Background Takayasu arteritis (TAK) is well known to be more common in East and Southeast Asian countries compared with the Western countries. In Japan, 5881 persons were registered in the fiscal year 2012 by the report on public health administration and service by the Ministry of Health, Labour and Welfare. Moreover, TAK was most common vasculitis (43%) in the primary vasculitis in our recent database. However, only a few large scale and high quality case-series studies with sufficient information are reported worldwide. [1-5]
Objectives We investigated the medical information of the consecutive patients with TAK in a state of the art high volume center in central Tokyo, Japan and revealed recent clinical characteristics of TAK.
Methods All 52 patients who were clinically administrated as TAK in our department from April 2008 until December 2013 were enrolled in a single center case-series study. Clinical information including imaging from medical records was collected and statistically analyzed. Series of the data were compared with past studies including others countries.
Results The average age at the time of diagnosis was 34.6±18.7 years old. (Table 1) 7 were male and 45 were women. (86.5%) The disease classification according to the angiographic classification of the International TAK Conference in Tokyo 1994 are following. Type I were 15 (34.9%), Type IIa: 5 (11.6%), Type IIb: 4 (9.3%), Type III: 7 (16.3%), Type IV: 2 (4.7%), Type V: 10 (23.3%), and 9 were unclassified because of insufficient information. Type III were more frequent while Type V were less in comparison with others. New imaging devices such as computed tomography, magnetic resonance angiography, 18F-fluotodeoxyglucose positron emission tomography were used for the validation of vascular involvement in most of the recent cases. 6 TAK cases were accompanied with inflammatory bowel disease including 5 ulcerative colitis and 1 Crohn's disease seemed to be remarkable in this series. From the point of view of treatment, corticosteroids were used in 40 cases (88%) as standard treatment for active vasculitis. Various immunosuppressant were concomitantly used in 12 cases (23.1%) (methotrexate (MTX): 6, azathioprine (AZ): 3, MTX+AZ: 1, 6-mercaptopurine: 1, tacrolimus: 1). 7 cases had treated by biologics (13.5%) including 6 infliximab and 1 tocilizumab. Immunosuppressant and biologics were used for recurrent and intractable cases (25%). Biologics were selected not in a few cases (13.4%) different from past studies and successfully induced remission in 5 out of 6 cases (83%). Number of the cases with a surgical treatment history was 8 (15.4%) including Bentall procedure.
Conclusions Latest our case series in Japan shed the light on the successful intensive immunosuppressive and biologics treatment for the difficult TAK cases rather than characteristics in angiographic classification and surgical treatment in comparison with past reports.
Hata A et al. Int J Cardiol 1996;54 Suppl: S155-163
Bicakcigil M et al. Clin Exp Rheumatol. 2009; 27(Suppl 52): S59-64.
Jain S et al. Int J Cardiol. 1996;54 Suppl: S111-6.
Soto ME et al. Clin Exp Rheumatol. 2008;26 (Suppl 49): S9-15.
Ohigashi H et al. Circ J. 2012;76(4): 1004-11.
Disclosure of Interest None declared