Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study

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 thera...

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Published in:Arthritis research & therapy 2014-07, Vol.16 (4), p.R156-R156
Main Authors: Mizushima, Ichiro, Inoue, Dai, Yamamoto, Motohisa, Yamada, Kazunori, Saeki, Takako, Ubara, Yoshifumi, Matsui, Shoko, Masaki, Yasufumi, Wada, Takashi, Kasashima, Satomi, Harada, Kenichi, Takahashi, Hiroki, Notohara, Kenji, Nakanuma, Yasuni, Umehara, Hisanori, Yamagishi, Masakazu, Kawano, Mitsuhiro
Format: Article
Language:English
Subjects:
Adult
Aged
Anti-Inflammatory Agents - therapeutic use
Aortitis - drug therapy
Aortitis - immunology
Aortitis - pathology
Female
Glucocorticoids - therapeutic use
Humans
Immunoglobulin G
Male
Middle Aged
Polyarteritis Nodosa - drug therapy
Polyarteritis Nodosa - immunology
Polyarteritis Nodosa - pathology
Prednisolone - therapeutic use
Retroperitoneal Fibrosis - drug therapy
Retroperitoneal Fibrosis - immunology
Retroperitoneal Fibrosis - pathology
Retrospective Studies
Treatment Outcome
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Summary: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.
ISSN:1478-6354
1478-6362
DOI:10.1186/ar4671