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Objective: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory disease which affects many systems, as well as the cardiovascular system. Identifying the coronary involvement like periaortitis, coronary periarteritis and pericarditis is important, as they often cause unfavorable outcomes., Methods: Eighty-one patients with IgG4-RD were retrospectively evaluated for symptomatic coronary artery involvement from Hacettepe University Vasculitis Research Center (HUVAC) database. The demographic, laboratory, radiologic and clinical characteristics of the patients were assessed., Results: Among 81 patients with IgG4-RD, 6 patients (M/F:5/1) had coronary artery involvement. The patients' median age was 57 and serum IgG4 levels were above normal except for one case. All patients with coronary arteritis revealed an increased coronary vessel wall thickening and stenotic lesions. The coronary aneurysm and pericarditis were observed in half of the patients. Immunosuppressive treatments were given to all the patients and most of them followed in stable condition., Conclusion: Coronary arteritis is a rare but notable manifestation of IgG4-RD. Although coronary periarteritis can cause significant morbidity and mortality, it seems better results can be achieved with early diagnosis and treatment., (© 2021 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Background: Immunoglobulin G4-related disease (IgG4-RD) is an uncommon chronic systemic autoimmune disease, pathologically characterized by lymphoplasma cell and IgG4 plasma cell infiltration with storiform fibrosis. IgG4-RD is a new disease and is not yet widely recognized. The aim of this study was to describe the clinical manifestations and outcomes in Thai patients with IgG4-RD., Methods: This multicenter retrospective cohort study included patients aged ≥ 18 years who were diagnosed with IgG4-RD, according to the 2011 comprehensive or consensus diagnostic criteria, between 2000 and 2019 in four academic centers in Thailand. Baseline characteristics, laboratory and pathologic findings, treatments, and outcomes were systematically reviewed., Results: The study included 110 patients (71% male) with a mean age (SD) of 59.6 (13.3) years and median disease duration (interquartile range [IQR]) of 28.8 (14.6-53.5) months. Single organ involvement was observed in 60 patients (54.5%). Most patients (96%) had an IgG4 level of more than 135 mg/dL at presentation. Also, most (92%) were treated with corticosteroid (CS) alone or in combination with immunosuppressive agents. The most commonly used immunosuppressive agents were azathioprine (47%) and methotrexate (11%). Additionally, 20% required surgery, and 6.4% underwent stent insertion. One-quarter (26%), 37%, and 29% were in remission with successfully tapering CS, complete and partial response. Nevertheless, 22% relapsed, with a median time to relapse (IQR) of 22.2 (12.8-41.1) months., Conclusion: IgG4-RD is a chronic systemic autoimmune disease with diverse manifestations, response to treatment, and outcomes. Most patients responded well to treatments but with a notable relapse rate., (© 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Aim: The characteristics and the pathogenesis of the concomitant antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and immunoglobulin G4-related disease (IgG4-RD) have not been elucidated., Method: We included 92 AAV patients with renal biopsy results. Among them, 10 patients met both AAV and IgG4-RD criteria (concomitant group). The IgG subclasses of myeloperoxidase (MPO)-ANCA in both serum and renal tissue were measured and complement activation components were detected in serum., Results: Patients in the concomitant group had both elevated serum IgG4 levels and positive MPO-ANCA. They had higher levels of eosinophil counts, serum globulin, IgG, IgE and C-reactive protein than patients in the AAV alone group. All 10 patients had glomerulonephritis with crescents and seven patients also had segmental necrosis of the glomerular capillary wall. Most of them also presented with storiform fibrosis and lymphoplasmacytic infiltration in renal interstitium with IgG4 positive plasma cells more than 10/high-power field. Eight patients achieved remission with improved renal function, the other two patients were on maintenance dialysis. The IgG4 subclass of MPO-ANCA was higher in the concomitant group than that in AAV alone group. A merge of IgG4 and MPO immunofluorescence was observed in parts of the mesangium of concomitant AAV and IgG4-RD patients. For complement components, Bb and mannose-binding lectin were elevated in serum of concomitant AAV and IgG4-RD patients., Conclusion: We showed a new overlap syndrome of AAV and IgG4-RD, in which the IgG4 subclass of ANCA may be a pathogenic factor., (© 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related disease (IgG4-RD) have some common features. The co-occurrence/concurrence of AAV and IgG4-RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4-RD cohort. Second, a literature review of co-occurrence/concurrence of AAV and IgG4-RD was done., Methods: Data of 62 patients with IgG4-RD in Hacettepe Vasculitis Center Database were used. Patient dataset was designed to include demographic data, clinical characteristics, imaging and IgG4-RD, AAV and ANCA test results. At the next step, we performed a systematic literature review in PUBMED database covering the time period from 1976 until April 2018. Relevant publications were searched using these MeSH terms ''IgG4-related disease and Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis'', "IgG4-related disease and Eosinophilic Granulomatosis with Polyangiitis", "IgG4-related disease and Microscopic Polyangiitis" and "IgG4-related disease and Granulomatosis with Polyangiitis"., Results: Three (10.3%) of 29 patients had low titer ANCA positivity. These three patients didn't have any findings of vasculitis and no granuloma was seen in biopsy. In the literature review, we found 17 cases had features of both IgG4-RD and AAV. These cases were re-evaluated according to the Comprehensive Diagnostic Criteria for IgG4-RD. ANCA were positive in 15 of 17 patients (88%)., Conclusion: None of our IgG4-RD patients overlapped with AAV. Only two patients in the literature review seemed to be fully compatible with both diseases. Even though AAV and IgG4-RD share similar clinical features, we think this might be a co-occurrence instead of a histopathological link., (© 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Aim: The purpose of this study is to evaluate the therapeutic efficacy and safety of iguratimod plus corticosteroid as bridge therapy in the treatment of mild immunoglobulin G4-related disease (IgG4-RD)., Methods: Newly diagnosed IgG4-RD patients, without internal organ involvement were enrolled. Patients were given one dose of diprospan, intramuscular injection, and iguratimod, 25 mg, twice daily, for 24 weeks and were followed up at 0, 12 and 24 weeks. Follow-up indexes included IgG4-RD responder index (IgG4-RD RI), serology and imaging, plasma cytokines and adverse drug effect. Flow cytometry was performed for T, B cell subsets and plasma was collected for liquid chromatography mass spectrometry (LC-MS)-based metabolomic profiling and data processing., Results: Thirty patients were enrolled. At week 24, 9 (30.0%) patients achieved complete response, 17 (56.7%) patients with partial response, and 4 (13.3%) patients had no response to treatment. IgG4-RD RI, serum IgG and IgG4 levels decreased significantly at weeks 12 and 24 after treatment, as well as CD3+ CD8+ T cells, plasmablast/plasma cells and memory B cells. The LC-MS based plasma metabolomic profiles revealed significant changes between untreated patients and healthy donors, which became much similar to normal states after treatment., Conclusion: Iguratimod plus corticosteroid as bridge therapy is effective for the treatment of mild IgG4-RD, it can improve the clinical symptoms, reduce serum IgG and IgG4 levels, especially plasmablasts/plasma cells and memory B cells. In addition, the metabolite profiling became similar to normal controls after treatment., (© 2019 The Authors International Journal of Rheumatic Diseases published by Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)