Thus, halting progression of damage due to active vasculitis and thereby achieving kidney function recovery is a key therapeutic goal. ![]() It is well recognized that kidney function impairment at the first presentation is associated with a transition from acute kidney injury to chronic kidney disease (CKD). Still, a subset of predominantly MPO-ANCA-positive patients may follow a slowly progressive course of kidney function deterioration, frequently associated with irreversible kidney lesions at their first presentation. Typically, AAV patients present with the clinical picture of rapidly progressive glomerulonephritis and face a rapid decline in kidney function, necessitating immediate therapy initiation to preserve kidney function and avoid the immediate threat of ESKD. In contrast, EGPA patients have a substantially lower rate of kidney involvement (≈25%) however, differences according to ANCA positivity (predominantly MPO-ANCA) can be observed. Patients with systemic forms of MPA and GPA, characterized by multi-organ involvement, present with MPO- and PR3-ANCA and with kidney involvement in approximately 90–100% and 50–80%, respectively. Despite refinement of approaches to induce and maintain remission in recent years, the rate of end-stage kidney disease (ESKD) remains high. The frequency and severity of kidney involvement differ according to the clinical phenotype and ANCA serology. Although cardiovascular disease and infections are important contributors to premature death, the initial presence and the severity of kidney dysfunction are the most important predictors of mortality. There is a 2.7-fold increased all-cause mortality risk among AAV patients compared with the general population. AAV includes three clinically distinct groups: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In this review we discuss important points missed in the assessment of kidney function in patients with AAV and point towards the importance of defining renal response and clinically important short- and long-term predictors of renal outcome.ĪNCA vasculitis, ESKD, kidney function, outcome, renal response THE SCOPE OF THE PROBLEM: SEVERE KIDNEY DISEASE IMPACTS ON PROGNOSISĪnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare systemic autoimmune disease affecting small to medium-sized vessels and is characterized by autoantibodies against the major target of the neutrophil proteins, leucocyte proteinase 3 (PR3) and myeloperoxidase (MPO). The timing is favourable since large datasets of trials conducted in AAV are available and provide a valuable resource to establish renal surrogate markers and, likely, aim to investigate optimized and tailored treatment approaches according to a renal response score. Refinement of these markers and the incorporation of novel approaches such as the assessment of histopathological changes using cutting-edge molecular and machine learning mechanisms or new biomarkers could significantly improve prognostication. serum creatinine, glomerular filtration rate, proteinuria, hematuria and disease activity scores) has limitations. Its assessment through traditional surrogates (i.e. Differences in clinical features and histopathologic and therapeutic approaches will influence the course of kidney function. Although renal response is a crucial measure to capture clinically relevant changes, clinal trials have used various definitions and no well-studied key surrogate markers to predict renal outcome in AAV exist. Avoiding end-stage kidney disease in patients with anti-neutrophil cytoplasmic antibody–associated vasculitis (AAV) has a high therapeutic priority.
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