Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study.
Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study.
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DOI：
10.1186/ar4671
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发表时间：
2014-07-23
影响因子：
4.9
通讯作者：
Kawano M
中科院分区：
文献类型：
--
作者：
Mizushima I;Inoue D;Yamamoto M;Yamada K;Saeki T;Ubara Y;Matsui S;Masaki Y;Wada T;Kasashima S;Harada K;Takahashi H;Notohara K;Nakanuma Y;Umehara H;Yamagishi M;Kawano M
免疫球蛋白G4（IgG 4）相关性肠炎/腹膜炎和动脉周围炎是IgG 4相关性疾病的血管表现。在这种疾病中，受累的囊性病变被怀疑有破裂的危险。在这项研究中，我们的目的是澄清皮质类固醇治疗后的临床病程在IgG 4相关的肠炎/心包炎和动脉周围炎。我们回顾性评估了40例诊断为IgG 4相关主动脉炎/主动脉周围炎和动脉周围炎的患者的临床特征，包括实验室数据、影像学检查结果和皮质类固醇治疗后的病程，这些患者的诊断标准包括主动脉周围/动脉周围放射学检查结果、综合诊断标准或每个器官特异性诊断标准的满意度，并排除其他疾病。患者主要是老年人，平均年龄为66.4岁，男性占明显优势，并广泛累及其他器官。主观症状很少，只有一小部分血清C反应蛋白水平升高。在大多数情况下，受影响的主动脉/动脉是腹主动脉或髂动脉。36例患者接受泼尼松龙治疗，其中大部分患者的主动脉周围/动脉周围病变在随访期间得到改善。皮质类固醇治疗前受累病变管腔扩张的4例患者中有2例（50.0%）在治疗后管腔扩张加重，而26例无管腔扩张的患者在治疗后均未出现新的管腔扩张。这项回顾性多中心研究的结果强调了三个要点：（1）主动脉周围/动脉周围病变潜在存在和进展的可能性，（2）皮质类固醇治疗在无主动脉周围/动脉周围病变管腔扩张的患者中预防新动脉瘤形成的有效性，以及（3）小部分患者实际上可能发生主动脉周围/动脉周围病变管腔扩张的可能性。在IgG 4相关性腹膜炎/腹膜炎和动脉周围炎中的动脉周围病变。需要进行一项更大规模的前瞻性研究，以确认皮质类固醇治疗在有管腔扩张患者与无管腔扩张患者中的疗效和安全性，并设计更有用和安全的治疗策略，包括给予其他免疫抑制剂。
Immunoglobulin G4 (IgG4)–related aortitis/periaortitis and periarteritis are vascular manifestations of IgG4-related disease. In this disease, the affected aneurysmal lesion has been suspected to be at risk of rupture. In this study, we aimed to clarify the clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis. We retrospectively evaluated clinical features, including laboratory data, imaging findings and the course after corticosteroid therapy, in 40 patients diagnosed with IgG4-related aortitis/periaortitis and periarteritis on the basis of periaortic/periarterial radiological findings, satisfaction of the comprehensive diagnostic criteria or each organ-specific diagnostic criteria, and exclusion of other diseases. The patients were mainly elderly, with an average age of 66.4 years and with a marked male predominance and extensive other organ involvement. Subjective symptoms were scanty, and only a small proportion had elevated serum C-reactive protein levels. The affected aorta/artery were the abdominal aortas or the iliac arteries in most cases. Thirty-six patients were treated with prednisolone, and the periaortic/periarterial lesions improved in most of them during the follow-up period. Two (50.0%) of four patients with luminal dilatation of the affected lesions before corticosteroid therapy had exacerbations of luminal dilatation after therapy, whereas none of the twenty-six patients without it had a new appearance of luminal dilatation after therapy. The results of this retrospective multicenter study highlight three important points: (1) the possibility of latent existence and progression of periaortic/periarterial lesions, (2) the efficacy of corticosteroid therapy in preventing new aneurysm formation in patients without luminal dilatation of periaortic/periarterial lesions and (3) the possibility that a small proportion of patients may actually develop luminal dilatation of periaortic/periarterial lesions in IgG4-related aortitis/periaortitis and periarteritis. A larger-scale prospective study is required to confirm the efficacy and safety of corticosteroid therapy in patients with versus those without luminal dilatation and to devise a more useful and safe treatment strategy, including administration of other immunosuppressants.
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一种新的临床实体，IgG4 相关疾病 (IgG4RD)：一般概念和细节。
影响因子：
2.2
作者：
Umehara, Hisanori;Okazaki, Kazuichi;Masaki, Yasufumi;Kawano, Mitsuhiro;Yamamoto, Motohisa;Saeki, Takako;Matsui, Shoko;Sumida, Takayuki;Mimori, Tsuneyo;Tanaka, Yoshiya;Tsubota, Kazuo;Yoshino, Tadashi;Kawa, Shigeyuki;Suzuki, Ritsuro;Takegami, Tsutomu;Tomosugi, Naohisa;Kurose, Nozomu;Ishigaki, Yasuhito;Azumi, Atsushi;Kojima, Masaru;Nakamura, Shigeo;Inoue, Dai
通讯作者：
Inoue, Dai
影响因子：
19.7
作者：
Inoue, Dai;Zen, Yoh;Matsui, Osamu
通讯作者：
Matsui, Osamu
影响因子：
4.3
作者：
Kasashima, Satomi;Zen, Yoh;Kasashima, Fuminori
通讯作者：
Kasashima, Fuminori
影响因子：
3.7
作者：
Siddiquee, Zakir;Zane, Nicholas A.;Stone, James R.
通讯作者：
Stone, James R.
影响因子：
1.2
作者：
Naitoh, Itaru;Nakazawa, Takahiro;Joh, Takashi
通讯作者：
Joh, Takashi