Nonspecific aortoarteritis (Takayasu’s disease)

Abstract

The article presents a clinical case of Takayasu’s disease (nonspecific aortoarteritis). On the example of the course of the disease in a young woman, the features of clinical symptoms and diagnostic difficulties are analyzed. Particular attention is paid to the visualization of vascular lesions and the assessment of the dynamics of changes in the vessels as the disease progresses. Changes in laboratory parameters are presented in details according with ongoing therapy. The analysis of literature data of the effectiveness of drug therapy for the disease was carried out. It is emphasized that there is a very limited number of quality placebo-controlled studies in the literature. The practicing physician can only rely on the results of observational studies in his decision’s making process. Also, all studies presented in the literature included a very small number of patients. The algorithm of therapy prescription is given, the first line of which is glucocorticoids. Although there are very few literature data, it is noted that the achievement of remission with isolated glucocorticoid therapy is observed in 50 % of patients. According to the updated EULAR recommendations, after disease verification, therapy should be initiated immediately, the recommended starting dose is 40–60 mg of prednisolone (or equivalent). After achieving control of the disease, it is recommended to gradually reduce the dose to the target 15–20 mg/day for 2 to 3 months after 1 year to 10 mg/day. The next step is to prescribe a non-glucocorticoid immunosuppressant in order to provide both a «steroid sparing» effect and long-term control under the disease. All available information on the efficacy of methotrexate, mycophenolate mofetil, cyclophosphophomide, leflunamide, and azathioprine comes from open-label prospective or retrospective studies with a limited number of participants. Tumor necrosis factor inhibitors and tocilizumab are recommended as second-line drugs. A satisfactory clinical and laboratory response was observed in more than 80 % of patients receiving tocilizumab therapy. In this clinical case, tocilizumab was used and the effects of the drug during treatment for 8 months are described in detail. Immunobiological therapy with tocilizumab was associated with a good clinical and laboratory response, a decrease in vascular stenosis, and an improvement in the patient’s well-being.