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Vasculitides

Vasculitis is inflammation and necrosis of a blood vessel with subsequent impairment of blood flow. The vessel wall destruction leads to perforation and hemorrhage into adjacent tissues. The endothelial injury leads to thrombosis and subsequent impairment of blood flow causing ischemia/infarction of dependent tissues.

  • There is no single typical presentation of vasculitis.

  • Vasculitides are classified by the size of the blood vessel involved: large-, medium-, or small-vessel vasculitis.

  • The types of vasculitis differ widely with regard to age, gender, ethnicity, and clinical presentation.

  • Appropriate tissue biopsies or angiographic studies are usually necessary for diagnosis.

  • The aggressiveness of treatment should be commensurate with the extent of end-organ involvement.

Characteristic histologic features of vasculitis:

  • Infiltration of the vessel wall by neutrophils, mononuclear cells, and/or giant cells

  • Fibrinoid necrosis (panmural destruction of the vessel wall)

  • Leukocytoclasis (dissolution of leukocytes, yielding “nuclear dust”

Immune mechanism:

Vasculitis Nomenclature Agreed at the International Chapel Hill Consensus Conference

2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

NOMENCLATURE

VASCULITIS

Large-vessel vasculitis

Takayasu arteritis

Giant cell (temporal) arteritis (GCA)

Medium-vessel vasculitis

Polyarteritis nodosa (PAN)

Kawasaki disease

Small-vessel vasculitis

Immune-complex-mediated

Anti-glomerular basement membrane (anti-GBM) disease

Cryoglobulinemic vasculitis

IgA vasculitis (Henoch–Schönlein purpura)

Hypocomplementemic urticarial vasculitis (HUVS, anti-Clq vasculitis)

Antineutrophil cytoplasmic antibody-associated (pauci-immune)

Granulomatosis with polyangiitis (GPA)(Wegener’s granulomatosis)

Microscopic polyangiitis (MPA)

Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss)

Variable-vessel vasculitis

Morbus Behçet

Cogan syndrome

Single-organ vasculitis

Cutaneous leukocytoclastic vasculitis

Cutaneous arteritis

Primary central nervous system vasculitis (isolated angiitis of CNS)

Isolated aortitis

Others

Vasculitis associated with systemic disease

Lupus vasculitis

Rheumatoid vasculitis

Sarcoid vasculitis

Others

Vasculitis associated with probable etiology

Hepatitis C virus-associated cryoglobulinemic vasculitis

Hepatitis B virus-associated vasculitis

Syphilis-associated vasculitis

Drug-associated immune complex vasculitis (hypersensitivity vasculitis)

Drug-associated ANCA-associated vasculitis

Cancer-associated vasculitis

Others

There are specific American College of Rheumatology classification criteria for many of the major types of vasculitis. These differentiate one vasculitis from another, rather than describing all of the manifestations of a particular form of vasculitis. Thus, these criteria are rarely useful for diagnosis, and appropriate tissue biopsy is generally necessary for confirmation.

Vasculitis should be suspected in any constitutionally ill patient who has evidence of multisystem inflammatory disease. The clinical manifestations may suggest the size of vessel involved and the most likely vasculitis. Typical presentations:

Large-vessel vasculitis: limb claudication, bruits, asymmetric blood pressures, absence of pulses

Medium-vessel vasculitis: cutaneous nodules, ulcers, livedo reticularis, digital gangrene, mononeuritis multiplex, renovascular hypertension

Small-vessel vasculitis: palpable purpura, urticaria, glomerulonephritis, alveolar hemorrhage, scleritis

--> Headache or visual loss in the elderly (GCA), asymmetric pulses with bruits in a patient <30 years of age (Takayasu arteritis), mononeuritis multiplex (PAN), rapidly progressive pulmonary–renal syndrome (ANCA-associated vasculitis), and palpable purpura (immune-complex-mediated vasculitis) are the most common presentations suggesting vasculitis.

Localized Clinical Features that Suggest Different Types of Vasculitis

SYMPTOMS

DIAGNOSIS

Jaw claudication, visual loss, palpable, thickened, tender temporal artery, or

Giant cell arteritis (GCA)

Diminished temporal artery pulsation

Absent radial pulses, difficulty obtaining a blood pressure in one arm

Takayasu arteritis or large artery involvement in GCA

Sinus involvement, otitis media, scleritis

GPA (Wegener) or EGPA (Churg–Strauss syndrome)

Hypertension, renal vascular involvement

Polyarteritis nodosa or Takayasu arteritis

Asthma

EGPA (Churg–Strauss syndrome)

Testicular tenderness

Polyarteritis nodosa

Pulmonary–renal syndromes (hemoptysis and glomerulonephritis)

GPA (Wegener) and microscopic polyangiitis

Treatment of a vasculitis

General approach

  • Identify and remove inciting agents (i.e., medications, infection, etc.).

  • Treat the primary underlying disease associated with the vasculitis (antibiotics for endocarditis, antiviral therapy for hepatitis B or C).

  • Initiate antiinflammatory and/or immunosuppressive therapy commensurate with the extent of the vasculitis.

  • The combination of cyclophosphamide and prednisone is often regarded as the first choice for induction therapy of generalized and severe types of vasculitis. New targeted biologic agents may replace cyclophosphamide as induction therapy for some forms of vasculitis (e.g., rituximab for ANCA-associated vasculitis).

  • Prevent complications such as infection (PPD, Pneumovax, and other immunizations; trimethoprim–sulfamethoxazole prophylaxis if on high dose prednisone), osteoporosis, and atherosclerosis (control blood pressure and lipids).


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Web:

Vasculitis Foundation

American College of Rheumatology

OrphaNet

Johns Hopkins Vasculitis Center

MayoClinic

Arthritis Foundation