Chapter 25 - Vasculitis and arteritis: how to recognize and manage them

Abstract

Vasculitis and arteritis are rare heterogeneous disease entities that affect blood vessel walls and perivascular tissues, mainly as a consequence of immunologically mediated inflammatory reaction. These diseases are complex disorders due to vessel wall damage (aneurysm and rupture) or obstruction of lumen leading to infarction of tissue. Both venous and arterial vessels can be affected, in a pattern of single-organ or multisystemic disease. The pathogenetic mechanisms of systemic vasculitis are still partly unknown, as well as their genetic basis. For most of the primary systemic vasculitis, a single gold standard test is not available and diagnosis is often made after having ruled out other mimicking conditions. The annual incidence of vasculitis is 4060 cases per 1 million persons.