Vasculitis & polymyalgia rheumatica

Systematic literature review informing the 2022 update of the EULAR recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV): Part 2 - Treatment of eosinophilic granulomatosis with polyangiitis and diagnosis and general management of AAV

RMD OpenPublished Systematic literature review

Authors' conclusion

This SLR provides current evidence to inform the 2022 update of the EULAR recommendations for the management of AAV.

Study context

Population
Studies on treatment of eosinophilic granulomatosis with polyangiitis (EGPA) and on diagnostic testing and general management of all AAV syndromes26, 110 and 63 articles included
Methodological detailsthree systematic literature reviews, PubMed, EMBASE and Cochrane library searched 1 February 2015 to 25 February 2022
  • Three systematic literature reviews
  • PubMed, EMBASE and Cochrane library searched 1 February 2015 to 25 February 2022

Main finding

As reported in the abstract

  • Newly diagnosed EGPA without unfavourable prognostic factors: AZA plus glucocorticoids (GC) not superior to GC monotherapy to induce remission (LoE 2b)
  • Active EGPA with unfavourable prognostic factors: cyclophosphamide or RTX can be used for remission induction (LoE 2b)
  • Relapsing or refractory EGPA without active organ- or life-threatening manifestations: mepolizumab added to standard treatment gives higher sustained remission rates (LoE 1b) and reduces GC use
  • Kidney biopsies have prognostic value in AAV with renal involvement (LoE 2a)
  • Suspected AAV: PR3- and MPO-ANCA immunoassays have higher diagnostic accuracy than indirect immunofluorescent testing (LoE 1a)

Original publication

Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): Part 2 - Treatment of eosinophilic granulomatosis with polyangiitis and diagnosis and general management of AAV. RMD Open. Published 22 June 2023. doi:10.1136/rmdopen-2023-003083

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