title: A multicenter phase II prospective clinical trial of glucocorticoid for patients with untreated IgG4-related disease.
abstract: OBJECTIVE
  Although glucocorticoids are effective for patients with IgG4-related disease, the treatment has not yet been standardized. Therefore, the treatment strategy should be established.
  
  
  METHODS
  Patients who fulfilled the comprehensive diagnostic criteria for definite IgG4-related disease were started on prednisolone (0.6 mg/kg body weight) with the dose reduced every two weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients. The primary endpoint was the complete remission (CR) rate at one year. Secondary endpoints included overall response rate (ORR), the maintenance dose, the relapse rate, and adverse events.
  
  
  RESULTS
  This study enrolled 61 patients. After clinicopathological review, three patients were excluded, and one, 13, and 44 patients were diagnosed with probable, possible, and definite IgG4-related disease, respectively. Of the 44 patients with definite IgG4-RD, 29 (65.9%) achieved CR, and the ORR was 93.2%. No patient was refractory to primary treatment. The most frequent adverse events were glucose intolerance. Six patients relapsed.
  
  
  CONCLUSIONS
  Glucocorticoid treatment is usually effective for patients with IgG4-RD, and we should examine the possibility of other disorders when a patient is glucocorticoid refractory. Some patients are misdiagnosed, making central clinicopathological review of diagnosis very important in conducting clinical studies.
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Masaki
authors.affiliation: b First Department of Internal Medicine , University of Toyama , Toyama , Japan.
authors.forename: Shoko
authors.initials: S
authors.lastname: Matsui
authors.affiliation: c Department of Internal Medicine , Nagaoka Red Cross Hospital , Nagaoka , Japan.
authors.forename: Takako
authors.initials: T
authors.lastname: Saeki
authors.affiliation: d Department of Internal Medicine , University of Tsukuba , Tsukuba , Japan.
authors.forename: Hiroto
authors.initials: H
authors.lastname: Tsuboi
authors.affiliation: e The First Department of Internal Medicine, School of Medicine , University of Occupational and Environmental Health Japan , Fukuoka , Japan.
authors.forename: Shintaro
authors.initials: S
authors.lastname: Hirata
authors.affiliation: f Department of Rheumatology , NHO Nagasaki Medical Center , Nagasaki , Japan.
authors.forename: Yasumori
authors.initials: Y
authors.lastname: Izumi
authors.affiliation: f Department of Rheumatology , NHO Nagasaki Medical Center , Nagasaki , Japan.|g Miyashita Rheumatology Clinic , Nagasaki , Japan.
authors.forename: Taiichiro
authors.initials: T
authors.lastname: Miyashita
authors.affiliation: h Department of Rheumatology , Japan Community Healthcare Organization (JCHO), Isahaya General Hospital , Nagasaki , Japan.
authors.forename: Keita
authors.initials: K
authors.lastname: Fujikawa
authors.affiliation: i Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University , Takamatsu , Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Dobashi
authors.affiliation: i Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University , Takamatsu , Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Susaki
authors.affiliation: j Department of Internal Medicine , Mitoyo General Hospital , Kan'onji , Japan.
authors.forename: Hisanori
authors.initials: H
authors.lastname: Morimoto
authors.affiliation: k Division of Hematology and Immunology , Maizuru-Kyousai Hospital , Kyoto , Japan.
authors.forename: Kazutaka
authors.initials: K
authors.lastname: Takagi
authors.affiliation: l Division of Rheumatology, Department of Internal Medicine , Kanazawa University Graduate School of Medical Sciences , Kanazawa , Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kawano
authors.affiliation: m Laboratory of Basic Physical Therapy Science , Nagasaki Graduate School of Health Sciences , Nagasaki , Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Origuchi
authors.affiliation: n Division of Clinical Nephrology and Rheumatology , Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Wada
authors.affiliation: o Division of Nephrology, Department of General Medicine, Faculty of Medical Sciences , University of Fukui , Fukui , Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Takahashi
authors.affiliation: d Department of Internal Medicine , University of Tsukuba , Tsukuba , Japan.
authors.forename: Masanobu
authors.initials: M
authors.lastname: Horikoshi
authors.affiliation: d Department of Internal Medicine , University of Tsukuba , Tsukuba , Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ogishima
authors.affiliation: l Division of Rheumatology, Department of Internal Medicine , Kanazawa University Graduate School of Medical Sciences , Kanazawa , Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Suzuki
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kawanami
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Haruka
authors.initials: H
authors.lastname: Kawanami Iwao
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Sakai
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Yoshimasa
authors.initials: Y
authors.lastname: Fujita
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Fukushima
authors.affiliation: p Division of Respiratory Medicine , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Saito
authors.affiliation: q Department of Oncology/Hematology, School of Medicine , Shimane University , Izumo , Japan.
authors.forename: Ritsuro
authors.initials: R
authors.lastname: Suzuki
authors.affiliation: r Department of Epidemiology and Public Health (School of Nursing) , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Morikawa
authors.affiliation: s Department of Pathology , Okayama University Graduate School of Medicine , Okayama , Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
authors.affiliation: t Department of Pathology and Laboratory Medicine , Nagoya University Hospital , Nagoya , Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.affiliation: u Department of Anatomic and Diagnostic Pathology , Dokkyo University School of Medicine , Mibu , Japan , and.
authors.forename: Masaru
authors.initials: M
authors.lastname: Kojima
authors.affiliation: v Department of Pathology and Laboratory Medicine , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Nozomu
authors.initials: N
authors.lastname: Kurose
authors.affiliation: s Department of Pathology , Okayama University Graduate School of Medicine , Okayama , Japan.
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Sato
authors.affiliation: e The First Department of Internal Medicine, School of Medicine , University of Occupational and Environmental Health Japan , Fukuoka , Japan.
authors.forename: Yoshiya
authors.initials: Y
authors.lastname: Tanaka
authors.affiliation: a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan.
authors.forename: Susumu
authors.initials: S
authors.lastname: Sugai
authors.affiliation: d Department of Internal Medicine , University of Tsukuba , Tsukuba , Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Sumida
mesh_terms: D000328:Adult; D000368:Aged; D004305:Dose-Response Relationship, Drug; D054796:Drug Dosage Calculations; D016903:Drug Monitoring; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000009:adverse effects; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D011446:Prospective Studies; D012074:Remission Induction / Q000379:methods; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone
keywords: 18FDG-PET; Glucocorticoid; Lymphoma; Multicentric Castleman's disease; Prospective trial