Hypocomplementemic urticarial vasculitis syndrome — clinical presentation, diagnosis and treatment
Abstract
Hypocomplementemic urticarial vasculitis syndrome (HVUS) belongs to the group of systemic small-vessel vasculitis and is characterized by persistent urticarial lesions lasting more than 24 hours, hypocomplementemia and frequent coexistence of organ involvement. Lungs, kidneys, joints, gastrointestinal tract, eyes and central nervous system may be involved in the course of the condition. The diagnosis of HUVS is based on the diagnostic criteria – fulfilment of both major criteria (the presence of chronic urticarial lesions with hypocomplementemia) and at least two minor criteria. HUVS may be associated with other systemic conditions. The diagnosis of the syndrome may also precede the development of systemic lupus erythematosus (SLE) in both the paediatric and adult populations. Due to the rarity of the disease, no randomized clinical trials are assessing the effectiveness of individual therapeutic options, and recommendations for the management of HUVS are based on case reports. Glucocorticosteroids (GCSs) are most commonly used as monotherapy or in combination with cyclosporine A, methotrexate, azathioprine, cyclophosphamide or mycophenolate mofetil. In the paper, the clinical picture, recommended diagnostics and therapeutic options for HUVS are discussed.
Keywords: urticarial vasculitissmall-vessel vasculitisHUVShypocomplementemiaanti-C1q
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