ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1383

T-Cell Subset Analysis in IgG4-Related Disease and Lymphocyte-Variant Hypereosinophilic Syndrome

Mollie Carruthers1, Bakul Dalal2, Sujin Park3, Graham Slack2, Andre Mattman4 and Luke Chen5, 1Rheumatology, University of British Columbia, Vancouver, BC, Canada, 2Pathology, University of British Columbia, Vancouver, BC, Canada, 3University of British Columbia, Vancouver, BC, Canada, 4Medical Biochemistry, University of British Columbia, Vancouver, BC, Canada, 5Hematology, University of British Columbia, Vancouver, BC, Canada

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords:

Session Information

Date: Monday, November 9, 2015

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster Session II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

IgG4-related disease (IgG4-RD) and lymphocyte-variant hypereosinophilic syndrome (L-HES) share elevated peripheral eosinophilia and IgE levels as well as common clinical features including atopy, cutaneous lesions and lymphadenopathy. Distinguishing between these two diseases is a diagnostic challenge.  L-HES is driven by an aberrant population of T lymphocytes characterized by an abnormal immunophenotype, clonal T-cell receptor rearrangements, or both.  Determining whether there is overlap in the immunphenotype of T-cells in IgG4-RD with L-HES will be helpful in the diagnosing and understanding the pathophysiology of these conditions.

Methods:

IgG4-RD patients were biopsy proven according to consensus guidelines and HES patients fit WHO criteria for HES including persistent blood eosinophilia >1,500/mm3 at a single Canadian academic center. There were 9 patients with L-HES and 11[p1]  patients with IgG4-related disease. Peripheral blood T-cell immunophenotyping was performed by multicolor flow cytometry using an antibody panel consisting of CD2, 3, 4, 7, 8, 45 and 56. Polymerase chain reaction was used to analyze T-cell receptor clonality.

Results:

There were three IgG4-related disease patients with elevated CD4+/7- T-cell subsets median 14.5% (range 11-16.5; cut-off>10) and 6 additional IgG4-RD patients had normal subsets. Two IgG4-related disease patients had elevated NK cells. None of the IgG4-RD patients had a clonal T-cell receptor rearrangement by PCR.  All nine L-HES patients had an abnormal phenotype, including increased CD4+/3- (n=4), CD4+/3-/8- (n=3), CD4+/7- (n=1) and increased NK cells (n=1). There were 5 HES patients, out of 8 total measured, with clonal T-cell receptor rearrangements by PCR, including the patient with an elevated CD4+/7- population.[p2]

Conclusion:

Five of eleven patients with IgG4-RD had immunophenotypic abnormalities of the T-lymphocytes which overlap with the immunophenotypic abnormalities seen in L-HES.  However, none of the IgG4-RD patients had a clonal T-cell rearrangement by PCR, indicating that the expanded CD4+/7- and NK populations seen in this disease are likely a nonspecific finding also seen in other inflammatory/autoimmune disorders.

Disclosure: M. Carruthers, None; B. Dalal, None; S. Park, None; G. Slack, None; A. Mattman, None; L. Chen, None.

To cite this abstract in AMA style:

Carruthers M, Dalal B, Park S, Slack G, Mattman A, Chen L. T-Cell Subset Analysis in IgG4-Related Disease and Lymphocyte-Variant Hypereosinophilic Syndrome [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/t-cell-subset-analysis-in-igg4-related-disease-and-lymphocyte-variant-hypereosinophilic-syndrome/. Accessed .

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