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

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 cortic...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4220557/
https://www.ncbi.nlm.nih.gov/pubmed/25056443
http://dx.doi.org/10.1186/ar4671
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author 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
author_facet 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
author_sort Mizushima, Ichiro
collection PubMed
description 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.
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spelling pubmed-42205572014-11-06 Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study 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 Arthritis Res Ther Research Article 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. BioMed Central 2014-07-23 2014 /pmc/articles/PMC4220557/ /pubmed/25056443 http://dx.doi.org/10.1186/ar4671 Text en © Mizushima et al.; licensee BioMed Central Ltd. 2014 This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
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
Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
title Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
title_full Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
title_fullStr Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
title_full_unstemmed Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
title_short Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
title_sort clinical course after corticosteroid therapy in igg4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4220557/
https://www.ncbi.nlm.nih.gov/pubmed/25056443
http://dx.doi.org/10.1186/ar4671
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