ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2021

The Proportion Of IgG4-Related Aortitis In Patients Diagnosed As Chronic Inflammation Of Aorta In Pathology

Bon San Koo1, Bin Yoo1, Chang-Keun Lee1, You Jae Kim1, Seokchan Hong2, Yong-Gil Kim1, Wook Jang Seo3 and Kyung joo Ahn4, 1Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea, 2Department of rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea, 3Seoul Veterans Hospital, Seoul, South Korea, 4KEPCO medical center, Seoul, South Korea

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords:

Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases II: Miscellaneous Rheumatic Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose: The purpose of our study was to identify the proportion of IgG4-related aortitis in patients diagnosed with chronic inflammation of aorta in pathology and evaluate histological and clinical characteristics of IgG4-related aortitis.

Methods: We searched electronic medical records of pathologic reports including description of chronic inflammation in aorta specimen. Forty-seven patients had chronic inflammation of aorta and    immunostaining of IgG4 were performed on all biopsy specimen of aorta. If IgG4 positive plasma cell counts were more than 50 per high power field (HPF) and IgG4/IgG ratio were more than 40% with dense lymphoplasmacytic infiltration, fibrosis, or obliterative phlebitis, the patient classified as IgG4-related aortitis.

Results:

Total 47 patients who had chronic inflammation in pathologic specimen of aorta were identified which consisted of 29 idiopathic aortitis, 6 takayasu’s arteritis, 6 behcet’s disease, 3 infection, 3 graft failure patients. Among 29 idiopathic aortitis patients, three patients (10.3%) were classified as IgG4-related aortitis patients. The mean IgG4 positive plasma cell counts of IgG4-related aortitis and idiopathic aortitis were 120.0 ± 52.0 and 7.1 ± 11.7, respectively. One patients had thoracic aortic aneurysm and two patients had abdominal aortic aneurysm. The mean age of IgG4-related aortitis and idiopathic aortitis was 67.0 ± 2.0 and 54.7 ± 15.5 years, respectively (p=0.086). The sex ratio was all male (100%) in IgG4-related aortitis and 19 male in idiopathic aortitis (65%). In one IgG4-related aortitis patient, chronic pancreatitis with atrophy was observed. All IgG4-related aortitis patients underwent surgical treatement with no immunosuppressant. There was no recurrence of IgG4-related aortitis during follow-up period (45.3 ± 28.7 months).

Conclusion: The ratio of IgG4-related aortitis patients was 10% in patients which were diagnosed as idiopathic aortitis in the past. Although the small number of IgG4-related aortitis was found, the old and male patient who had chronic inflammation of aorta with no secondary cause might be related to IgG4-related disease.

Disclosure:

B. S. Koo,
None;

B. Yoo,
None;

C. K. Lee,
None;

Y. J. Kim,
None;

S. Hong,
None;

Y. G. Kim,
None;

W. J. Seo,
None;

K. J. Ahn,
None.

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