Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances

Abstract

Takayasu arteritis (TAK) is a rare granulomatous vasculitis of unknown etiology that mainly affects the aorta and its major branches. The aim is to describe the clinical features, diagnostic procedures, pathogenesis, and management of TAK in a longitudinal cohort of patients recruited within a single region of southern Italy. The cohort included 43 patients who were diagnosed with TAK and followed up according to a standard protocol, in a collaboration between four university tertiary referral centers and a regional hospital. Clinical and imaging classification criteria were those established by the American College of Rheumatology. Thirty-five patients (81.4%) were female, and the mean age at disease onset was 32.6 (range 16–54) years. Angiographic assessment of the vascular involvement allowed disease classification in five different types. Clinical features ranged from constitutional symptoms in the early inflammatory stage of the disease to cardiovascular ischemic symptoms in the late, chronic stage. Noninvasive imaging techniques were employed to assess the extent and severity of the arterial wall damage and to monitor the clinical course and response to therapy. Medical treatment, based on pathogenetic insights into the roles of humoral and cell-mediated immune mechanisms, included glucocorticoids mostly combined with steroid-sparing immunosuppressive agents and, in patients with relapsing/refractory disease, biologic drugs. Significant clinical and angiographic differences have been detected in TAK patients from different geographic areas. Patients with life-threatening cardiovascular and neurologic manifestations as well as sight-threatening ophthalmologic signs and symptoms should be promptly diagnosed, properly treated, and closely followed up to avoid potentially severe consequences.

Arterial Stiffness and Adult Onset Vasculitis: A Systematic Review

“…Takayasu's arteritis (TAK) is a chronic vasculitis of unknown etiology with a predilection for the major elastic arteries such as the aorta, its main branches and the pulmonary arteries ( 41 , 42 ). The media and the adventitia of the arterial walls are predominantly involved, with intimal thickening and arterial occlusions as important late phenomena ( 42 , 43 ).…”

“…The media and the adventitia of the arterial walls are predominantly involved, with intimal thickening and arterial occlusions as important late phenomena ( 42 , 43 ). During the early active stage of the disease, arterial biopsy shows granulomatous inflammation and patchy destruction of the medial musculoelastic lamellae ( 41 , 44 ). Later the microscopic changes become nonspecific and consist of sclerosing arteritis, fibrous intimal hyperplasia, medial scarring and adventitial fibrosis ( 43 , 45 ).…”

Takayasu’s Arteritis With Acute Severe Aortic Regurgitation Requiring a Bio-Bentall Procedure

“…If the coronary or pulmonary arteries are involved, a distinction is added to the type, C(+) or P(+), respectively. A small European study reports type I as the most common variant (25.6%), followed by type V (20.9%), type IIa (18.6%), type IV (13.9%), type IIb (11.7%), and type III (9.3%) [12].…”

Anemia in Patients with Takayasu’s Arteritis and its Correlation with Disease Activity

“…The prevalence of anemia in women is higher than that in men [11], TAK is more common in women, the male/female ratio ranges from 1:5 to 1:9 [12], and anemia patients with TAK are also more common in women. We found anemia patients with TAK were younger.…”