Antineutrophil cytoplasmic antibody associated vasculitides with renal involvement: Open challenges in the remission induction therapy

Table 2 Current guidelines in the remission induction therapy for antineutrophil cytoplasmic antibody associated vasculitides with severe renal involvement

KDIGO recommendations[23]

Initial treatment of pauci-immune focal and segmental necrotizing GN with or without systemic vasculitis, and with or without circulating ANCA:

We recommend that CYC and CCS be used as initial treatment (1A) We recommend that RTX and CCS be used as an alternative initial treatment in patients without severe disease or in whom CYC is contraindicated (1B) We recommend the addition of PLEX for patients requiring dialysis or with rapidly increasing sCr (1C)

Treatment of relapse

We recommend treating patients with severe relapse of ANCA vasculitis (life- or organ threatening) according to the same guidelines as for the initial therapy (1C)

EULAR/ERA-EDTA recommendations[26]

For remission-induction of new-onset organ-threatening or life threatening AAV we recommend treatment with a combination of CCS and either CYC or RTX

CYC: Level of evidence 1A for GPA and MPA; grade of recommendation A; strength of vote 100%

RTX: Level of evidence 1B for GPA and MPA; grade of recommendation A; strength of vote 82%

For a major relapse of organ-threatening or life-threatening disease in AAV we recommend treatment as per new disease with a combination of CCS and either CYC or RTX

CYC: Level of evidence 1A for GPA and MPA; grade of recommendation A; strength of vote 88%

RTX: Level of evidence 1B for GPA and MPA; grade of recommendation A; strength of vote 94%

PLEX should be considered for patients with AAV and a serum creatinine level of > 500 mmol/L (5.7 mg/dL) due to rapidly progressive glomerulonephritis in the setting of new or relapsing disease. Level of evidence 1B; grade of recommendation B; strength of vote 77%

AAV: Antineutrophil cytoplasmic antibody associated vasculitides; KDIGO: Kidney disease improving global outcomes; EULAR: European league against rheumatism; ERA-EDTA: European renal association-european dialysis and transplant association; GN: Glomerulonephritis; GPA: Granulomatosis with polyangiitis; MPA: Microscopic polyangiitis; CYC: Cyclophosphamide; RTX: Rituximab; CCS: Corticosteroids; PLEX: Plasma exchange.