title: Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study.
abstract: BACKGROUND
  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.
authors: Ichiro
authors: I
authors: Mizushima
authors: Dai
authors: D
authors: Inoue
authors: Motohisa
authors: M
authors: Yamamoto
authors: Kazunori
authors: K
authors: Yamada
authors: Takako
authors: T
authors: Saeki
authors: Yoshifumi
authors: Y
authors: Ubara
authors: Shoko
authors: S
authors: Matsui
authors: Yasufumi
authors: Y
authors: Masaki
authors: Takashi
authors: T
authors: Wada
authors: Satomi
authors: S
authors: Kasashima
authors: Kenichi
authors: K
authors: Harada
authors: Hiroki
authors: H
authors: Takahashi
authors: Kenji
authors: K
authors: Notohara
authors: Yasuni
authors: Y
authors: Nakanuma
authors: Hisanori
authors: H
authors: Umehara
authors: Masakazu
authors: M
authors: Yamagishi
authors: Mitsuhiro
authors: M
authors: Kawano
mesh_terms: D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001025:Aortitis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007074:Immunoglobulin G; D008297:Male; D008875:Middle Aged; D010488:Polyarteritis Nodosa / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D012185:Retroperitoneal Fibrosis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone