Granulomatosis with polyangiitis: An atypical initial presentation

Abstract

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small and medium vessels with involvement of the upper and lower respiratory tract and necrotizing pauci-immune glomerulonephritis [1]. This vasculitis has a higher incidence in men in the sixth decade of life and more than 80% of patients have positive anti-neutrophil cytoplasm (ANCA) antibodies [1,2].We present the case of a 23-year-old man with two weeks of evolution with polyarthralgia, asthenia, and cough with hemoptoic sputum. He did a chest radiography that showed diffuse bilateral alveolar infiltrates, on the second stage. The patient presented a rapid clinical worsening, with moderate hemoptysis and severe respiratory failure requiring invasive mechanical ventilation.The autoimmune study revealed positivity for ANCA PR3 in titer >200, having started pulses of methylprednisolone, plasmapheresis and later cyclophosphamide, with clinical improvement. His high-resolution chest computed tomography (CT) showed areas of diffuse ground glass densification suggesting capillaritis/alveolar hemorrhage and two subpleural nodular areas suggestive of granulomatous vasculitis. CT of the nasal sinuses showing findings compatible with acute inflammatory changes, with histology of the nasal mucosa inconclusive. Thus, this case shows an exuberant and potentially fatal form of diffuse alveolar hemorrhage that culminated in the initial diagnosis of granulomatous vasculitis in a young adult.

Keywords

• Granulomatosis with polyangiitis
• ANCA
• Vasculitis