Vasculitis

Vasculitis, characterized by inflammation and necrosis, manifests a wide spectrum of presentation by involving a vasculature of various sizes and locations. A definitive diagnosis of vasculitis invariably requires histologic confirmation since there are no diagnostic clinical, imaging, or laboratory findings. The most widely adopted vasculitis classification is the Chapel Hill Consensus Conference (CHCC) nomenclature of systemic vasculitis which integrated clinical symptoms, histopathologic features, and laboratory findings. This classification accounts for the size of the involved vessels. This chapter outlines the clinical and histologic features of the small-vessel vasculitis including the immune complex vasculitis and antineutrophil cytoplasmic antibody-associated vasculitis; medium-vessel vasculitis such as polyarteritis nodosa and Kawasaki disease; large-vessel vasculitis, namely, giant cell arteritis and Takayasu arteritis; variable-vessel vasculitis such as Behcet disease and Cogan syndrome; and vasculitis associated with systemic diseases including rheumatoid arthritis, lupus vasculitis, and sarcoid vasculitis. Vasculitis can also be secondary to drugs, infection, underlying systemic disease, or trauma. Therefore, a diagnosis of vasculitis cannot be based on histologic ground alone. Clinical pathologic correlation is necessary.