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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 |
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| creator | 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 |
| description | 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. |
| doi_str_mv | 10.1186/ar4671 |
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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.</description><identifier>ISSN: 1478-6354</identifier><identifier>EISSN: 1478-6362</identifier><identifier>DOI: 10.1186/ar4671</identifier><identifier>PMID: 25056443</identifier><language>eng</language><publisher>England: BioMed Central Ltd</publisher><subject>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</subject><ispartof>Arthritis research & therapy, 2014-07, Vol.16 (4), p.R156-R156</ispartof><rights>COPYRIGHT 2014 BioMed Central Ltd.</rights><rights>Mizushima et al.; licensee BioMed Central Ltd. 2014</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c569t-e6de713a3a00c2653e1dd78bf95ef1c0b533434e021f5365886f0ce7491b1e553</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4220557/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4220557/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,315,733,786,790,891,27957,27958,53827,53829</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25056443$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mizushima, Ichiro</creatorcontrib><creatorcontrib>Inoue, Dai</creatorcontrib><creatorcontrib>Yamamoto, Motohisa</creatorcontrib><creatorcontrib>Yamada, Kazunori</creatorcontrib><creatorcontrib>Saeki, Takako</creatorcontrib><creatorcontrib>Ubara, Yoshifumi</creatorcontrib><creatorcontrib>Matsui, Shoko</creatorcontrib><creatorcontrib>Masaki, Yasufumi</creatorcontrib><creatorcontrib>Wada, Takashi</creatorcontrib><creatorcontrib>Kasashima, Satomi</creatorcontrib><creatorcontrib>Harada, Kenichi</creatorcontrib><creatorcontrib>Takahashi, Hiroki</creatorcontrib><creatorcontrib>Notohara, Kenji</creatorcontrib><creatorcontrib>Nakanuma, Yasuni</creatorcontrib><creatorcontrib>Umehara, Hisanori</creatorcontrib><creatorcontrib>Yamagishi, Masakazu</creatorcontrib><creatorcontrib>Kawano, Mitsuhiro</creatorcontrib><title>Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study</title><title>Arthritis research & therapy</title><addtitle>Arthritis Res Ther</addtitle><description>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.</description><subject>Adult</subject><subject>Aged</subject><subject>Anti-Inflammatory Agents - therapeutic use</subject><subject>Aortitis - drug therapy</subject><subject>Aortitis - immunology</subject><subject>Aortitis - pathology</subject><subject>Female</subject><subject>Glucocorticoids - therapeutic use</subject><subject>Humans</subject><subject>Immunoglobulin G</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Polyarteritis Nodosa - drug therapy</subject><subject>Polyarteritis Nodosa - immunology</subject><subject>Polyarteritis Nodosa - pathology</subject><subject>Prednisolone - therapeutic use</subject><subject>Retroperitoneal Fibrosis - drug therapy</subject><subject>Retroperitoneal Fibrosis - immunology</subject><subject>Retroperitoneal Fibrosis - pathology</subject><subject>Retrospective Studies</subject><subject>Treatment Outcome</subject><issn>1478-6354</issn><issn>1478-6362</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><recordid>eNptkt9rFDEQxxdRbG31T5CAIL5sm9-b86FQDq2FQl_a55BLJtfI7mZNsoX7C_y3zXrtYUHykJnJZ76ZZKZpPhB8RoiS5yZx2ZFXzTHhnWolk_T1wRb8qHmX80-MKV1R_rY5ogILyTk7bn6v-zAGa3pk45wyIOMLpOqkEmzM1Y7BofIAyUw7FEZ0vb3ibYLeFHDILFgJ-XyCFJ4dZEaH_gZSTV8iX5FBCUqKeQJbwiOgYe6rPozLXbnMbnfavPGmz_D-aT9p7r9_u1v_aG9ur67XlzetFXJVWpAOOsIMMxhbKgUD4lynNn4lwBOLN4IxzjhgSrxgUiglPbbQ8RXZEBCCnTQXe91p3gzglhKS6fWUwmDSTkcT9MuTMTzobXzUnFIsRFcFvjwJpPhrhlz0ELKFvjcjxDlrIoQSVAlFKvppj25NDzqMPlZFu-D6UnCsGOvwQp39h6rLwVBbMIIPNf4i4fM-wdYPzQn8oXqC9TIMej8MFfz471sP2HP32R8c27Hv</recordid><startdate>20140723</startdate><enddate>20140723</enddate><creator>Mizushima, Ichiro</creator><creator>Inoue, Dai</creator><creator>Yamamoto, Motohisa</creator><creator>Yamada, Kazunori</creator><creator>Saeki, Takako</creator><creator>Ubara, Yoshifumi</creator><creator>Matsui, Shoko</creator><creator>Masaki, Yasufumi</creator><creator>Wada, Takashi</creator><creator>Kasashima, Satomi</creator><creator>Harada, Kenichi</creator><creator>Takahashi, Hiroki</creator><creator>Notohara, Kenji</creator><creator>Nakanuma, Yasuni</creator><creator>Umehara, Hisanori</creator><creator>Yamagishi, Masakazu</creator><creator>Kawano, Mitsuhiro</creator><general>BioMed Central Ltd</general><general>BioMed Central</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20140723</creationdate><title>Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study</title><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></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c569t-e6de713a3a00c2653e1dd78bf95ef1c0b533434e021f5365886f0ce7491b1e553</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Anti-Inflammatory Agents - therapeutic use</topic><topic>Aortitis - drug therapy</topic><topic>Aortitis - immunology</topic><topic>Aortitis - pathology</topic><topic>Female</topic><topic>Glucocorticoids - therapeutic use</topic><topic>Humans</topic><topic>Immunoglobulin G</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Polyarteritis Nodosa - drug therapy</topic><topic>Polyarteritis Nodosa - immunology</topic><topic>Polyarteritis Nodosa - pathology</topic><topic>Prednisolone - therapeutic use</topic><topic>Retroperitoneal Fibrosis - drug therapy</topic><topic>Retroperitoneal Fibrosis - immunology</topic><topic>Retroperitoneal Fibrosis - pathology</topic><topic>Retrospective Studies</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mizushima, Ichiro</creatorcontrib><creatorcontrib>Inoue, Dai</creatorcontrib><creatorcontrib>Yamamoto, Motohisa</creatorcontrib><creatorcontrib>Yamada, Kazunori</creatorcontrib><creatorcontrib>Saeki, Takako</creatorcontrib><creatorcontrib>Ubara, Yoshifumi</creatorcontrib><creatorcontrib>Matsui, Shoko</creatorcontrib><creatorcontrib>Masaki, Yasufumi</creatorcontrib><creatorcontrib>Wada, Takashi</creatorcontrib><creatorcontrib>Kasashima, Satomi</creatorcontrib><creatorcontrib>Harada, Kenichi</creatorcontrib><creatorcontrib>Takahashi, Hiroki</creatorcontrib><creatorcontrib>Notohara, Kenji</creatorcontrib><creatorcontrib>Nakanuma, Yasuni</creatorcontrib><creatorcontrib>Umehara, Hisanori</creatorcontrib><creatorcontrib>Yamagishi, Masakazu</creatorcontrib><creatorcontrib>Kawano, Mitsuhiro</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Arthritis research & therapy</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mizushima, Ichiro</au><au>Inoue, Dai</au><au>Yamamoto, Motohisa</au><au>Yamada, Kazunori</au><au>Saeki, Takako</au><au>Ubara, Yoshifumi</au><au>Matsui, Shoko</au><au>Masaki, Yasufumi</au><au>Wada, Takashi</au><au>Kasashima, Satomi</au><au>Harada, Kenichi</au><au>Takahashi, Hiroki</au><au>Notohara, Kenji</au><au>Nakanuma, Yasuni</au><au>Umehara, Hisanori</au><au>Yamagishi, Masakazu</au><au>Kawano, Mitsuhiro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study</atitle><jtitle>Arthritis research & therapy</jtitle><addtitle>Arthritis Res Ther</addtitle><date>2014-07-23</date><risdate>2014</risdate><volume>16</volume><issue>4</issue><spage>R156</spage><epage>R156</epage><pages>R156-R156</pages><issn>1478-6354</issn><eissn>1478-6362</eissn><notes>ObjectType-Article-1</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-2</notes><notes>content type line 23</notes><abstract>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.</abstract><cop>England</cop><pub>BioMed Central Ltd</pub><pmid>25056443</pmid><doi>10.1186/ar4671</doi><oa>free_for_read</oa></addata></record> |
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| 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 |
| title | Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study |
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