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.
"Additionally, CRP is produced in the liver in response to the circulating cytokine IL-6, rather than at the site of inflammation, which may account for its low sensitivity [7,21,23]. Relying on these inflammatory markers alone to monitor disease progression can have severe and even lethal consequences for patients [8,10]. Therefore, ESR and CRP should be viewed in the context of the patient’s clinical course and imaging. "
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
Journal of Pharmacology and Pharmacotherapeutics 02/2014; 5(3):193-196. DOI:10.4103/0976-500X.136101
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