Histopathological prognostic factors in ANCA-associated glomerulonephritis

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a group of multisystemic autoimmune diseases characterized by necrotizing inflammation of small vessels. Kidney involvement is frequent in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and accounts for a significant proportion of the morbidity and mortality related to these diseases. Despite improvement in therapeutic management of ANCA-glomerulonephritis (ANCA-GN), end-stage kidney disease (ESKD) still occurs in up to 30% of affected patients within 5 years following diagnosis. Thus, identifying patients for whom aggressive immunosuppressive therapy will be more beneficial than deleterious is of great importance. Several clinical, biological and histological factors have been proposed as predictors of ESKD. The kidney biopsy is essential not only for the diagnosis, but also for evaluating renal prognosis. In this review, we discuss the prognostic value of renal lesions at the diagnosis of ANCA-GN by analyzing each compartment of the nephron. We also review existing ESKD risk classification in ANCA-GN and finally propose an example of a standardized pathology report that could be used in routine practice.

Keywords: ANCA renal risk score; ANCA-associated vasculitis; End-stage kidney disease; Glomerulonephritis; Histopathological classification; Mayo Clinic chronicity score; Prognostic factors.