2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (Part 4)
This week, we are continuing with the recommendations formulated by the American College of Rheumatology (ACR) for the management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (ANCA-AAV).
Let me remind our readers that ANCA-AAV is a group of diseases that includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
The use of cyclophosphamide is feasible when rituximab needs to be avoided or when patients have active disease despite receiving rituximab treatment. It remains to be seen whether cyclophosphamide should be preferred for certain types of severe disease, such as acute renal failure with serum creatinine >4.0 mg/dl). The recommendation says that either intravenous (IV) pulse or daily oral cyclophosphamide are feasible.
For adults, the choice the treatment options should be based on patient and physician decision.
In children, IV cyclophosphamide may be chosen to ensure that toxicity levels are monitored well.
Data regarding the efficacy of combined cyclophosphamide and rituximab use remain limited. Toxicity issues remain a huge concern.
Combining rituximab with cyclophosphamide is not widely used in the United States, as the said combination’s efficacy compared to the use of the said drugs in a monotherapy settingis not established.
The use of the combination of rituximab with cyclophosphamide remains under clinical trails up to the present.
The recommendation for patients with GPA/MPA with active glomerulonephritis, the ACR, with certain conditions, recommend against the routine addition of plasma exchange to remission
induction therapy.
Plasma exchange should not be used in all patients with active glomerulonephritis but can be considered for patients who are at risk of developingend-stage renal disease (ESRD) and those who understand the potential heightened risk of infection.
This recommendation is based on data from the 2 largest trials of plasma exchange for the treatment of glomerulonephritis in AAV.
The ACR panel does not recommend plasma exchange for all patients with active glomerulonephritis but favors consideration of the treatment for patients at a higher risk of progression to end stage renal disease.
Plasma exchange may be used in patients with GPA or MPA who also have anti–glomerular basement membrane disease.
We will continue with another set of recommendations as we discuss this topic in our next column.