Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

Abstract

Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a destructive small vessel vasculitis affecting multiple organs. Renal involvement often leads to end-stage renal disease and increases mortality. Prompt diagnosis and initiation of adequate immunosuppressive therapy are critical for the best patient and kidney outcomes. However, considerable heterogeneity in symptoms and severity across the patients frequently hinder the diagnosis and management. The objective of this review is to emphasize the heterogeneity of the ANCA-associated vasculitis, facilitate the recognition and give guidance to the therapeutical possibilities. We present epidemiologic and risk factors, pathogenesis, and provide comprehensive clinical features of the disease. This article also focuses on the currently available therapeutic options and emerging cellular and molecular targets for the management of systemic and especially renal disease. We conducted extensive literature research published on PubMed and Google Scholar. We systematically reviewed, analyzed, and assembled databases, covering a broad spectrum of aspects of the disease. We compared and summarized the recommendations of two recent guidelines on ANCA-associated vasculitis. The incidence of ANCA-associated vasculitis, hence glomerulonephritis shows a steady increase. Familiarity with the presenting symptoms and laboratory abnormalities are necessary for rapid diagnosis. Early initiation of treatment is the key aspect for favorable patient and renal outcomes. A better understanding of the pathogenesis constantly leads to more targeted and therefore more efficient and less toxic treatment.

Glomerular crescents, IgA-deposits, ANCA, infection – unravelling the diagnostic conundrum

“…But up to 10 patients were managed only with supportive measures. Management for ANCA-GN included treatment with corticosteroids, and cyclophosphamide (or Rituximab) with or without plasmapheresis 22, 23 .…”

“…Based on the pathology reports, the cases grouped as follows: Group 1-IgAN, Group 2 -SAGN/IRGN (only included cases with culture-proven infection around the time of GN), Group 3 -ANCA-GN, and Group X -could not be definitively differentiated into any of the three groups based on the biopsy findings and clinical information available to the pathologist at the time of biopsy evaluation. The diagnostic criteria for IgAN, SAGN/IRGN, and ANCA-GN included a combination of clinical, laboratory, and pathologic features as described in reference texts 6,7,[22][23][24][25][26] .…”

ANCA Glomerulonephritis after COVID-19 and post-COVID-19 vaccination – current state of knowledge

“…Resistant hypertension is also common. There is a reduction in glomerular filtration and a decrease in GFR, even within a few days [3,17,19]. Changes progress at a rapid pace, leading to progression to end-stage renal failure if the disease is not recognized and treated early.…”

“…Renal biopsy typically reveals focal and segmental glomerulonephritis, as well as diffuse necrotizing and crescentic glomerulonephritis. The most crucial approach is rapid diagnosis and initiation of appropriate immunosuppressive treatment [2,3].…”

Evaluation of a renal risk score for Japanese patients with ANCA-associated glomerulonephritis in a multi-center cohort study

“…Renal involvement is also associated with a worse prognosis than AAV patients without impaired renal function (4,6,7). Clinicians need to perform prompt diagnosis and initiation of adequate immunosuppressive therapy to preserve patient and kidney outcomes, but avoidance of adverse events such as treatmentrelated complications, infection, cardiovascular diseases, and cancer is also a priority (8). Therefore, careful selection of patients who would benefit from intensive immunosuppressive therapy is highly required.…”