Management of severe ANCA-associated glomerulonephritis: comparison of international recommendations and narrative review

Severe kidney injury in ANCA-associated vasculitis remains a major challenge for healthcare practitioners due to the difficulty in defining the term and the limitations in the evidence base associated with the exclusion of the most severely ill patients from majority of clinical trial protocols. The ACR/VF, EULAR, and KDIGO recommendations intended to answer the questions regarding the management of this patient’s population, but some recommendations on both initial and supportive therapy, provided be the expert panels, differ, which increase the number of treatment options, but also leads to the uncertainty of the expected effect of the treatment. Kidney biopsy plays an important role in identifying patients with a favorable and unfavorable kidney prognosis. Timely histological assessment is necessary not only to confirm the diagnosis, but also to decide whether to intensify therapy if active changes are detected, or, conversely, to discontinue immunosuppressive treatment and switch to renal replacement therapy if chromic changes dominate and the extrarenal manifestations are quiescent. Several scoring systems for assessing kidney damage and risks in the in ANCA-associated vasculitis were proposed during the last decade. Glucocorticoid’s dosing and the choice between cyclophosphamide and rituximab for the initial therapy remains controversial, and the most controversial issue to date remain the indications for the plasma exchange usage. New treatment options for ANCA-associated nephritis, such as the usage of complementblocking therapy are likely to improve the prognosis for the patients with severe renal damage, taking into account the maximum effect on renal function restoration in this particular category.