Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances.
Franco DammaccoAnna CirulliAnnalisa SimeonePatrizia LeoneRaffaele PulliDomenico AngilettaGiuseppe RubiniAlessandra Di PaloAngelo VaccaRosanna DammaccoPublished in: Clinical and experimental medicine (2020)
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.
Keyphrases
- end stage renal disease
- newly diagnosed
- ejection fraction
- chronic kidney disease
- multiple sclerosis
- machine learning
- primary care
- mesenchymal stem cells
- patient reported outcomes
- mass spectrometry
- systemic lupus erythematosus
- early onset
- pulmonary arterial hypertension
- photodynamic therapy
- depressive symptoms
- blood brain barrier
- systemic sclerosis
- smoking cessation
- minimally invasive
- adverse drug