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

Abstract

ObjectiveTo summarise and update evidence to inform the 2022 update of the European Alliance of Associations of Rheumatology (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).MethodsThree systematic literature reviews (SLR) were performed. PubMed, EMBASE and the Cochrane library were searched from 1 February 2015 to 25 February 2022. The evidence presented herein covers the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) as well as diagnostic testing and general management of all AAV syndromes.ResultsFor the treatment of EGPA, diagnostic procedures and general management 3517, 4137 and 4215 articles were screened and 26, 110 and 63 articles were included in the final evidence syntheses, respectively. For EGPA patients with newly diagnosed disease without unfavourable prognostic factors, azathioprine (AZA) combined with glucocorticoids (GC) is not superior to GC monotherapy to induce remission (LoE 2b). In patients with active EGPA and unfavourable prognostic factors, cyclophosphamide or rituximab can be used for remission induction (LoE 2b). Treatment with Mepolizumab added to standard treatment results in higher rates of sustained remission in patients with relapsing or refractory EGPA without active organ-threatening or life-threatening manifestations (LoE 1b) and reduces GC use. Kidney biopsies have prognostic value in AAV patients with renal involvement (LoE 2a). In the context of suspected AAV, immunoassays for proteinase 3 and myeloperoxidase-ANCA have higher diagnostic accuracy compared with indirect immunofluorescent testing (LoE 1a).ConclusionThis SLR provides current evidence to inform the 2022 update of the EULAR recommendations for the management of AAV.

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

“…Supplementary studies from the ‘General Management’ section in the second SLR manuscript report dose-dependent increase of malignancy risk and early menopause under CYC treatment that need to be considered when choosing agents for induction treatment. 18 …”

“… 17 A second complementary article will cover the treatment of eosinophilic granulomatosis with polyangiitis as well as diagnostic procedures and general management of AAV. 18 …”

The Evaluation of Changing the Eponym Churg–Strauss Syndrome Due to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

“…However, organ damage, relapses, and exacerbations can disrupt patients’ quality of life [ 16 ]. Glucocorticoids, azathioprine, cyclophosphamide, rituximab, and mepolizumab are among the treatment regimens [ 17 ]. The American College of Rheumatology developed classification criteria to include biopsies showing asthma, eosinophilia, neuropathy, unstable pulmonary infiltrate, paranasal sinus abnormality, and extravascular eosinophils [ 18 ].…”

Systemic vasculitis involving the kidney: the nephropathologist’s point of view

“…In particular, Berden et al proposed a four-tiered classification based on the prevalence of glomeruli with no lesions, sclerosis, crescents or mixed lesions (focal, sclerotic, crescentic, and mixed, respectively) 22 . Although subsequent meta-analyses confirmed the prognostic role of this classification, further efforts have been made to improve these classification systems 29 . Among them, the recently proposed Renal Risk Score by Brix et al merged clinical and histological information to obtain a reliable stratification of patients’ prognosis 30 .…”