Vasculitis

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Home :: Diseases :: Vasculitis
 

Vasculitis - leukocytoclastic, urticarial, allergic vasculitis Symptom

What is Vasculitis

Schemes for classifying vasculitis since those of Zeek and Churg and Strauss , have been devised to incorporate the flourishing knowledge of shared characteristics of vasculitis that are the basis for classification. The major classification systems are those of the American College of Rheumatology (ACR) and the Chapel Hill Consensus Conference (CHCC), which recognized the need for a scheme that would be more useful for diagnosis of specific diseases in individual patients. Although the ACR classification was constructed for the purpose of facilitating research, it was inevitably used for diagnosis, which requires careful investigation of history and symptoms as well as laboratory assessment. The performance of these criteria for diagnosing vasculitis was ascertained in 198 patients: the positive predictive values for Wegener's granulomatosis (WG), giant cell arteritis (GCA), polyarteritis nodosa (PAN), and hypersensitivity vasculitis ranged from only 17% to 29% . Other schemes have fared better for diagnosis of specific types of vasculitis, but their use for this purpose may be hampered by absence of established signs of the disease, particularly early in its course when diagnosis and effective treatment may limit injury.

Features and Symptom of Vasculitis

The characteristics of vasculitis that have been used for classification have included size, type, and distribution of involved vessels; microscopic appearances of vascular and extravascular lesions; clinical and laboratory features; age of the patient; and associations with other disorders. Of these, size of involved vessels has been a major criterion for classifying vasculitis, as it is a major determinant of clinical symptoms ). The value of size as the major criterion, as in the CCHC system, is vitiated by difficulty in acquiring tissue from viscera as well as by the overlapping size of affected vessels. An extreme example of overlapping size was observed in the autopsy of a man with fulminant proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA)-positive vasculitis. There were PAN-like lesions in medium-sized vessels, leukocytoclastic vasculitis in small vessels and capillaries, and inflammation of the endocardium and intima of the aorta. Classification of this syndrome would require more clinical data, but the PR3-ANCA positivity is of more importance in classification than is size of involved vessels

Classification of Vasculitis

Comprehensive classification of vasculitis, based partially on size of predominant vessels

Large vessel Uncommon:
    Giant cell (temporal) Tropical aortitis
        Arteritis Sarcoidosis
    Takayasu's arteritis Cogan's syndrome
Wiskott-Aldrich syndrome
Medium vessel Uncommon:
    Polyarteritis nodosa Lepromatous arteritis
    Familial Mediterranean Fever
    Cutaneous polyarteritis Nodosa
    Kawasaki's disease
Medium to small vessel Uncommon:
    Vasculitis of connective Primary angiitis of the CNS
        tissue diseases Thromboangiitis obliterans
    Behçet's syndrome
    ANCA-associated:
        Wegener's granulomatosis Churg-Strauss syndrome Microscopic polyangiitis
Small vessel Uncommon:
    Cutaneous leukocytoclastic Angiitis Paraneoplastic vasculitis Urticarial vasculitis
    Henoch-Schönlein purpura Degos disease
    Cryoglobulinemic vasculitis Myelodysplastic syndromes
Erythema elevatum diutinum
Hyperimmunoglobulin D

More information on leukocytoclastic, urticarial, allergic vasculitis

Secondary vasculitis may be difficult to classify using conventional criteria [e.g., human immunodeficiency virus (HIV) vasculitis may resemble Kawasaki's disease or leukocytoclastic vasculitis, and the vasculitis of hepatitis C virus (HCV) infection may be similarly pleomorphic. Cutaneous vasculitis, PAN, and cryoglobulinemic vasculitis have all been described with hepatitis B infections. The vasculitis of malignancies, including hairy cell leukemia, is not consistent for a given malignancy: some patients will have PAN and others leukocytoclastic vasculitis.

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