Combined immunosuppressive therapy including a TNF-alpha blocker induces remission in a difficult to treat patient with Takayasu arteriitis and coronary involvement

Section Angiology, University Center for Vascular Medicine and Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany.
VASA.: Zeitschrift für Gefässkrankheiten. Journal for vascular diseases (Impact Factor: 1). 11/2012; 41(6):451-7. DOI: 10.1024/0301-1526/a000236
Source: PubMed


A 40 year old woman presented with symptoms of a systemic inflammatory disease and obstruction of the left subclavian artery. Takayasu arteriitis (TA) was clinically diagnosed and confirmed by MR angiography and FDG-PET scan showing inflammation of the aortic arch and the left subclavian artery. Immunosuppression with glucocorticoids and methotrexate resulted in immediate clinical improvement and normalization of systemic markers of inflammation. Despite that the patient developed chest pain on exertion suggesting coronary involvement, which was confirmed by dobutamine stress echocardiography. After adding the TNF-alpha blocker infliximab coronary symptoms gradually improved and a clinically stable situation could be achieved for more than 6 months. Coronary angiography and aortography showed an occluded main stem of the left coronary artery, an occluded left subclavian artery, and stenoses of the brachiocephalic trunk and the left common carotid artery. Revascularization of the coronary artery and the aortic arch and its branches was performed. The patient returned to work two months after the operation. Immunosuppressive therapy with infliximab and methotrexate is continued, glucocorticoids were stopped after one year of treatment. This case shows that vascular progress in TA patients may occur even when systemic inflammation is controlled, therefore patients have to be carefully observed for new vascular manifestions. TNF-alpha blockers may be an additional treatment option in otherwise difficult to treat TA patients allowing to perform revascularization after a stable disease state has been achieved.

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    ABSTRACT: Takayasu arteritis (TA) is an idiopathic large-vessel vasculitis that can result in significant morbidity and mortality secondary to progressive stenosis and occlusion. Monitoring disease progression is crucial to preventing relapse, but is often complicated by the lack of clinical symptoms in the setting of active disease. Although acute phase reactants such as ESR and CRP are generally used as an indicator of inflammation and disease activity, mounting evidence suggests that these markers cannot reliably distinguish active from inactive TA. We report a 24-year-old Hispanic female with a 5-year history of TA who presented with stroke-like symptoms and evidence of left MCA occlusion on imaging, despite a history of decreasing inflammatory markers. CTA revealed complete occlusion of the left common carotid artery, left subclavian, and left MCA from their origins. It also revealed a striking compensatory circulation supplying the left anterior circulation as well as the left subclavian as a response to progressive stenosis. Monitoring ESR and CRP levels alone may not be a reliable method to evaluate disease progression in patients with TA, and should be taken in context with both patient's clinical picture and the imaging. We recommend that serial imaging be performed regularly in the setting of active disease to monitor progression and allow for immediate therapy in response to evidence of disease advancement, with a relaxation of the imaging interval once the disease is presumed inactive.
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    ABSTRACT: Objective: To explore the effect of sequential treatment with glucocorticoid and tumor necrosis factor‑alpha inhibitors in patients with Takayasu arteritis (TA). Materials and Methods: In five patients with TA, the effects of the sequential treatment with prednisone for 5‑7 months and then with adalimumab (ADA) + methotrexate (MTX) or infliximab + MTX, or with ADA only, for 12 months on both clinical and laboratory findings were evaluated. Results: All treatments improved both symptoms and laboratory parameters without the development of side‑effects. Conclusions: It was hypothesized that MMP‑9 and neutrophil gelatinase‑associated lipocalin could be markers of the response to the treatments.
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