Background Takayasu arteritis (TAK) is a rare, chronic large-vessel vasculitis of unknown etiology predominantly affecting the aorta, its major division branches, and the pulmonary arteries. TAK is more common in Asia and few series from European countries exist.
Objectives To describe the main epidemiologic, clinical, therapeutic, and survival features, of a series of patients with TAK from a single tertiary referral center in Spain.
Methods Retrospective study of all patients diagnosed and followed at the Internal Medicine Department of Vall d'Hebron University Hospital (Barcelona), between 1967 and 2014.
Results 23 patients (83% women) that fulfilled at least three of the 1990 ACR criteria, and one patient who was diagnosed at autopsy, were included. Mean age at diagnosis was 36 y (range 14-71), with 25% of patients diagnosed after 40 years of age. Mean diagnosis delay between the first clinical manifestation attributable to TAK and diagnosis of the disease was 38 months. Median follow-up was 12 years. Most common symptoms at diagnosis were claudication of the upper (42%) or lower (25%) limbs, and carotidynia (12%) and most common signs were asymmetry or absence of pulses (88%), hypertension (42%), a difference >10 mmHg in systolic blood pressure between arms (71%), and the presence of arterial bruits in carotid (29%), subclavian (33%) or aorta (29%). In four patients (17%) a neurovascular event was the presenting feature. Only 21% of patients reported systemic symptoms, but anemia or increased acute phase reactants were present in 38% of patients at diagnosis. Five patients (21%) had type I TAK, two (8%) have type IIa TAK, three (13%) have type IIb TAK, two (8%) have type III TAK, two (8%) have type IV TAK and ten (42%) have type V TAK. Pulmonary and coronary artery involvement was present in five (21%) and three (13%) patients, respectively. During follow-up most patients developed ischemic signs or symptoms at different levels: neurological (46%), upper limbs (42%), lower limbs (25%), renal (21%), mesenteric (12%) or coronary (12%). Antiplatelet drugs were used in 96% of patients. Corticosteroids were used as first-line treatment in 79% of cases, and 46% of patients received immunosuppressive treatment (methotrexate in nine patients (38%), azathioprine and micofenolate in two patients each, and infliximab in one patient). 54% of patients required vascular interventions: 29% a surgical procedure, and 25% a percutaneous procedure. Three patients died during follow-up. One of them (a 32-year woman) was definitely diagnosed of TAK at autopsy.
Conclusions Although TAK predominates in young women, due to diagnostic delay, it can be diagnosed in patients older than 40 y. Diagnosis is made usually in inactive phase and in basis of chronic sequelae (mainly stenosis). Immunosuppressant therapy is usually effective controlling the disease although half of patients require some vascular intervention during follow-up. Patients with TAK have a low mortality, but a substantial morbidity.
Arend et al.The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum.1990;33.
Hataet al. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol. 1996;54(Suppl).
Disclosure of Interest None declared