Systemic vasculitis

The systemic vasculitides are subacute illnesses associated with signs and symptoms of inflammation that can affect virtually any organ system.

Many of these diseases have typical patterns of organ involvement.

The diagnostic value of a biopsy, performed early in the course of illness, cannot be overstated. However, biopsy may not always be possible due to the site of affected tissue.

Treatment depends on the type of vasculitis and the severity of the clinical manifestations, but typically involves a corticosteroid and/or other immunosuppressive therapy.

In its strictest sense, the term vasculitis denotes inflammation of a blood vessel, which is characterized by the presence of an inflammatory infiltrate and destruction of the vessel wall. Commonly, however, vasculitis refers to the systemic vasculitides, which are autoimmune disorders characterized by inflammation of blood vessels. The systemic vasculitides are a diverse group of disorders that demonstrate a wide range of organ involvement and clinical severity.

This topic provides a general overview of the diagnosis and management of the systemic vasculitides. For more details on specific disorders see:

• Takayasu arteritis

• Giant cell arteritis

• Polyarteritis nodosa

• Kawasaki disease

• Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis)

• Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome)

• Goodpasture disease (also known as anti-glomerular basement membrane disease)

• Cryoglobulinemia (also known as cryoglobulinemic vasculitis)

• IgA vasculitis (formerly known as Henoch-Schonlein purpura)

• Behcet syndrome