Background/Purpose: Takayasu arteritis (TA) affects the aorta and its branches, pre-disposing to stenoses and aneurysmal dilatation. While morbidity remains high, evidence for efficacy of immunosuppressive therapy is relatively limited. The purpose of this study was to analyze our use of combined immunosuppression with serial non-invasive imaging, to show that early intervention and detailed monitoring improves outcome. 

Methods: A longitudinal study of case notes, imaging studies and outcome data in 110 patients seen with TA from 2000-2013.

Results: All TA sub-types were found, with a mean of 3 arteries involved (range 1-9). 89% were female, with mean age at diagnosis 31.5 yrs. ¹⁸FDG-CT-PET was used for detection of active disease at diagnosis and identified pre-stenotic disease. Ultrasound, magnetic resonance (MRA) and CT angiography (CTA) aided diagnosis, and also revealed pre-stenotic lesions. Combined immunosuppression was initiated at diagnosis, with 90% prescribed prednisolone, plus methotrexate (43%), azathioprine (37%), mycophenolate mofetil (9%) or cytoxan (10%). TNFa and IL-6R antagonists were used effectively in 9 patients with refractory disease. 10% had burnt out disease and were not immunosuppressed. Serial MRA was used for monitoring, revealing stable disease in 82% and sensitively detecting new stenoses/aneurysms in 9.8% of patients, many of whom had no change in ESR/CRP or clinical exam. Subsequent treatment escalation prevented progression, and MRA revealed improved arterial stenoses in 9 patients (8.2%). Echocardiography and cardiac MRI identified silent myocardial injury in 22%. MRA also aided management decisions, allowing accurate steroid titration and low maintenance doses (<10mg/d) to be safely achieved. Low dose prednisolone was continued for 38.2±27.2 mths to optimize disease activity control, with no osteoporotic fractures and <2% steroid-induced diabetes detected. Cross-sectional analysis (2013) revealed a mean prednisolone dose of 3.22±4.97 mgs. 50% had stopped prednisolone, 41.4% were receiving ≤10mg and 8.6% >10mg/d. Two deaths were recorded, both unrelated to complications of TA or its treatment. 

Conclusion: Our analysis suggests that non-invasive imaging aids early diagnosis, and that prednisolone with an immunomodulator controls disease activity and can prevent and occasionally reverse stenoses. Serial MRA allows accurate monitoring of arterial anatomy and facilitates targeted titration of therapy and steroid-sparing, improving outcome and minimizing side-effects. Arterial imaging is also essential due to the relative insensitivity of ESR/CRP in assessing disease activity, and their complete suppression by novel therapies, particularly tocilizumab.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/early-combination-immunosuppression-and-serial-non-invasive-imaging-improves-outcome-in-takayasu-arteritis/