An update on dıagnosıs and treatment of ANCA assocıated renal vasculıtıs
Kultigin Turkmen1, Hakan Ozer2, Vladimir Tesar3
1Meram Medical Faculty, Department of Internal Medicine, Division of Nephrology, Necmettin Erbakan University, Konya, Turkey.
Abstract:
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of diseases characterised by necrotizing inflammation of small vessels such as arterioles, venules, and capillaries. ANCA-associated vasculitides (AAV) are referred to as small vessel vasculitides. Three AAV subgroups, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), are defined according to clinical features. The most common disease with renal involvement in AAV is MPA Approximately 90% of patients with MPA have renal involvement. While this rate is 70-80% in GPA, less than half of EGPA patients have renal involvement. Untreated survival in AAVs is less than one year. With appropriate immunosuppressive therapy, the 5-year renal survival rate is 70-75%. Without therapy, the prognosis is poor but treatments, typically immunosuppressants, have improved survival, albeit with considerable morbidity from glucocorticoids and other immunosuppressive medications. Current challenges include improving the measures of disease activity and risk of relapse, uncertainty about optimal therapy duration and a need for targeted therapies with fewer adverse effects. In this review, we described the treatment of renal involvement in AAV in line with current studies.
Related Concept Videos
Imaging Studies VII: Vascular Imaging
Acute Kidney Injury IV: Diagnostic Studies and Prevention
Chronic Kidney Disease III: Interprofessional Care
Nephrotic Syndrome II : Assessment and Medical Management
Acute Pyelonephritis II: Diagnostic Studies and Management
Aneurysm III: Interprofessional Care


