Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients

• DOI: 10.1186/s13075-018-1567-2
• Publication Date: 2018-04-19T11:20:22Z
• Authors: Liwen Wang; Panpan Zhang; Mu Wang; Ruie Feng; Yamin Lai; Linyi Peng; Yunyun Fei; Xuan Zhang; Yan Zhao; Xiaofeng Zeng; Fengchun Zhang; Wen Zhang
• Keywords: Adult; Male; Adolescent; Remission; 2745 Rheumatology; 610; Diseases of the musculoskeletal system; Cohort Studies; 03 medical and health sciences; 0302 clinical medicine; Azathioprine; Humans; Glycosides; Relapse; IgG4-related disease; Child; Cyclophosphamide; Glucocorticoids; Aged; Aged, 80 and over; 2403 Immunology; Middle Aged; Mycophenolic Acid; 3. Good health; Methotrexate; RC925-935; 2723 Immunology and Allergy; Cyclosporine; Female; Immunoglobulin G4-Related Disease; Immunosuppressive agents; Immunosuppressive Agents; Leflunomide; Research Article

The aim of this study was to assess the outcomes remission induction in patients with IgG4-related disease (IgG4-RD) our cohort, and investigate characteristics, prognosis, risk factors failed induction. We prospectively enrolled 215 newly diagnosed IgG4-RD, who were initially treated glucocorticoid (GC) alone or combination immunosuppressive agents (IM), had at least 6 months follow up. therapeutic goals defined as fulfilling each following after 6-month stage: (1) ≥ 50% decline IgG4-RD responder index (RI); (2) GC tapered maintenance dose; (3) no relapse during tapering. not achieving considered have There 26 cohort induction, including 16 (20.8%) on monotherapy, 10 (7.2%) therapy comprising IM. lacrimal gland lung most common sites failure. Among relapsed stage, 52.9% secondary follow-up. Eosinophilia, higher baseline RI, more than five organs involved dacryoadenitis for failure incidence 71.4% three factors. After treatment, significantly erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) IgG4. In 20.8% while 7.2%