Vasculitis asociadas a los anticuerpos anticitoplasma de neutrófilos (ANCA): de la A a la Z

Abstract

Antineutrophil cytoplasmic antibodies associated vasculitis include microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. The pathogenicity of ANCA involves neutrophil activation, NET generation, and complement activation. A common feature of ANCA-associated vasculitides is the presence of necrotizing inflammation and fibrinoid necrosis in the walls of small to medium-sized blood vessels.

Microscopic polyangiitis primarily affects small blood vessels and is associated with glomerulonephritis. Granulomatosis with polyangiitis involves necrotizing granulomas, affecting the upper and lower respiratory tract, kidneys, and skin, often presenting with purpura. Eosinophilic granulomatosis with polyangiitis, predominantly eosinophilic, affects multiple organs, including the respiratory, gastrointestinal, renal, and cutaneous systems, featuring palpable purpura and characteristic ulcers.

Multidisciplinary treatment involves glucocorticoids, immunosuppressants, and monoclonal antibodies. Treatment duration depends on the disease phase, with aggressive therapy for active phases and maintenance therapy for remission. Close monitoring is essential. In the diagnosis and treatment of these vasculitis, collaboration between specialties, including dermatology, is crucial for a comprehensive approach and improved clinical outcomes.