Background Immunoglobulin G4 related disease (IgG4-RD) is a newly recognized systemic, immune-mediated and fibro-inflammatory disease. Hypocomplementemia was found in part of IgG4-RD patients especially in the setting of active disease. Objectives This study aimed to clarify the clinical features, treatment efficacy and outcome in IgG4-RD patients with hypocomplementemia. Methods 312 IgG4-RD patients were recruited in our prospective cohort conducted in Peking union medical college hospital. Patient’s demographic data, clinical characteristics, laboratory parameters, treatment and outcome were analyzed. Results Hypocomplementemia was identified in 65(20.8%) cases of untreated IgG4-RD patients at baseline. The average age of hypocomplementemia group was 55.85±10.89 years, with male predominance (72.3%). Compared with normal complement group, patients with hypocomplementemia were likely to have more involved organs,higher IgG4-RD responder index (IgG4-RD RI), higher laboratory parameters such as counts of eosinophils, inflammatory markers༌immunoglobulin G(IgG), IgG1, IgG3, IgG4 and IgE. In addition, lymph nodes, lacrimal gland༌submandibular gland༌parotid gland༌paranasal sinus༌bile ducts and prostate gland were more commonly affected(p < 0.05). Serum C3 and C4 were negatively correlated with the number of involved organs, IgG4-RD RI, hypersensitive C-reactive protein (hsCRP), IgG, IgG1, IgG3 and IgG4. 64(98.5%) patients responded quickly to initial therapy at 3-month follow-up. Fifteen (23.1%) patients relapsed during follow-up with mean recurrence time of 14.2±13.8 months. Compared with normal complement group, there was no significant difference of relapse rate in two groups (P = 0.7559). Conclusions Clinical characteristics of IgG4-related disease with hypocomplementemia differs from normal complement group.Serum C3 and C4 at baseline before treatment could be biological markers for disease activity. IgG4-RD with hypocomplementemia responded well to treatment and had no significant difference of relapse rate in IgG4-RD with normal complement.
Objective: The presence of circulating antinuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). A variety of ANAs are associated with unique sets of disease manifestations and are widely used in clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvement and prognosis in SSc. This study aimed to investigate the clinical features of SSc patients negative for ANAs in a Chinese Rheumatism Data Center (CRDC) multicenter cohort in China.Methods: Based on the CRDC database, patients were prospectively recruited between April 2008 and June 2019 from 154 clinical centers nationwide, and all cases fulfilled the 2013 ACR/EULAR classification criteria for systemic sclerosis. Results for antinuclear antibodies were intensively collected. Demographic, clinical, and laboratory data were compared between ANA-positive SSc patients and those negative for ANAs.Results: Antinuclear antibodies were detected in 2129 of 2809 patients enrolled in the study; 4.2% patients were negative. There was a significant difference between patients negative and positive for ANAs based on sex (29/60 vs 294/1746, p<0.001). The presence of Raynaud’s phenomenon was less common (71.8% vs 91.8%, p<0.001) in the ANA-negative patients. In addition, the incidence of certain critical organ involvement, including gastroesophageal reflux (5.6% vs 18.5%, p=0.002), interstitial lung disease (65.2% vs 77.9%, p=0.015) and pulmonary arterial hypertension (11.5% vs 29.0%, p=0.006), was significantly lower in ANA-negative patients than in ANA-positive patients. The proportion of abnormal ESR (32.4% vs 47.6%, p=0.013) and IgG elevation (14.3% vs 37.0%, p=0.003), an indicator of disease activity, was significantly lower in ANA-negative patients than in ANA-positive patients.Conclusion: Antinuclear antibodies are strongly associated with the clinical manifestations of systemic sclerosis, with ANA-negative SSc patients tending to exhibit relatively milder disease.
Objectives. Immunoglobulin-G4-related disease (IgG4-RD) is a distinct systemic autoimmune-mediated disease that characterized by tissue fibrosis. The purpose of this study is to investigate the epigenetic modifications in IgG4-RD. Methods. A genome-wide DNA methylation study was performed in B cells, CD4+ T cells and salivary gland tissue from IgG4-RD patients and matched controls using the Illumina HumanMethylation 850K BeadChip. Results. We identified differentially methylated CpG sites including 44 hypomethylated and 166 hypermethylated differentially methylated probes (DMPs) in B cells, 260 hypomethylated and 260 hypermethylated DMPs in CD4 + T cells from 10 IgG4-RD patients compared with 10 healthy controls. We also identified 36945 hypomethylated and 78380 hypermethylated DMPs in salivary gland tissue of 4 IgG4-RD patients compared with 4 controls. We found that DPM2 (cg21181453), IQCK (cg10266221), ABCC13 (cg05699681, cg04985582) were hypermethylated and MBP (cg18455083) was hypomethylated in B cells, CD4+ T cells and tissue of IgG4-RD patients compared with controls. Also, we observed hypomethylated HLA class in both B cells and CD4+ T cells from IgG4-RD patients compared with controls. Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis of DMPs in salivary gland of IgG4-RD patients demonstrated enrichment of pathways in regulation of immune cells responses and fibrosis. Conclusion. Our study is the first DNA methylation study in peripheral B cells, CD4+ T cells as well as salivary gland tissues from patients with IgG4-related disease. Our findings highlighted the role of epigenetic modification of DNA methylation and identified several genes and pathways that might involve in the pathogenesis of IgG4-RD.
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