Large vessel vasculitides

Giant cell arteritis (GCA) and Takayasu's arteritis are distinct diseases associated with granulomatous large vessel vasculitis. They present with constitutional symptoms such as fatigue, anorexia, weight loss and fever. In addition, there are specific symptoms related to inflammation and ischaemia in the territory of the affected vessels. In giant cell arteritis which usually occurs in people over age 50 the extracranial arteries are most often involved. In Takayasu's arteritis, which usually occurs in people under age 40, the aorta and its branches are the main sites affected. Ischaemia or aneurysm formation are responsible for the clinical features. Polymyalgia rheumatica (PMR) is a clinical syndrome that may precede GCA. As with GCA and Takayasu's arteritis there is a high acute phase response but there is no diagnostic test. In GCA and Takayasu's arteritis the diagnosis is confirmed by biopsy and angiography respectively. In PMR the classical limb girdle pain, stiffness and constitutional upset respond dramatically to low dose steroids. For GCA and Takayasu's arteritis, high dose steroids are required to prevent permanent damage, and cytotoxic agents may need to be considered.