Vasculitides of large vessels

Abstract

Vasculitides are inflammatory diseases affecting large, medium or small sized vessels, caused by various immunological processes and possibly triggered by infectious agents. Vascular medicine is particularly concerned with vasculitides affecting large vessels: Takayasu arteritis (TA) and giant cell arteritis (GCA, temporal arteritis), which are determined by a similar special immunopathology, further aortitis and Behçet disease (BD). Recent progress in vascular imaging allows the detection of vessel alterations at an earlier stage and a better control of the course of disease. While cranial symptoms prevail in most elder GCA patients, involvement of larger vessels is increasingly perceived. This especially concerns a subgroup called large vessel GCA in which inflammatory obstructions mainly affect the proximal arm arteries or aorta, predominantly in women of the 6th and 7th decade. Initial therapy is based on corticosteroids. Of other immunosuppressive drugs only methotrexate might be considered in GCA. Disease activity has to be monitored both by laboratory parameters and vascular imaging. Aortitis due to GCA may result in late aneurysms even in patients with good response to medical treatment. Aneurysm development may also be the result of isolated necrotizing aortitis. In Behçet disease, vessel involvement is seen in about 20 % of patients. Regarding arteries, aneurysm formation is more frequent than obstruction. Venous thrombosis is often extended and of central location but rarely leads to pulmonary embolism. Although a specific therapy is not established, immunosuppression seems to be of greatest importance and anticoagulation may be withheld.