Systematic review and meta-analysis for 2023 clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis

Abstract

Objectives To provide evidence for the revision of clinical practice guidelines for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis by the Japan Research Committee for Intractable Vasculitis. Methods PubMed, CENTRAL, and the Japan Medical Abstracts Society databases were searched for articles published between 2015 and 2020 to update the systematic review for existing clinical questions, while PubMed, CENTRAL, EMBASE, and the Japan Medical Abstracts Society were searched for articles published between 2000 and 2020 to conduct a systematic review for newly developed clinical questions. The certainty of evidence was assessed with the GRADE approach. Results For remission induction, when used in conjunction with cyclophosphamide or rituximab, reduced-dose glucocorticoid lowered the risk of serious adverse events compared to standard-dose glucocorticoid. Avacopan improved sustained remission at 12 months compared to high-dose glucocorticoid. Addition of plasma exchange to remission induction therapy did not reduce the risk of death, end-stage kidney disease, or relapse. For remission maintenance, rituximab reduced the risk of relapse compared to azathioprine. Long-term rituximab or azathioprine reduced the risk of relapse compared to short-term rituximab or azathioprine, respectively. Conclusion This systematic review provided evidence required to develop the 2023 clinical practice guideline for the management of ANCA-associated vasculitis.

Improved Hearing Impairment of Granulomatosis with Polyangiitis Treated with Rituximab and Avacopan without Glucocorticoids

“…Active glomerulonephritis is a life-threatening condition. Glucocorticoids and cyclophosphamide, or glucocorticoids and rituximab are recommended in the clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare as induction therapy for GPA (14). Rituximab was selected because the patient lived on a remote island, so frequent hospitalization for treatment with intravenous cyclophosphamide was difficult.…”

“…Rituximab was selected because the patient lived on a remote island, so frequent hospitalization for treatment with intravenous cyclophosphamide was difficult. Furthermore, the EULAR (9) and Japan Research Committee of the Ministry of Health, Labour, and Welfare (14) suggest the use of avacopan in combination with rituximab or cyclophosphamide in the induction of remission in GPA or MPA as part of a strategy to substantially reduce exposure to glucocorticoids. Following the patient's wishes and these recommendations, avacopan was used without glucocorticoids in order to avoid glucocorticoids' side effects of infection, especially that caused by P. aeruginosa cultured in sputum.…”

T follicular helper cells and T peripheral helper cells in rheumatic and musculoskeletal diseases

“…ANCA-associated vasculitis can affect any organs of the body and present with fever, weight loss, vision and hearing loss, epistaxis, haemoptysis, pneumonia, renal failure, joint pain, skin rash and neurological involvements 69–71. Early detection and treatment initiation are crucial, as delayed treatment may lead to serious complications and worse prognosis 72 73…”

Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations

“…In addition, the optimal initial GC dose and methods for tapering the GC dose have not yet been fully established. In remission induction therapy for MPA and GPA, a reduced-dose GC regimen is almost as effective as a standard-dose regimen and has fewer side effects [ 111 , 112 , 113 ]. In addition, avacopan, a C5a receptor inhibitor, has emerged as an alternative to high-dose GC therapy for MPA and GPA but not for EGPA [ 114 ].…”