Granulomatosis with polyangiitis with varying clinical manifestations and response. Report of two cases

Granulomatosis con poliangeitis con manifestaciones y respuesta clínica diferentes. Reporte de dos casos

Polyangiitis with granulomatosis, previously known as Wegener's granulomatosis, is a necrotizing vasculitis of small and medium-sized vessels characterized by the formation of necrotizing granulomas that primarily affect the upper respiratory tract, lungs, and kidneys. Clinically, it presents with symptoms such as chronic sinusitis, hemoptysis, dyspnea, rapidly progressive glomerulonephritis, and systemic manifestations such as fever, weight loss, and asthenia. Diagnosis is based on detecting anti-neutrophil cytoplasmic antibodies (ANCA) type PR3 and histopathological studies. Treatment includes immunosuppressants, such as corticosteroids and cyclophosphamide or rituximab, to induce remission.

Two clinical cases with diagnoses of polyangiitis with granulomatosis are reported. One was a 58-year-old man with orbital involvement, saddle nose, recurrent epistaxis, chronic rhinosinusitis, and bilateral pulmonary cavitary involvement. A second case is a 44-year-old man with chronic rhinosinusitis, recurrent epistaxis, hearing loss, and bilateral tracheobronchial stenosis and chondritis. Both cases have been positive for C-ANCA; the first involved a lung biopsy that revealed necrotizing vasculitis, and the second involved a positive skin biopsy for necrotizing vasculitis. In both cases, induction treatment started with an excellent response and is currently being maintained.