Takayasu arteritis: showed by a hypertensive crisis

ABSTRACT

Takayasu Arteritis (TA) is a primary granulomatous large vessel vasculitis, affecting predominantly young women1. It mainly affects the aorta and its major branches. TA and the required immunosuppressive therapy cause considerable morbidity and mortality2. Early in the disease course, symptoms can be non-specific, leading to a difficult diagnosis3. Consequences of vascular stenosis, occlusions, and, less commonly, vascular dilation, account for the typical clinical presentation; the latter can sometimes lead to aneurysmal rupture or dissection. Though the importance of a comprehensive history and a thorough physical examination cannot be over-emphasized, clinical assessment is frequently inaccurate when evaluating disease activity, which may sometimes progress silently. Also the diagnostic modalities currently used are unsatisfactory. X-Ray angiography, the "gold standard" for TA diagnosis, can image luminal defects but does not detect changes of the vessel wall4. The diagnosis of TA can be confirmed with histopathological examination; however, tissue from blood vessels is obtained only in the minority of patients requiring a surgical intervention. There are currently no specific biomarkers for diagnosing TA. Identifying disease activity in TA is challenging3.Glucocorticosteroids are anchor drugs for this disease, like other vasculitis. Most cases in Japan respond with 0.3-0.5 mg/kg/day prednisolone, but we frequently found that some patients revealed flare-ups during tapering of glucocorticosteroids. Since TAK mainly affects young women, side-effects of glucocorticosteroids, especially moon face, severely damage their quality of life. Immunosuppressive agents, including methotrexate, cyclosporine, cy-clophosphamide, mycophenolate mofetil and TACROLIMUS have been used for patients with TAK. Biological agents targeting tumor necrosis factor (TNF) have also been used for patients with TAK. Since IL-6 is highly expressed within inflamed arteries and serum levels correlate with disease activity, block-ing IL-6 showed effectiveness in TA. Tocilizumab is a humanized monoclonal antibody against the IL-6 receptor, and the first report of successful use of tocilizumab in a patient with refractory TA was published in 2008. Later, nine additional cases of TA treated with tocilizumab 8 mg/kg every 4 weeks were reported. In the majority of the cases, disease activity improved and CS doses were discontinued or tapered. Abatacept is another promising biologic agent inhibiting the co-stimulation of T cells, and is currently being investigated in the first randomized, placebo-controlled trial of LVV patients including TA7.This article aims the description of a clinical case of a serious appearance form of an uncommon disease, Takayasu's arteritis

RESUMEN

No disponible