Abstract Number: 1384 • 2015 ACR/ARHP Annual Meeting
Comparison of IgG4-Related and Non-IgG4-Related Retroperitoneal Fibrosis; A 12 Year Retrospective Study
Comparison of IgG4-related and Non-IgG4-related Retroperitoneal fibrosis; a 12 year Retrospective Study Background/Purpose: Retroperitoneal fibrosis (RPF) is a rare disease characterized by a progressive proliferation…Abstract Number: 2805 • 2014 ACR/ARHP Annual Meeting
IgG4-Related Disease: Baseline Features in 100 Patients with Biopsy-Proven Disease
Background/Purpose IgG4-related disease (IgG4-RD) has emerged as an immune-mediated disease that links multiple fibro-inflammatory conditions through common pathology and pathophysiologic mechanisms. Most of the literature…Abstract Number: 2806 • 2014 ACR/ARHP Annual Meeting
Characteristic Phenotype of Peripheral Blood Lymphocytes in Patients with IgG4-Related Disease, Comparing to Primary Sjögren Syndrome and Healthy Controls
Background/Purpose : IgG4-related disease (IgG4-RD) is a systemic disease that is characterized by the infiltration of IgG4-positive plasma cells and T cells into various organs.…Abstract Number: 1650 • 2014 ACR/ARHP Annual Meeting
Prevalence and Prognostic Implications of IgG4 in Membranous Lupus Nephritis
Background/Purpose: Patients with membranous lupus nephritis (MLN) have increased risk of thrombosis and different prognosis than other classes of lupus nephritis (LN). Previous studies demonstrated…Abstract Number: 1239 • 2014 ACR/ARHP Annual Meeting
Is Lymphocytic Sialadenitis IgG4-Related?
Background/Purpose: To assess the prevalence of IgG4-related disease among patients with lymphocytic sialadenitis on labial salivary gland biopsy. Methods: All labial salivary gland biopsies (LSGB)…Abstract Number: 1238 • 2014 ACR/ARHP Annual Meeting
Proportion of Peripheral Plasmacytoid Dendritic Cells and Plasmablasts Reflects Disease Activity in IgG4-related Disease
Background/Purpose Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disease with multi-organ system involvement. Affected patients frequently have a history of bronchial asthma and allergic rhinitis. The reported pathogenesis of IgG4-RD describes the clear involvement of excessive Th2 cells and regulatory immune reaction in addition to plasma cells 1). However, peripheral immune cell phenotype, which reflects disease status, has not been comprehensively evaluated. Our aim was to definitively determine peripheral blood cell abnormalities and their correlation with disease activity in patients with IgG4-RD.Methods Peripheral blood samples were obtained from active untreated IgG4-RD patients (n=11) and healthy controls (n=16). Comprehensive immunophenotyping assay with information on activation status was done by multi-color flow cytometry, and the proportion of peripheral blood mononuclear cells (PBMCs), including T cells (naïve/memory, Th1/2/17, Treg, and Tfh), B cells (naïve/memory, plasmablast, Breg), monocytes (classical, intermediate, non-classical) and dendritic cells (myeloid, plasmacytoid), and their activity status were precisely defined. Disease activity was measured using the IgG4-RD responder index (RI). Statistical analysis was done using the Mann-Whitney U test and Spearman rank correlation coefficient test.Results The proportion of plasmablasts (CD19+CD20-CD27+CD38+), memory Th2 cells (CD3+CD4+CXCR3-CCR6-CD45RA-), Tregs (CD3+CD4+CD25+CD127low), Tfh (CD3+CD4+CXCR5+), and mDCs (CD3-CD19-CD14-HLA-DR+CD1c+CD303-) in peripheral blood was significantly increased in IgG4-RD patients compared with HC, whereas the proportion of pDCs (CD3-CD19-CD14-HLA-DR+CD1c-CD303+)was significantly decreased. Interestingly, the proportion of pDCs in total DCs was negatively correlated with IgG4-RD RI (r=-0.778, p=0.005) while the proportion of plasmablasts in CD19+cells was positively correlated with RI (r=0.701, p=0.016). Further, the increased proportion of plasmablasts was positively correlated with serum IgG4 level (r=0.718, p=0.013) while the decreased proportion of pDCs tended to be negatively correlated with the number of affected organs (r=-0.518, p=0.061). Conclusion Our comprehensive analysis identified distinct proportional changes in PBMCs in IgG4-RD. In particular, the decrease in pDCs and increase in plasmablasts were strongly linked with disease activity. These combined measurements are expected to be clinically useful surrogate cell markers. This newly identified decrease in circulating pDCs may be involved in the pathogenesis in IgG4-RD via the recently described role in the enhancement of Th2 response 2).References: 1) Stone JH et.al. N Engl J Med 2012;366:539-51 2) Maazi H et.al. Allergy 2013;68:695-701Abstract Number: 1236 • 2014 ACR/ARHP Annual Meeting
IgG4 Immunostaining Is Common but Not Specific in Orbitbal Inflammatory Diseases
Background/Purpose: IgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we…Abstract Number: 1235 • 2014 ACR/ARHP Annual Meeting
Recovery of Renal Function after Corticosteroid Therapy for IgG4-Related Kidney Disease
Background/Purpose: In our earlier study of IgG4-related kidney disease (IgG4-RKD), we found that renal dysfunction, which was mostly attributable to IgG4-related tubulointerstitial nephritis, was significantly…Abstract Number: 784 • 2013 ACR/ARHP Annual Meeting
IgG4+ Plasmablasts Are A Novel Biomarker In IgG4-Related Disease
Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated disorder that responds to B cell depletion with rituximab (RTX). We detected IgG4+ plasmablasts in the sera of…Abstract Number: 785 • 2013 ACR/ARHP Annual Meeting
Ophthalmic Manifestations Of IgG4-Related Disease: A Single-Center Experience
Background/Purpose: IgG4-related disease (IgG4-RD) is an inflammatory disorder responsible for fibrosing, tumefactive lesions that can present in nearly any anatomic location. The orbital manifestations of…Abstract Number: 2649 • 2013 ACR/ARHP Annual Meeting
Rituximab For The Treatment Of IgG4-Related Disease: A Prospective Clinical Trial
Background/Purpose: IgG4-related disease (IgG4-RD) is a multi-organ, fibro-inflammatory disorder. An open label pilot trial of rituximab (RTX) in IgG4-RD conducted at twocentershas enrolled 29 of…Abstract Number: 2020 • 2013 ACR/ARHP Annual Meeting
Clinical Characteristics In Japanese Patients With IgG4-Related Disease
Background/Purpose: IgG4-related disease (IgG4-RD) is the new entity, which is a multisystem and fibroinflammatory condition. It is characterized by progressive scarring and dysfunction of affected…Abstract Number: 2024 • 2013 ACR/ARHP Annual Meeting
Elevated IgG4+ Plasmablasts In Patients With Active, Untreated IgG4-Related Disease
Background/Purpose: IgG4-delated disease (IgG4-RD) is an immune-mediate condition responsible for inflammatory lesions that can affect nearly every organ. Diagnosis is challenging as it relies upon…Abstract Number: 924 • 2013 ACR/ARHP Annual Meeting
B Cell Subsets and Dysfunction Of Regulatory B Cells In IgG4-Related Diseases and Primary Sjögren Syndrome: The Similarities and Differences
Background/Purpose: IgG4 related disease (IgG4-RD) is a chronic, multisystem-involved autoimmune disease. Abnormally activated and differentiated B cells may play important roles. Regulatory B cells (Breg)…
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