Granulomatosis with polyangiitis

updates from diagnosis to treatment

Abstract

Introduction: Granulomatosis with polyangiitis (GPA) is a pauci-immune necrotizing granulomatous vasculitis that affects predominantly small vessels. It is a rare disease with a multifactorial etiology and an important differential diagnosis of Pulmonary-renal syndromes. Objective: to update the main topics about granulomatosis with polyangiitis, in addition to bringing future perspectives and advances in the clinical management of this vasculitis. Methodology: literature review conducted in 2018 from the publications of the last 5 years in PubMed Central® and SciELO® databases. Results: 29 selected bibliographical references, of which: 16 literature reviews; 2 systematic reviews; 1 cross-sectional study; 2 case-control studies; 6 cohorts; 2 updated consensuses (1 Brazilian, 2017 and 1 international, 2017). Conclusion: granulomatosis with polyangiitis is an entity with high morbidity and mortality, and its treatment algorithm has recently been updated, as discussed in this review. Further studies are underway to evaluate the efficacy and safety of novel therapeutic approaches.