Severe therapy-resistant necrotizing vasculitis associated with hepatitis C virus infection: successful treatment of the vasculitis with extracorporeal immunoadsorption.
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Conditions that mask or mimic vascular inflammation are many, the paths to diagnostic distinction are varied, and the prognostic and therapeutic consequences of misreading the signposts are profound. Six cases illustrate.
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The systemic necrotizing vasculitides represent a difficult group of diseases from a therapeutic perspective. Even with successful therapy patients often experience a high degree of disease and treatment morbidity. Refractory disease, or that which fails to respond to standard therapy, is particularly challenging. A clinical approach to several vasculitic syndromes is outlined in this article with emphasis given to therapies supported by controlled investigations and those supported by consensus opinion where available.
The clinical severity of ANCA-associated vasculitides (AAV) ranges widely from relatively benign courses to rapidly progressive, acutely life-threatening disease. The aggressiveness in the individual patient depends on the site and size of vessels involved and on the severity of the associated inflammatory/immunological features. The scope of life-threatening lesions reaches from the well-known pulmonary renal syndrome to cardiac, gastrointestinal and central nervous system manifestations. Treatment must be instituted as soon as possible and consists of daily cyclophosphamide and high-dose glucocorticosteroids. Plasmapheresis, intravenous immunoglobulin, and monoclonal antibodies may be of benefit in some cases.
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