ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1382

Clinical and Serum IgG4 Characteristics of a Unique British Columbian IgG4-Related Disease Cohort

Luke Chen1, Andre Mattman2, Sujin Park3, Brian Skinnider4, Graham Slack4 and Mollie Carruthers5, 1Hematology, University of British Columbia, Vancouver, BC, Canada, 2Medical Biochemistry, University of British Columbia, Vancouver, BC, Canada, 3University of British Columbia, Vancouver, BC, Canada, 4Pathology, University of British Columbia, Vancouver, BC, Canada, 5Rheumatology, 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) is a fibro-inflammatory disorder that is known to have protean manifestations and variably elevated serum IgG4 concentrations. We present the serum IgG4 characteristics and clinical features of a unique cohort of IgG4-related disease patients in British Columbia. Anecdotally, Asians seem to have more exuberant immunoglobulin production but this has not been formally studied to date.

Methods:

All patients are residents of British Columbia and have biopsy proven IgG4-related disease according to consensus guidelines. Each patient underwent full history and physical examination by two IgG4-related disease physician experts. All patients underwent serum IgG4 levels by nephlometry. Unpaired t-test was used for serum IgG4 comparisons.

Results:

There were 19 patients with IgG4-related disease. The mean age at time of IgG4-RD diagnosis was 61 years old (range: 28-85) and the male:female ratio was 55:45. The time to diagnosis was lengthy at 9 years, on average, from start of symptoms. The organ involvement included salivary glands (n=7), lymph nodes (n=3), lacrimal gland (n=3), kidneys (n=2), genitourinary tumor (n=2), amongst others. Treatments included prednisone (n=10), rituximab (n=5), azathioprine (n=2), cyclosporine (n=2), fludarabine (n=1) and bortezomib (n=1). An additional 4 patients were undergoing observation only. Serum IgG4 levels were on average elevated at 10 g/L ± 9 (cutoff >1.25) as were IgG levels at 35 g/L ± 18 (cutoff >15.2). There was a statistically significant difference between IgG4-RD levels of Chinese and East Asians patients (n=10) averaging 13.8 g/L (range: 2.3-26.9) and Caucasians (n=9) averaging 3.0 g/dL (range: 0.418-9.75) (p=0.021). 

Conclusion:

This is a unique multi-ethnic cohort of IgG4-related disease patients in Canada. There are statistically significant, possibly genetically related, differences in Asian and Caucasian serum IgG4 concentrations.

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

To cite this abstract in AMA style:

Chen L, Mattman A, Park S, Skinnider B, Slack G, Carruthers M. Clinical and Serum IgG4 Characteristics of a Unique British Columbian IgG4-Related Disease Cohort [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/clinical-and-serum-igg4-characteristics-of-a-unique-british-columbian-igg4-related-disease-cohort/. Accessed .

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-serum-igg4-characteristics-of-a-unique-british-columbian-igg4-related-disease-cohort/