Prognosis

Remission of disease is usually defined as the lack of clinical and laboratory features of disease, with no evidence of new vascular lesions on follow-up imaging examinations.[2][8] Most patients achieve disease remission, although the majority require immunosuppressive therapy in addition to glucocorticoids.[8] Monophasic disease is described in about 20% of patients.[2] In one series, sustained remission, lasting for at least 6 months while on <10 mg of prednisone daily, was attained by only 28% of patients, and only 17% remained in remission after prednisone was discontinued.[8]

Disease relapses occur in >80% of all patients who go into remission.[2][8] Relapses can occur despite ongoing immunosuppressive treatment. Relapses manifest as new vascular lesions on imaging studies are typically associated with elevation of acute phase markers, but this laboratory evidence of active disease can be lacking.[8][56]

Mortality and morbidity

Cardiac failure is a common cause of death.[24] Long-term morbidity is related primarily to complications from vascular ischemia. Symptomatic extremity claudication occurs in about 50% of patients. Upper-extremity claudication is more common than lower-extremity symptoms. Thoracic aortic aneurysm, aortic valve involvement, and arteritis of coronary and pulmonary arteries are known complications that are associated with increased mortality. The 5-year mortality in Takayasu arteritis is estimated to be between 70% and 93%.[57]

Pregnancy

Because Takayasu arteritis is primarily a disease of young women, pregnancy is often a consideration. There are few data about pregnancy in patients with Takayasu arteritis, but successful pregnancies have been reported.[8][58] In one series of patients, the annual incidence of pregnancies fell after the diagnosis of Takayasu arteritis, and the percentage of miscarriages showed an upward trend.[58] Careful management of hypertension is necessary during pregnancy.

Use of this content is subject to our disclaimer