Yasufumi Masaki1, Shoko Matsui2, Takako Saeki3, Hiroto Tsuboi4, Shintaro Hirata5, Yasumori Izumi6, Taiichiro Miyashita6,7, Keita Fujikawa8, Hiroaki Dobashi9, Kentaro Susaki9, Hisanori Morimoto10, Kazutaka Takagi11, Mitsuhiro Kawano12, Tomoki Origuchi13, Yoko Wada14, Naoki Takahashi15, Masanobu Horikoshi4, Hiroshi Ogishima4, Yasunori Suzuki12, Takafumi Kawanami1, Haruka Kawanami Iwao1, Tomoyuki Sakai1, Yoshimasa Fujita1, Toshihiro Fukushima1, Masatoshi Saito16, Ritsuro Suzuki17, Yuko Morikawa18, Tadashi Yoshino19, Shigeo Nakamura20, Masaru Kojima21, Nozomu Kurose22, Yasuharu Sato19, Yoshiya Tanaka5, Susumu Sugai1, Takayuki Sumida4. 1. a Division of Hematology and Immunology , Kanazawa Medical University , Uchinada , Japan. 2. b First Department of Internal Medicine , University of Toyama , Toyama , Japan. 3. c Department of Internal Medicine , Nagaoka Red Cross Hospital , Nagaoka , Japan. 4. d Department of Internal Medicine , University of Tsukuba , Tsukuba , Japan. 5. e The First Department of Internal Medicine, School of Medicine , University of Occupational and Environmental Health Japan , Fukuoka , Japan. 6. f Department of Rheumatology , NHO Nagasaki Medical Center , Nagasaki , Japan. 7. g Miyashita Rheumatology Clinic , Nagasaki , Japan. 8. h Department of Rheumatology , Japan Community Healthcare Organization (JCHO), Isahaya General Hospital , Nagasaki , Japan. 9. i Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University , Takamatsu , Japan. 10. j Department of Internal Medicine , Mitoyo General Hospital , Kan'onji , Japan. 11. k Division of Hematology and Immunology , Maizuru-Kyousai Hospital , Kyoto , Japan. 12. l Division of Rheumatology, Department of Internal Medicine , Kanazawa University Graduate School of Medical Sciences , Kanazawa , Japan. 13. m Laboratory of Basic Physical Therapy Science , Nagasaki Graduate School of Health Sciences , Nagasaki , Japan. 14. n Division of Clinical Nephrology and Rheumatology , Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan. 15. o Division of Nephrology, Department of General Medicine, Faculty of Medical Sciences , University of Fukui , Fukui , Japan. 16. p Division of Respiratory Medicine , Kanazawa Medical University , Uchinada , Japan. 17. q Department of Oncology/Hematology, School of Medicine , Shimane University , Izumo , Japan. 18. r Department of Epidemiology and Public Health (School of Nursing) , Kanazawa Medical University , Uchinada , Japan. 19. s Department of Pathology , Okayama University Graduate School of Medicine , Okayama , Japan. 20. t Department of Pathology and Laboratory Medicine , Nagoya University Hospital , Nagoya , Japan. 21. u Department of Anatomic and Diagnostic Pathology , Dokkyo University School of Medicine , Mibu , Japan , and. 22. v Department of Pathology and Laboratory Medicine , Kanazawa Medical University , Uchinada , Japan.
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. PATIENTS AND 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.
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. PATIENTS AND 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: George Goodchild; Rory Jr Peters; Tamsin N Cargill; Harry Martin; Adetokunbo Fadipe; Maria Leandro; Adam Bailey; Jane Collier; Louisa Firmin; Manil Chouhan; Manuel Rodriguez-Justo; Ross Sadler; Roger W Chapman; Helen Bungay; Eve Fryer; Joel David; Raashid Luqmani; Eleanor Barnes; George J Webster; Emma L Culver Journal: Clin Med (Lond) Date: 2020-05 Impact factor: 2.659
Authors: Riccardo Capecchi; Domenico Giannese; Diego Moriconi; Angelo G Bonadio; Federico Pratesi; Cristina Croia; Maria F Egidi; Ilaria Puxeddu; Antonio G Tavoni; Paola Migliorini Journal: Front Med (Lausanne) Date: 2021-03-31