336. Plasma exchange and glucocorticoid dosing for patients with ANCA-associated vasculitis: a clinical practice guideline
Creators: name: Walsh1, Michael
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background/Objective:</strong> To provide guidance on the role of plasma exchange and the optimal dose of glucocorticoids during remission induction in ANCA-associated vasculitis (AAV) following the publication of the PEXIVAS trial. <strong>Methods:</strong> A guideline panel including patients, caregiver, clinicians, content experts, and methodologists produced these recommendations using GRADE and in adherence with standards for trustworthy guidelines. The recommendations are based on systematic reviews. The panel took an individual patient perspective in the development of recommendations. <strong>Results:</strong> From the systematic review, plasma exchange had little or no effect on mortality or disease relapse. However, plasma exchange probably reduces the one-year risk of ESKD (graded absolute risk reduction from ~0.4% in those with low risk to ~16.8% in those with high risk) but increases the risk of serious infections (graded from ~2.7% in those with low risk to ~13.5% in those at high risk) at 1 year (moderate to high certainty). The panel agreed the majority (50-90%) of patients with low or low-moderate risk of developing ESKD would consider the har outweigh the benefits, while the majority (50-90%) of those with moderate-high or high risk would consider the benefits outweigh the har For patients with pulmonary hemorrhage without significant kidney involvement, based on indirect evidence, plasma exchange has little or no effect on death (very low certainty) but may have an important increase in serious infections at 1 year (low certainty). The systematic review of different dose regimens of glucocorticoids identified one RCT that enrolled 704 patients. A reduced-dose regimen of glucocorticoid probably reduces the one-year risk of serious infections by ~5.9 % and probably does not increase the risk of ESKD at the follow-up of longer than 1 year (moderate certainty for both outcomes). Based on these data, the panel made a weak recommendation against plasma exchange in patients with low or low-moderate risk of developing end-stage kidney disease (ESKD) (approximate probability ≤ 10% at 1 year), and a weak recommendation in favour of plasma exchange in patients with moderate-high or high risk of developing ESKD (approximate probability &gt; 10% at 1 year). For patients with pulmonary hemorrhage without impaired kidney function, the panel suggests not using plasma exchange (weak recommendation). The panel made a strong recommendation in favour of a reduced rather than standard-dose regimen of glucocorticoids, which involves a more rapid taper rate and lower cumulative dose during the first 6 months of therapy. <strong>Conclusions:</strong> The panel concluded that the majority (50-90%) of fully informed patients with AAV and with low or low-moderate risk of developing ESKD would decline plasma exchange while the majority of patients with moderate-high or high risk would choose to receive plasma exchange. The panel also inferred that the majority of fully informed patients with pulmonary hemorrhage without significant kidney involvement would decline plasma exchange and that all or almost all (at or over 90%) fully informed patients with AAV would choose a reduced-dose regimen of glucocorticoids during the first 6 months of therapy. <strong>Disclosures:</strong> None