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.
Acknowledgments
We thank all participants in the All Japan, Ministry of Health, Labor, and Welfare (MHLW) IgG4 Team including for their critical discussion. We thank participating investigators: Kazuhiro Sato (Nagaoka Red-Cross Hospital), Naoko Sato (Nagaoka Red-Cross Hospital), Eri Hirakawa (University of Occupational and Environmental Health Japan), Satoshi Nakajima (University of Occupational and Environmental Health Japan), Masao Nawada (University of Occupational and Environmental Health Japan), Takahisa Suzuki (Nagasaki Graduate School of Health Sciences), Masataka Umeda (Nagasaki Graduate School of Health Sciences), Hiroki Ozaki (Kagawa University), Shusaku Nakashima (Kagawa University), Hironori Shimizu (Kanazawa Medical University), Akio Nakajima (Kanazawa Medical University), Miyuki Miki (Kanazawa Medical University), Tomomi Nakamura (Satoh) (Kanazawa Medical University), and Takuji Nakamura (Kanazawa Medical University).
Conflict of interest
None.
Funding
This work was partially supported by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare (MHLW) of Japan, and by the Japanese Ministry of Education, Culture, Sports, Science and Technology (Grant Nos. 17591060 and 15K09510), the Kanazawa Medical University Research Foundation (Grant Nos. S2004-16 and S2007-5), Grant for Assist KAKEN from Kanazawa Medical University (Grant No. K2011-7), Grant for Project Research from High-Tech Research Center of Kanazawa Medical University (Grant No. H2011-11), and Grant for Alumni Research (A) from Kanazawa Medical University (AR2012-06).