Background Takayasu arteritis (TA), a granulomatous large vessel vasculitis affecting young patients, pre-disposes to stenoses and aneurysmal dilatation of the aorta and its branches. Despite morbidity remaining high, evidence for the efficacy of corticosteroids ± an immunosuppressant drug is relatively limited.
Objectives The aim of this study was to determine whether our use of early combined immunosuppression and serial non-invasive imaging improves outcomes.
Methods The case notes, serial imaging and outcome data of 120 patients with TA seen between 2000-2013 were studied longitudinally.
Results 89% of patients were female, with mean age at diagnosis of 31.7 yrs, and a mean of 3 arteries involved (range 1-9). 18FDG-CT-PET facilitated diagnosis, with sensitive detection of active TA. However, it proved relatively insensitive for partially-treated disease. Ultrasound, magnetic resonance (MRA) and CT angiography (CTA) also aided diagnosis and revealed arterial wall thickening and pre-stenotic lesions. Combined immunosuppression was initiated at diagnosis in those with clinically active TA. 89% were prescribed prednisolone plus: methotrexate (45%), azathioprine (38%), mycophenolate mofetil (9%) or cyclophosphamide (10%). Refractory TA, despite at least one immunosuppressant, was seen in 10 patients and responded to TNFa or IL-6R antagonists. 11% had burnt out disease and were not immunosuppressed. Serial annual MRA proved optimal for monitoring, revealing stable disease in 77% and sensitively detecting new lesions in 13%, many of whom had no change in ESR/CRP or clinical exam. Combined immunosuppression reduced the degree of arterial stenosis in 10% of patients. MRA also aided management decisions, allowing accurate steroid titration and low maintenance doses to be achieved. Low dose prednisolone (<10mg/d) was continued for 38.2±27.2 mths to optimize disease control. Cross-sectional analysis (2013) revealed a mean prednisolone dose of 4.01±6.42 mgs. 42.4% had stopped prednisolone, 38.8% were receiving <10mg and 14.1% ≥10mg/d. There were no osteoporotic fractures and <2% steroid-induced diabetes. Open or endovascular surgical intervention was required in 34%, and at a mean 6 years, the patency rates were 79 and 52% respectively, with procedural failure reduced in those receiving adequate preoperative immunosuppression (P=0.001).
Conclusions Non-invasive imaging aids early diagnosis in TA. Combination immunosuppression controls disease activity in the majority and may prevent and occasionally reverse stenoses. Biologics are effective for refractory TA. Serial MRA allows accurate monitoring of arterial anatomy and facilitates targeted titration of therapy and steroid-sparing, improving outcome and minimizing side-effects. Imaging is essential due to the relative insensitivity of ESR/CRP, and complete suppression of acute phase reactants by novel therapies, particularly tocilizumab.
Disclosure of Interest None declared