Background/Purpose: Takayasu arteritis (TAK) typically affects young women under 40 years old, whereas patients with onset age over 40 years are occasionally observed. It still remains unclear whether the treatment outcomes in patients with elderly onset TAK are different from those in patients with young onset TAK. The aim of this study is to compare clinical features of TAK in patients under 40 years old (yoTAK) with those over 40 years old (eoTAK).

Methods: From a retrospective, multi-center, nationwide registry of TAK and giant cell arteritis (GCA), we enrolled 130 newly diagnosed TAK patients who were treated with glucocorticoids (GCs) between 2007 and 2014. Diagnosis of TAK was made according to the criteria for TAK established by the Ministry of Health, Labour and Welfare for intractable disease in Japan. Overall, 75 of the 130 patients (58%) satisfied the ACR classification criteria for TAK; 54 of 83 (65%) patients with yoTAK (26±7 y.o.) and 21 of 47 (45%) patients with eoTAK (59±12 y.o.). None except one patient in eoTAK met the ACR classification criteria for GCA. The primary outcomes were achievement of remission (disappearance of clinical symptoms with normal C-reactive protein) and relapse-free survival rate.

Results: Among the complication of TAK, hypertension, diabetes mellitus and dyslipidemia were more frequent in eoTAK than yoTAK. No significant differences were observed in chief clinical symptoms (high fever, systemic symptoms, large-vessel lesion, cranial lesion, musculoskeletal disorders, ulcerative colitis) at onset. One-fourth of yoTAK complicated aortic regurgitation, compared with 44% in eoTAK (p=0.0543). Regarding laboratory data, no significant differences were observed between yoTAK and eoTAK except anemia. Serum C-reactive protein concentration in eoTAK tended to be higher than that in yoTAK (yoTAK; 5.1±4.6 mg/dL eoTAK; 7.0±6.7 mg/dL, p=0.0674) but was not statistically significant. HLA-B*52 did not differ between yoTAK (66%) and eoTAK (65%). Remission was achieved 95.2% in yoTAK and 89.4% in eoTAK throughout observational period of two years. The cumulative rate of remission of the yoTAK was significantly higher than that of eoTAK (log-rank test, p=0.0444). The relapse-free survival rate was 50.0% in yoTAK and 41.0% in eoTAK (P=0.4657). The cumulative rate of relapse was not significantly different between yoTAK and eoTAK (log-rank test, p=0.2351). One patient with yoTAK and two patients with eoTAK deceased; no significant difference were found in the cumulative rate of survival between yoTAK and eoTAK.

Conclusion: Clinical features such as symptoms, complications and HLA-B*52 positivity were similar between yoTAK and eoTAK, in Japan. Although both yoTAK and eoTAK achieved remission within 2 years without any significant differences, cumulative rate of remission was poorer in eoTAK.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-and-treatment-outcomes-of-takayasu-arteritis-in-a-nationwide-retrospective-cohort-study-in-japan/