Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches

摘要: Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. The predominantly affects small-calibre blood vessels and associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). was considered to be a entity Savage et al. in 1985. has reported low incidence slight male predominance. aetiology MPA remains unknown. There is, however, increased evidence that which ANCAs, particularly those reacting MPO, are pathogenic. belongs vasculitides, indicating multiple organs can affected. major involved kidneys lungs. As expected for illness organ systems, patients present myriad different symptoms. Ear, nose throat (ENT) manifestations not clinical symptoms MPA, but majority populations described, ENT involvement found surprisingly high percentages. part ANCA-associated necrotizing vasculitis small vessels. Diagnosis mainly established manifestations, computed tomography (TC), ANCA antibody detection renal pulmonary biopsy. introduction aggressive immunosuppressive treatment substantially improved prognosis. standardized therapeutic regimen based on cyclophosphamide corticosteroids. Using this regimen, remission achieved most patients. Rituximab may represent important alternative who respond adequately antimetabolite therapies.