Publication date: Available online 27 September 2018
Source: Journal of Allergy and Clinical Immunology
Author(s): Pavel Kolkhir, Maria Grakhova, Hanna Bonnekoh, Karoline Krause, Marcus Maurer
Abstract
Urticarial vasculitis (UV) is a difficult-to-treat condition characterized by long-lasting urticarial rashes and histopathologic findings of leukocytoclastic vasculitis. Treatment is dictated by severity of skin and systemic involvement and the underlying systemic disease. This is a comprehensive systematic review of the efficacy of current UV treatment options. We searched for relevant studies in seven databases including MEDLINE, Scopus, and Web of Science. In total, 261 eligible studies and 789 unique UV patients were included in the systematic review. Most UV patients are adult females with chronic (≥6 weeks) and systemic disease. UV is mostly idiopathic, but can be associated with drugs, malignancy, autoimmunity and infections. It usually resolves with their withdrawal or cure. Corticosteroids are effective for the treatment of skin symptoms in >80% of UV patients. However, their long-term administration can lead to potentially serious adverse effects. The addition of immunomodulatory or immunosuppressive agents often allows corticosteroids tapering and improves the efficacy of therapy. Biologicals, including omalizumab, as well as corticosteroids, cyclophosphamide, dapsone, mycophenolate mofetil, plasmapheresis, colchicine, hydroxychloroquine, intravenous immunoglobulins, NSAIDs, and cyclosporine can be effective for skin as well as systemic symptoms in UV patients. H1-antihistamines, montelukast, danazol, H2-antihistamines, pentoxifylline, doxepin and tranexamic acid are not effective in most UV patients. As of yet, no drugs have been approved for UV, and management recommendations are based mostly on case reports and retrospective studies. Prospective studies investigating the effects of treatment on the signs and symptoms of UV are needed.
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