Fain Olivier, Hamidou Mohamed, Cacoub Patrice, Godeau Bertrand, Wechsler Bertrand, Pariès Jacques, Stirnemann Jérôme, Morin Anne-Sophie, Gatfosse Marc, Hanslik Thomas, Belmatoug Nadia, Blétry Olivier, Cevallos Ramiro, Delevaux Isabelle, Fisher Evelyne, Hayem Gilles, Kaplan Gérard, Le Hello Claire, Mouthon Luc, Larroche Claire, Lemaire Véra, Piette Anne-Marie, Piette Jean-Charles, Ponge Thierry, Puechal Xavier, Rossert Jérôme, Sarrot-Reynauld Françoise, Sicard Didier, Ziza Jean-Marc, Kahn Marcel-Francis, Guillevin Loïc
Assistance Publique Hôpitaux de Paris, Hôpital Jean-Verdier, Université Paris, Bondy, France.
Arthritis Rheum. 2007 Dec 15;57(8):1473-80. doi: 10.1002/art.23085.
To describe characteristics and outcomes of vasculitides associated with malignancies.
The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy.
Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schönlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02).
Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.
描述与恶性肿瘤相关的血管炎的特征及预后。
本项为期10年的回顾性研究纳入标准为患有进展性恶性肿瘤的患者发生血管炎。排除因用于治疗血管炎的免疫抑制剂导致的继发性恶性肿瘤。根据恶性肿瘤类型分析并比较血管炎的主要特征。
共纳入60例患者（男女比例为2.53，平均年龄62.4岁）。平均随访时间为45.2个月。血管炎类型包括皮肤白细胞破碎性血管炎（45%）、结节性多动脉炎（36.7%）、韦格纳肉芽肿（6.7%）、显微镜下多血管炎（5%）和过敏性紫癜（5%）。恶性肿瘤分布如下：血液系统肿瘤占63.1%，骨髓增生异常综合征（MDS）占32.3%，淋巴系统肿瘤占29.2%，实体瘤占36.9%。38%的病例中血管炎与恶性肿瘤同时诊断。血管炎的表现包括发热（41.7%）、皮肤受累（78.3%）、关节痛（46.7%）、周围神经病变（31.7%）、肾脏受累（23.3%；肾小球肾炎11.7%、微动脉瘤11.7%、肾功能不全6.7%）以及抗中性粒细胞胞浆抗体（20.4%）。血管炎治疗方法为使用糖皮质激素（78.3%）和免疫抑制剂（41.7%）。65%的患者血管炎治愈，但58.3%的患者死亡，其中1例死于血管炎。与其他恶性肿瘤相关的血管炎患者相比，无论亚型如何，与MDS相关的血管炎患者更常出现肾脏表现（P = 0.02）和激素依赖（P = 0.04），且完全缓解的频率更低（P = 0.04）。与实体瘤相关的血管炎患者更常出现周围神经受累（P = 0.05）。与淋巴系统恶性肿瘤相关的血管炎患者关节痛（P = 0.01）和肾脏受累（P = 0.02）的频率较低。
恶性肿瘤期间发生的血管炎根据血管炎亚型和恶性肿瘤性质呈现出不同特征。
