Granulomatosis with polyangiitis: news in diagnosis and treatment

Czech version

Authors: E. Lokočová; P. Horák; M. Skácelová
Authors‘ workplace: III. interní klinika NRE LF UP a FN, Olomouc
Published in: Čes. Revmatol., 27, 2019, No. 3, p. 126-135.
Category: Review Article

Overview

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis which belongs to ANCA associated vasculitides. It is characterized by the granulomas, most commonly affecting the nasal cavities, upper respiratory tract, lungs and kidneys. 82–94% of pacients with GPA are ANCA positive (antineutrophil cytoplasmic antibodies), especially type c-ANCA (specifically proteinase 3), in 20% p-ANCA (anti myeloperoxidase antibody) and ANCA negative in 10%. In edition to basic imaging we use as a standard lung HRCT (high resolution computed tomography) and brain and paranasal sinuses MRI (magnetic resonance imaging). Renal biopsy is important for diagnoses and assessing the severity of the disease. The Birmingham Vasculitis Activity Score (BVAS) is used to assess disease activity, both for initial diagnosis and for subsequent monitoring of treatment effect and relapse. Inicial therapy of GPA consists of high-dose glucocorticoids and cyclophosphamide or rituximab. Recently, a comparable effect has been demonstrated for mycophenolate mofetil. Plasma exchange is used for patients with rapidly progressing renal or pulmonary failure with pulmonary haemorrhage. Maintenance therapy of GPA consists of azathioprine, rituximab, methotrexate, mycophenolate mofetil, alternatively may be used leflunomide. Avacopan – C5a receptor inhibitor is new effective drug in replacing high-dose glucocorticoids.

Keywords:

azathioprine – rituximab – granulomatosis with polyangiitis – ANCA-associated vasculitis – Birmingham Vasculitis Activity Score – Cyclophosphamide – mycophenolate mofetil – Methotrexate – avacopan

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Odborné události

Article was published in

Czech Rheumatology

2019 Issue 3