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Postinfectious vasculitis
Vasculitis, characterized by inflammatory lesions in the wall of vessels, may be due to different viruses.
ORPHA:48435
Classification level: Disorder
Prevalence: Unknown
Inheritance: Not applicable
Age of onset: All ages
CMV induces vasculitis mainly in immunocompromised patients. Lesions may be cerebral, retinal, colonic, radicular... In less common cases, vasculitis affects immunocompetent patients, thus resembling polyarteritis nodosa (PAN) or cutaneous angiitis. Varicella-Zoster virus (VZV) is responsible for two types of vasculitides which are sometimes associated: · a vasculitis affecting large arteries which usually affects the anterior cerebral circulation and is characterized by an acute focal deficit. Most often it occurs in immunocompetent adults after zoster of trigeminal distribution. · a vasculitis affecting small cerebral vessels occurring in immunocompromised individuals and is responsible for multifocal deficit. VZV vasculitis can appear a few months after rash or even without any rash. HIV is responsible for a protean group of vasculitides. Vessels of all sizes may be affected and histological findings are variable. Nevertheless a particular form consists in a necrotizing vasculitis of the aorta and large arteries (resembling Takayasu's arteritis) with aneurysm formation and risk of rupture. Parvovirus B19 has been incriminated in some cases of PAN, cutaneous and placenta angiitis or pseudo Henoch-Schönlein purpura. Other case reports mention vasculitis related to hepatitis A virus, HTLV1 (Human T-cell Leukemia Virus 1), EBV (Epstein-Barr virus) persistent infection, Hantaan virus.
Pathophysiology is partially misunderstood. However, mechanisms including direct injury by the virus or vascular damage resulting from immune reaction are incriminated. For instance, endothelial infection by CMV (cytomegalovirus) seems to play a major role in the occurrence of a vasculitis. Immune complexes, associating an immunoglobulin and a viral antigen, can lead to vasculitis because of the activation of complement and inflammatory cells. Few virus are thought to be responsible for vasculitis even though their triggering role is not always easily confirmed.
Treatment is based on antiviral agents. Corticotherapy and immunosuppressants are very controversial because of the risk of virus replication. A least, for some authors, adjuvant treatment with immunoglobulins or plasma exchange may be helpful.
Last update: September 2003
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