Takayasu arteritis: challenges in diagnosis and management

Abstract

Takayasu arteritis (TA) is a rare disease affecting chiefly young women, although it can affect both men and women and persons of many different ethnicities. TA carries a high morbidity rate, but importantly, overall mortality has declined over time such that the 15-year survival rate has increased from 82.9% for patients diagnosed between 1957 and 1975 to 96.5% for those diagnosed from 1976 to 1990. Severity of presenting arterial complications and delay to diagnosis have also decreased over the past decade owing to advances in non-invasive diagnostic imaging and the development of medical therapies. Despite these advances, there still remain significant gaps in the diagnosis and management of these complex patients. These gaps encompass the basic, yet extremely complex, tasks of defining a universally accepted diagnostic criterion, accurate assessment of disease activity and development of clinically meaningful and accurate outcome measures to guide necessary clinical trials for the management of these complex patients.

Pulmonary hypertension in Takayasu arteritis

“…All of our patients had favorable short‐term prognoses. Nevertheless, the reported mortality rate of 8% in TA patients with PH during approximately 3 years of follow up, is clearly higher than the expected mortality rate in TA patients and supports the prognostic importance of PH . Therefore, echocardiography should take part in the diagnostic workup of all TA patients during the initial evaluation and repeated echocardiographic assessment should be performed if there are symptoms suggesting PH.…”

Translating IL-6 biology into effective treatments

“…Takayasu arteritis and GCA are chronic, potentially life-threatening, primary systemic large-vessel vasculitides 122,123 . Takayasu arteritis affects the aorta and its major branches in adolescents and young adults, whereas GCA affects large and medium-sized arteries and usually affects individuals above the age of 50 years.…”

Large-Vessel Vasculitides: Takayasu Arteritis and Giant Cell Arteritis

“…There are no gold standard imaging or laboratory tests with adequate sensitivity or specificity for the diagnosis of TA [5]. Testing for inflammatory markers such as the erythrocyte sedimentation rate (ESR) and the acute-phase reactant C-reactive protein (CRP) indicates the presence of a systemic inflammatory process; however, normal values may occasionally be observed and should not unequivocally rule out a diagnosis of TA.…”