- Definition and Pathogenesis
- Primary Vasculitis Syndromes
- Granulomatosis with Polyangiitis (Wegener's)
- Microscopic Polyangiitis
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
- Polyarteritis Nodosa
- Giant Cell Arteritis
- Takayasu Arteritis
- IgA Vasculitis (Henoch-Schönlein)
- Cryoglobulinemic Vasculitis
- Idiopathic Cutaneous Vasculitis
- Miscellaneous Vasculitic Syndromes
- Secondary Vasculitis Syndromes
- Evaluation
- Differential Diagnosis
A clinicopathologic process characterized by inflammation of and damage to blood vessels, compromise of vessel lumen, and resulting ischemia. Clinical manifestations depend on size and location of affected vessel. Most vasculitic syndromes appear to be mediated by immune mechanisms. May be primary or sole manifestation of a disease or secondary to another disease process. Unique vasculitic syndromes can differ greatly with regards to clinical features, disease severity, histology, and treatment.
Granulomatosis with Polyangiitis (Wegener's) 
Granulomatous vasculitis of upper and lower respiratory tracts together with glomerulonephritis; upper airway lesions affecting the nose and sinuses can cause purulent or bloody nasal discharge, mucosal ulceration, septal perforation, and cartilaginous destruction (saddlenose deformity). Lung involvement may be asymptomatic or cause cough, hemoptysis, dyspnea; eye involvement may occur; glomerulonephritis can be rapidly progressive and asymptomatic and can lead to renal failure.
Small-vessel vasculitis that can affect the glomerulus and lungs; medium-sized vessels also may be affected.
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
Granulomatous vasculitis of multiple organ systems, particularly the lung; characterized by asthma, peripheral eosinophilia, eosinophilic tissue infiltration; glomerulonephritis can occur. Heart involvement is the leading cause of death.
Medium-sized muscular arteries involved; frequently associated with arteriographic aneurysms; commonly affects renal arteries, liver, GI tract, peripheral nerves, skin, heart; can be associated with hepatitis B and C.
Inflammation of medium- and large-sized arteries; primarily involves branches of the carotid artery but systemic and large vessel involvement may occur; symptoms include headache, jaw/tongue claudication, scalp tenderness, fever, musculoskeletal symptoms (polymyalgia rheumatica); sudden blindness from involvement of optic vessels is a dreaded complication.
Vasculitis of the large arteries with strong predilection for aortic arch and its branches; most common in young women; presents with inflammatory or ischemic symptoms in arms, legs, head, and neck, systemic inflammatory symptoms, aortic regurgitation.
IgA Vasculitis (Henoch-Schönlein)
Characterized by involvement of skin, GI tract, kidneys; more common in children.
Majority of cases are associated with hepatitis C where an aberrant immune response leads to formation of cryoglobulin; characterized by cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis.
Idiopathic Cutaneous Vasculitis
Cutaneous vasculitis is defined broadly as inflammation of the blood vessels of the dermis; due to underlying disease in >70% of cases with 30% occurring idiopathically and isolated to the skin.
Secondary Vasculitis Syndromes
- Drug-induced vasculitis
- Serum sickness
- Vasculitis associated with infection, malignancy, rheumatic disease
(Fig. 165-1. Algorithm for the Approach to a Pt with Suspected Diagnosis of Vasculitis)
- Thorough history and physical examination: special reference to ischemic manifestations and systemic inflammatory signs/symptoms.
- Laboratories: important in assessing organ involvement: CBC with differential, ESR, renal function tests, UA. Should also be obtained to rule out other diseases: ANA, rheumatoid factor, anti-GBM, hepatitis B/C serologies, HIV.
- Antineutrophil cytoplasmic autoantibodies (ANCA): associated with granulomatosis with polyangiitis, microscopic polyangiitis, and some pts with eosinophilic granulomatosis with polyangiitis; presence of ANCA is adjunctive and should usually not be used in place of biopsy.
- Radiographs: CXR should be performed even in the absence of symptoms in small-vessel vasculitides.
- Diagnosis: can usually be made only by arteriogram or biopsy of affected organ(s).
Guided by organ manifestations. In many instances includes infections and neoplasms, which must be ruled out prior to beginning immunosuppressive therapy. Consideration must also be given for diseases that can mimic vasculitis (Table 165-1 Conditions That Can Mimic Vasculitis).
| TREATMENT | ||
VasculitisTherapy is based on the specific vasculitic syndrome and the severity of its manifestations. Immunosuppressive therapy should be avoided in disease that rarely results in irreversible organ system dysfunction or that usually does not respond to such agents (e.g., isolated cutaneous vasculitis). Antiviral agents play an important role in treating vasculitis occurring with hepatitis B or C. Therapy that combines glucocorticoids with another immunosuppressive agent is particularly important in syndromes with life-threatening organ system involvement, especially active glomerulonephritis. Frequently used agents:
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