Centre de Référence des Maladies Systémiques Auto-immunes rares, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Institut Cochin, INSERM U1016, CNRS UMR 8104, Paris, France.
Joint Bone Spine. 2020 Dec;87(6):572-578. doi: 10.1016/j.jbspin.2020.06.005. Epub 2020 Jun 17.
Granulomatosis with polyangiitis (GPA) (Wegener) is a necrotizing vasculitis combining inflammation of the vascular wall and peri- and extravascular granulomatosis. Clinically, GPA is characterized in its full form by ENT signs, lung, and kidney involvement. One of the key features of GPA is the presence of ANCAs-cytoplasmic in approximately 90% of systemic forms and in 50% of localized forms-directed against proteinase 3 in most cases. PR3-ANCAs are highly specific to GPA and therefore have a high diagnostic value. Treatment of GPA is based on a combination of corticosteroids and cyclophosphamide or rituximab, which allows remission to be achieved in more than 80% of cases. Azathioprine was the most widely used maintenance treatment, but low-dose semi-annual rituximab maintenance infusions further decrease relapses with acceptable safety. Nevertheless, relapses occur in more than 50% of cases. One of the biggest treatment challenges is the occurrence of side effects, the severity and frequency of which are often linked to the prolonged treatment course, which is difficult to avoid.
肉芽肿性多血管炎（GPA）（韦格纳）是一种坏死性血管炎，合并血管壁炎症和血管外及血管周围肉芽肿。临床上，GPA 以其完全形式表现为 ENT 症状、肺部和肾脏受累。GPA 的一个关键特征是存在 ANCAs-细胞质，约 90%的系统性形式和 50%的局部形式，大多数情况下针对蛋白酶 3。PR3-ANCAs 对 GPA 高度特异，因此具有很高的诊断价值。GPA 的治疗基于皮质类固醇和环磷酰胺或利妥昔单抗的联合治疗，这使得 80%以上的病例能够缓解。硫唑嘌呤是最广泛使用的维持治疗药物，但低剂量半年一次的利妥昔单抗维持输注进一步降低了复发率，且安全性可接受。然而，仍有超过 50%的病例复发。最大的治疗挑战之一是发生副作用，其严重程度和频率通常与延长治疗过程有关，而这是难以避免的。
