@article{oai:kanazawa-u.repo.nii.ac.jp:00044306,
 author = {水島, 伊知郎 and 井上, 大 and 山田, 和徳 and 和田, 隆志 and 笠島, 里美 and 原田, 憲一 and 中沼, 安二 and 梅原, 久範 and 山岸, 正和 and 川野, 充弘 and Mizushima, Ichiro and Inoue, Dai and Yamamoto, Motohisa and Yamada, Kazunori and Saeki, Takako and Ubara, Yoshifumi and Matsui, Shoko and Masaki, Yasufumi and Wada, Takashi and Kasashima, Satomi and Harada, Kenichi and Takahashi, Hiroki and Notohara, Kenji and Nakanuma, Yasuni and Umehara, Hisanori and Yamagishi, Masakazu and Kawano, Mitsuhiro},
 issue = {4},
 journal = {Arthritis Research and Therapy},
 month = {Jul},
 note = {Introduction: 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.Methods: 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. Results: 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. Conclusions: 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. © 2014 Mizushima et al.; licensee BioMed Central Ltd., 金沢大学医薬保健研究域医学系},
 title = {Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: A retrospective multicenter study},
 volume = {16},
 year = {2014}
}