011 Takayasu arteritis: a case series of unusual complications and comorbidities

Abstract

Background: Takayasu arteritis (TA) is a rare vasculitis typically presenting in young women. Localised symptoms result from impaired vascular supply, whilst systemic symptoms are also typical. It mainly affects the thoracic aorta and its branches. Co-existing rheumatological conditions are rarely reported and may lead to diagnostic delay and potentially devastating complications. Methods: We reviewed four cases of patients with Takayasu Arteritis who were seen by the Rheumatology Department in 2017. Results: The first patient presented with fever, back pain, stiff hips and lower limb claudication. MRI of the spine and pelvis showed hip synovitis, enthesitis and inflammation suggestive of axial spondyloarthropathy (axSpA). CT-angiography showed abnormal appearance bilaterally of the common femoral, superficial femoral, popliteal and crural arteries. Rapid onset and symptom severity required potent immunosuppression with prednisolone (1 mg/kg/day), methotrexate (15mg orally/week) and tocilizumab (162mg subcutaneously/week). The second patient was diagnosed with TA in 2006 following left frontoparietal stroke due to right internal carotid artery stenosis. Further investigations revealed additional left subclavian artery stenosis. She received high-dose prednisolone and methotrexate (15 mg/week), later reduced to 7.5mg methotrexate weekly. Nine years after the initial event she was admitted to ICU due to subarachnoid haemorrhage secondary to a dissecting right vertebral artery aneurysm. She subsequently developed bowel infarction secondary to superior mesenteric artery stenosis. CT-angiogram showed stenosis of the superior and inferior mesenteric arteries. In ITU treatment was escalated to pulsed iv methylprednisolone and monthly iv tocilizumab (8mg/kg). The third patient was diagnosed in 2000 with left subclavian artery stenosis despite radiological evidence since 1993. Additionally she developed thrombosis in the left subclavian artery and was diagnosed with primary antiphospholipid syndrome. Treatment with iv weekly tocilizumab (8 mg/kg/month) and methotrexate had to be stopped due to liver impairment. After 16 months off treatment she presented with fatigue, raised inflammatory markers and repeat CT-angiogram showed new vertebral artery stenosis. Treatment with iv tocilizumab was restarted at a reduced dose (4mg/kg/bimonthly). The fourth patient had been referred to the vascular surgeons with aches in her upper limbs and intermittent hand discolouration. She had been diagnosed with hypertension aged 25. Duplex angiography demonstrated left subclavian and axillary stenoses and right brachial stenosis. CT-angiography showed widespread aortic and arterial wall thickening and stenoses, particularly subclavian and axillary arteries, mild thickening of proximal common iliac arteries and irregularity of the left renal artery. Management will be determined pending assessment of disease activity with CT-PET. Conclusion: These cases demonstrate a range of unusual presentations of Takayasu arteritis. Arteries of the aortic arch are most commonly involved, however our cases demonstrate mesenteric, femoral and vertebral artery involvement. We also describe associations with axSpA and primary antiphospholipid syndrome which are rarely reported comorbidities in large vessel vasculitis.