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Abstract Number: 757

Long-term Clinical Course and Outcomes of 2013 Patients with Takayasu Arteritis

Hajime Yoshifuji1, Haruhito Uchida 2, Yoshikazu Nakaoka 3, Takahiko Sugihara 4, Mitsuaki Isobe 5 and Masayoshi Harigai 6, 1Kyoto University, Kyoto, Japan, 2Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan, 3National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan, 4Tokyo Medical and Dental University, Tokyo, Japan, 5Sakakibara Heart Institute, Tokyo, Japan, 6Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Shinjuku-ku, Tokyo, Japan

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: damage, severity and activities of daily living (ADL), Takayasu arteritis, treatment

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Session Information

Date: Sunday, November 10, 2019

Session Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster I: Takayasu's Arteritis & Polymyalgia Rheumatica

Session Type: Poster Session (Sunday)

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

Background/Purpose: Takayasu arteritis (TAK) occurs at a young age and has a long-term clinical course. Progression of arterial stenosis or dilatation leads to organ dysfunction. To provide information for improvement of the treatment strategies, we surveyed the treatments, complications, damages, and activities of daily living in the long-term clinical course of TAK, using the data of 2013 patients from the national registry of Japan.

Methods: Data of 211 newly registered and 2584 continuously registered patients were obtained from the case report forms for TAK in the year 2013 from the national registry. We excluded cases with inconsistent data and age of onset >60 years, leaving 76 newly registered and 1937 continuously registered patients. The patients were analyzed by stratification into four groups: 1) new and 2) disease duration < 5 years, 3) 5–20 years, and 4) >20 years (n=76, 273, 766, and 898; mean current age=34, 35, 46, and 62 years; female ratio=84%, 85%, 86%, and 95%, respectively).

Results: The mean prednisolone dose was lower in patients with longer disease duration: (new, < 5 years, 5-20 years, >20 years = 34, 14, 8, and 6 mg/day, respectively) (Table 1). Immunosuppressants (IS) were used in 44% of patients after initiation of glucocorticoid (GC). The frequencies of fever (67%, 30%, 11%, and 4%, respectively) and CRP levels (6.7, 0.8, 0.5, and 0.6 mg/dL, respectively) were also lower in patients with longer disease duration. Conversely, the frequencies of aortic regurgitation (24%, 22%, 32%, and 47%, respectively), aortic aneurysm (12%, 9%, 12%, and 15%, respectively), ischemic heart disease (3%, 4%, 6%, and 19%, respectively), and blindness (0%, 0%, 0.4%, and 1.8%, respectively) tended to be higher in patients with longer disease duration (Fig. 1). The percentage of patients with severe disease (Class IV + V) (20%, 10%, 13%, and 22%, respectively) was high at onset, decreased after initiation of therapy, and showed an increasing tendency depending on disease duration. The percentage of workers was < 50% irrespective of disease duration (Fig. 2).

Conclusion: In this cohort, treatments with GC (initially medium dose) and IS (approximately 40%) were suggested to improve the disease activity of TAK. However, the frequency of complications and damages showed an increasing tendency depending on disease duration, leading to a limited social role.

n=76, 273, 766, and 898 -New, <5 years, 5-20 years, 20 years<-. Items with missing data have smaller numbers. PSL, prednisolone.

Figure 1. Long-term clinical trends of Takayasu arteritis. We stratified 2013 patients by disease duration divided into 5-year intervals and plotted the mean values or percentages of clinical items. IHD, ischemic heart disease; IS, immunosuppressant; PSL, prednisolone.

Social situation of 1963 patients with Takayasu arteritis. We stratified 1963 patients with available data by disease duration divided into 5-year intervals and calculated the percentage of each item.


Disclosure: H. Yoshifuji, Astellas Pharma, 2, Chugai Pharmaceutical, 8; H. Uchida, Kawanishi Holdings, 9, Chugai pharmaceutical, 9, Boehringer Ingelheim, 9, MSD, 9; Y. Nakaoka, Chugai pharmaceutical, 2, 8, Actelion pharmaceuticals, 8, Nippon Shinyaku, 8, Bayer Yakuhin, 2, 8, Astellas Pharma, 8, Novartis, 8, MSD, 2, 8, Daiichi-Sankyo, 2, Takeda Pharmaceutical, 2, Otsuka Pharmaceutical, 2, Pfizer, 2, Mitsubishi Tanabe Pharma, 2; T. Sugihara, Ayumi Pharmaceutical, 9, Ayumi Pharmaceutical Corporation, 2, Chugai Pharmaceutical, 9, Chugai Pharmaceutical Co., Ltd., 2, CSL Behring, 9, CSL Behring K.K., 2, Japan Blood Products Organization, 2, 9, UCB Japan, 9, UCB Japan Co. Ltd., 2; M. Isobe, Chugai pharmaceutical, 8, Daiichi Sankyo, 2, 8, Otsuka Pharmaceutical, 2, 8, Teijin pharma, 2, Mitsubishi Tanabe Pharma, 2, Ono Pharmaceutical, 2; M. Harigai, AbbVie Japan GK, 2, 8, Ayumi Pharmaceutical Co. Ltd., 2, Bristol Meyers Squib, 2, 5, 8, Bristol-Myers Squibb Co. Ltd, 2, 5, 8, Chugai Pharmaceutical Co. Ltd., 2, 5, 8, Chugai Pharmaceutical Co., Ltd., 2, 5, 8, Eisai Co. Ltd., 2, Eisai Co., Ltd., 2, Eli Lilly, 5, 8, Mitsubishi Tanabe Pharma Co., 2, Mitsubishi Tanabe Pharma Corp., 2, Nippon Kayaku Co. Ltd., 2, Taisho Toyama Pharmaceutical Co. Ltd., 2, Takeda Pharmaceutical Co., 2, Takeda Pharmaceutical Co., Ltd., 2, Teijin Pharma Ltd., 2, 8, Teijin Pharma, Ltd., 2, 8.

To cite this abstract in AMA style:

Yoshifuji H, Uchida H, Nakaoka Y, Sugihara T, Isobe M, Harigai M. Long-term Clinical Course and Outcomes of 2013 Patients with Takayasu Arteritis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/long-term-clinical-course-and-outcomes-of-2013-patients-with-takayasu-arteritis/. Accessed February 3, 2023.
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