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ANCA-associated vasculitis, granulomatosis with polyangiitis, clinical features, prognosis, immunobiological therapy, rituximab

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The article highlights the new views on classification and nomenclature of systemic vasculitis, the meaning of detection of antineutrophil cytoplasmic antibodies (ANCA) in the diagnosis and prognosis of the disease. The literature data and own research regarding clinical manifestations of ANCA-associated vasculitis are presented.  Analyzing the first clinical manifestations in 41 patients with granulomatosis with polyangiitis, we identified four variants of the debut: with involvement of ENT organs (n ​=21), with lung lesions without involvement of the upper respiratory tract (n=8), with skin lesions, joint involvement and fever (n=7) and other variants (n=5). Fever (76%), involvement of ENT organs (51%), skin (41%), nervous system (39%) and arthritis/arthralgia (37%) were the most frequent first manifestations of granulomatosis with polyangiitis. In comparison with the presenting features there were more often lesions of the skin (66% vs. 41%), nervous system (51% vs. 39%), kidneys (41% vs. 10%), lungs (63% vs. 30%), eyes (32% vs. 10%) and myalgia (34% vs. 12%) throughout course of disease.  Among the lesions of the ENT organs, sinusitis (n=19), rhinitis (n=8) and otitis (n=6) were predominate, laryngotracheitis, sublottic stenosis, saddle-shaped deformation of the nose, destruction of the walls of the paranasal sinuses and mastoiditis were diagnosed less frequently. The latest clinical recommendations for treating patients with ANCA-associated vasculitis, including the use of immunobiological therapy are presented, as well as describing of the results of our own experience in the using of immunobiological therapy in patients with granulomatosis with polyangiitis.
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