title: Clinical features of patients with IgG4-related disease complicated with perivascular lesions.
abstract: OBJECTIVE
  To define the clinical features of IgG4-related disease (IgG4-RD) complicated with perivascular lesions.
  
  
  METHODS
  The clinical features of seven patients with IgG4-RD and perivascular lesions diagnosed at the University of Tsukuba Hospital between October 2008 and October 2013, were analyzed, including clinical background, results of imaging studies, satisfaction of the 2011 comprehensive diagnostic criteria (CDC) for IgG4-RD, laboratory data, distribution of perivascular lesions, involvement of other organs, and response to steroid therapy.
  
  
  RESULTS
  We studied six men and one woman with a mean age of 66.9 ± 6.7 years (± SD). Six of seven patients were diagnosed as definite IgG4-RD, while the seventh was considered possible IgG4-RD, based on the CDC for IgG4-RD. Serum IgG4 levels at diagnosis were higher than 135 mg/dl in all seven patients (mean, 933 ± 527). Serum C-reactive protein (CRP) levels were elevated in two only (mean, 1.42 ± 3.56 mg/dl). The perivascular lesions were located in the pulmonary artery (n = 1), thoracic aorta (n = 2), abdominal aorta (n = 6), coronary (n = 1), celiac (n = 1), superior mesenteric (n = 1), renal (n = 2), inferior mesenteric (n = 5), and iliac (n = 3) arteries. In addition to perivascular lesions, six patients showed involvement of other organs. All seven patients were treated with prednisolone (0.6 mg/kg/day), which rapidly improved the perivascular and other organ lesions in six patients (the other one patient have not yet been evaluated due to the short follow-up).
  
  
  CONCLUSIONS
  Perivascular lesions show wide distribution in patients with IgG4-RD. Serum CRP levels are not necessarily elevated in these patients. Steroid therapy is effective in IgG4-RD and results in resolution of lesions.
authors.affiliation: Department of Internal Medicine, Faculty of Medicine, University of Tsukuba , Tsukuba , Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ebe
authors.forename: Hiroto
authors.initials: H
authors.lastname: Tsuboi
authors.forename: Chihiro
authors.initials: C
authors.lastname: Hagiya
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Takahashi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yokosawa
authors.forename: Shinya
authors.initials: S
authors.lastname: Hagiwara
authors.forename: Tomoya
authors.initials: T
authors.lastname: Hirota
authors.forename: Yuko
authors.initials: Y
authors.lastname: Kurashima
authors.forename: Chinatsu
authors.initials: C
authors.lastname: Takai
authors.forename: Haruka
authors.initials: H
authors.lastname: Miki
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Asashima
authors.forename: Naoto
authors.initials: N
authors.lastname: Umeda
authors.forename: Yuya
authors.initials: Y
authors.lastname: Kondo
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ogishima
authors.forename: Takeshi
authors.initials: T
authors.lastname: Suzuki
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Chino
authors.forename: Isao
authors.initials: I
authors.lastname: Matsumoto
authors.forename: Takayuki
authors.initials: T
authors.lastname: Sumida
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014652:Vascular Diseases / Q000097:blood / Q000150:complications / Q000175:diagnosis*
chemical_list: D007074:Immunoglobulin G
keywords: IgG4-related disease; Perivascular lesions; Steroid therapy