抗中性粒细胞胞浆抗体相关性血管炎和 IgG4 相关疾病：一种新的重叠综合征。
Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.
机构信息
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Nephrology Unit, University Hospital, Parma, Italy.
出版信息
Autoimmun Rev. 2017 Oct;16(10):1036-1043. doi: 10.1016/j.autrev.2017.07.020. Epub 2017 Aug 2.
OBJECTIVE
Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap.
METHODS
We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria.
RESULTS
Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases.
CONCLUSION
AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions.
目的
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者存在非典型表现，如硬脑膜炎、眼眶肿块或慢性大动脉炎。由于这些表现与 IgG4 相关疾病（IgG4-RD）的谱相关，我们假设这两种疾病可能重叠。
方法
我们进行了一项欧洲回顾性多中心观察性研究，纳入符合 ACR 和 Chapel Hill AAV 标准以及 IgG4-RD 综合诊断标准的患者。
结果
共纳入 18 例患者（中位年龄 55.5 岁，13 例男性）。13/18（72%）例患者同时诊断为 AAV 和 IgG4-RD；3/18（17%）例患者 AAV 先于 IgG4-RD，2/18（11%）例患者 IgG4-RD 先于 AAV。AAV 诊断包括肉芽肿性多血管炎 14 例（78%）、显微镜下多血管炎 3 例（17%）、嗜酸性肉芽肿性多血管炎 1 例。IgG4-RD 诊断包括明确 IgG4-RD 5 例（28%）、可能 IgG4-RD 5 例（28%）和可能 IgG4-RD 8 例（44%）。18 例患者中有 9 例（50%）存在慢性大动脉炎、4 例（22%）存在眼眶肿块和肾小管间质性肾炎、3 例（17%）存在前纵膈纤维化、2 例（11%）存在硬脑膜炎和自身免疫性胰腺炎。患者需要联合糖皮质激素使用中位数 2（0-4）线免疫抑制剂。在随访期间（中位数 49.8 个月，范围 17.25-108 个月），10/18（56%）例患者出现 AAV 表现复发，5/18（28%）例患者出现 IgG4-RD 病变。在用于治疗复发时，利妥昔单抗对所有病例均有反应。
结论
AAV 和 IgG4-RD 可能重叠。临床医生应考虑到 AAV 期间的非典型表现可能与 IgG4-RD 有关，而不是与难治性肉芽肿性或血管炎性病变有关。
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