Resumo

HOYOS PATINO, Simón. Granulomatosis with polyangiitis: update and key concepts. Rev Cuba Reumatol [online]. 2016, vol.18, n.1, pp. 36-44. ISSN 1817-5996.

Granulomatosis with polyangiitis is a multisystem disease that is part of the small vessel vasculitis associated with ANCA (antibodies against the cytoplasm of neutrophils), along with eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis and ANCA associated vasculitis limited to an organ. This condition is characterized by a necrotizing inflammatory vasculitis with granuloma formation. Mainly affects the respiratory tract and the renal system, with a higher incidence between 40-55 years. It is more prevalent in caucasians and in northern Europe. Its etiology is unknown, but in its occurrence different aspects environmental, infectious,   pharmacological, immunological, and toxic in addition to a genetic predisposition are involved. The aim of this text is to review key aspects of the disease based on new developments in literature, including aspects such as epidemiology, pathophysiology, clinical manifestations, diagnosis and treatment. With special emphasis on the pathophysiology and clinical manifestations, which are fundamental to the development of new therapies for disease management, along with early diagnosis to minimize complications arising from this.

Palavras-chave : Granulomatosis with polyangiitis; vasculitis; ANCA; NETs.

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