Efficacy of plasma exchange for antineutrophil cytoplasmic antibody-associated systemic vasculitis: a systematic review and meta-analysis

Abstract

Objective To assess through systematic review and meta-analysis whether plasma exchange (PE) is associated with prognosis in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. Methods A systematic search of PubMed, MEDLINE, Embase, and CENTRAL databases from inception to 17 June 2020 was conducted. Ongoing or unpublished trials were also searched in ClinicalTrials.gov and the World Health Organization trials portal. Randomised controlled trials (RCTs) comparing PE vs. non-PE in AAV patients (microscopic polyangiitis [MPA], granulomatosis with polyangiitis [GPA], or eosinophilic granulomatosis with polyangiitis [EGPA]) were included. The combined risk ratio (RR) was calculated by the random-effects model using the Mantel-Haenszel method. Heterogeneity was measured using the I2 statistic. Primary outcomes were mortality, clinical remission (CR), and adverse events (AEs). Results Four RCTs comparing PE vs. no PE (N = 827) and 1 RCT comparing PE vs. pulse steroid treatment (N = 137) were included. All participants were MPA or GPA patients (no EGPA patients). PE was not associated with main primary outcomes compared with no PE (mortality RR 0.93 [95% confidence interval {CI} 0.70–1.24], I2 = 0%; CR RR 1.02 [95% CI 0.91–1.15], I2 = 0%; and AE RR 1.10 [95% CI 0.73–1.68], I2 = 37%) or pulse steroid (mortality RR 0.99 [95% CI 0.71–1.37]; CR [the Birmingham Vasculitis Activity score] mean difference − 0.53 [95% CI − 1.40–0.34]; and AE RR 1.05 [95% CI 0.74–1.48]). Focusing on the early treatment phases, PE was associated with a reduction in end-stage renal disease incidence compared with both no PE (PE 1/43 vs. no PE 10/41; RR 0.14 [0.03–0.77] at 3 months) and pulse steroid (PE 11/70 vs. pulse steroid 23/67; RR 0.46 [0.24–0.86] at 3 months). Conclusion We carried out a systematic review and meta-analysis targeting all AAV patients, including MPA, GPA, and EGPA. In AAV patients, performing PE was not associated with the risk of mortality, CR, and AE. No RCT exists evaluating the efficacy of PE for EGPA; hence, this is required in the future. The results may affect the development of guidelines for AAV and may indicate the direction of future clinical research on AAV. Trial registration UMIN R000045239, PROSPERO CRD42020182566.

Time-dependent risk of mortality and end-stage kidney disease among patients with granulomatosis with polyangiitis

“…Despite treatment advances that have been made for decades, GPA is still associated with a high mortality rate ( 8 , 17 ). Earlier studies revealed a considerable variation, with the majority coming from European countries, which assessed the long-term survival rate of GPA and reported a survival rate of from 85 to 97% at 1 year, 69 to 91% at 5 years, and 75 to 88% at 10 years ( 18 ).…”

Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): part 1—treatment of granulomatosis with polyangiitis and microscopic polyangiitis

“… 96 A lower risk of dialysis dependence at 1 year was also reported in a Cochrane Review by Walters et al that did not include PEXIVAS (total six RCTs). Three meta-analyses that included a low number of subjects 100 or included non-randomised observational studies 27 101 found that PLEX did not reduce the overall risk of ESKD at 1 year. Two meta-analyses found that benefit of PLEX may be highest in patients at high risk of ESKD.…”

A glance into the future of anti-neutrophil cytoplasmic antibody-associated vasculitis

“…154 However, in patients with severe kidney disease (SCr > 5.7 mg/dL) studies have reported conflicting results regarding the role of PLEX and international community is divi ded. 14,[169][170][171][172] These controversies are reflected in the most recent guidelines, with KDIGO considering PLEX in patients with SCr > 5.7 mg/dL, rapidly increasing SCr or who require dialysis, and in patients with DAH who have hypoxemia, while ACR/VF recommend against its routine use, but may consider PLEX in critically ill patients at high risk for progression to KF. 16,153 IV immunoglobulin may also be a therapeutic option as adjunctive therapy for patients with refractory or relapsing AAV.…”