA had to meet the GPA classification criteria defined by the American College of Rheumatology and/or the revised Chapel Hill Consensus Conference nomenclature for vasculitis. SROT was defined as achievement of remission (a Birmingham Vasculitis Activity Score of 0) that was sustained for ≥6 consecutive months after having discontinued glucocorticoid (GC) and immunosuppressant treatments. The characteristics of the patients at baseline and treatments received were compared at 3, 5, and 10 years postdiagnosis according to whether or not SROT had been reached and maintained.
Among 795 patients with GPA, 92 GPA patients with SROT at 3 years postdiagnosis were compared to 342 control subjects who had experienced disease relapse and/or were still receiving GCs or immunosuppressants. No baseline differences were found, but patients with SROT at 3 years postdiagnosis had more frequently received intravenous cyclophosphamide as induction therapy compared to control subjects (P = 0.01), with a higher median number of infusions (P = 0.05). At 5 years postdiagnosis, no baseline differences were observed between groups, but patients with SROT at 5 years postdiagnosis had received more cyclophosphamide infusions compared to control subjects (P = 0.03). More patients with SROT had received rituximab as maintenance therapy than control subjects at 3 years and 5 years postdiagnosis (P = 0.09 and P < 0.001, respectively). Of the 74 patients enrolled in the GPA Registry with 10-year follow-up data after having received conventional maintenance therapy, 15 (20%) had reached SROT at 3 years, and 5 (7%) maintained SROT at 10 years postdiagnosis.
After conventional therapies, 7% of GPA patients had reached SROT at 10 years postdiagnosis. No baseline vasculitis characteristics distinguished patients who achieved/maintained SROT from those who experienced disease relapse and/or those who continued to receive GCs or immunosuppressant therapy, but patients with SROT had received more intensive induction therapy and rituximab as maintenance therapy more frequently.
关于在停止治疗后（被称为治疗后持续性缓解的肉芽肿性多血管炎[GPA] [SROT]），GPA 缓解的持续性的数据很少。在本研究中，评估了法国血管炎研究组登记处 GPA 患者的 SROT，以确定与 SROT 发生和持续相关的因素。
为了将患者纳入研究，GPA 的诊断必须符合美国风湿病学会定义的 GPA 分类标准和/或修订的 Chapel Hill 共识会议血管炎命名法。SROT 定义为在停止糖皮质激素（GC）和免疫抑制剂治疗后连续 6 个月以上达到缓解（伯明翰血管炎活动评分 0）。根据是否达到和维持 SROT，比较患者在诊断后 3、5 和 10 年的基线特征和治疗情况。
在 795 例 GPA 患者中，比较了 92 例在诊断后 3 年达到 SROT 的 GPA 患者与 342 例疾病复发和/或仍接受 GC 或免疫抑制剂治疗的对照患者。未发现基线差异，但与对照患者相比，在诊断后 3 年达到 SROT 的患者更频繁地接受静脉注射环磷酰胺作为诱导治疗（P=0.01），输注中位数更高（P=0.05）。在诊断后 5 年，两组之间未观察到基线差异，但在诊断后 5 年达到 SROT 的患者接受的环磷酰胺输注次数多于对照组（P=0.03）。与对照组相比，在诊断后 3 年和 5 年，更多的 SROT 患者接受了利妥昔单抗作为维持治疗（P=0.09 和 P<0.001）。在接受常规维持治疗并随访 10 年的 74 例 GPA 登记患者中，有 15 例（20%）在诊断后 3 年达到 SROT，5 例（7%）在诊断后 10 年维持 SROT。
在常规治疗后，7%的 GPA 患者在诊断后 10 年达到 SROT。没有基线血管炎特征可以区分达到/维持 SROT 的患者与疾病复发和/或继续接受 GC 或免疫抑制剂治疗的患者，但 SROT 患者接受了更强化的诱导治疗和利妥昔单抗作为维持治疗。
