Management of ANCA-associated vasculitis: Current trends and future prospects

Figures & data

Figure 1 Chest radiograph showing multiple cavitating lung lesions in a patient with Wegener’s granulomatosis.

Figure 2 Renal biopsy from a patient with AAV and severe renal failure showing a glomerulus containing an extensive cellular crescent with a break in the basement membrane and surrounding fibrin deposition (arrowed) Methenamine silver stain, X400.

Abbreviation: AAV, ANCA-associated vasculititis.

Table 1 EUVAS disease categorization of ANCA-associated vasculitis⁹

Category	Definition
Localized	Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms
Early systemic	Any, without organ-threatening or life-threatening disease
Generalized	Renal or other organ threatening disease, serum creatinine <500 μmol/L (5.6 mg/dL)
Severe	Renal or other vital organ failure, serum creatinine >500 μmol/L (5.6 mg/dL)
Refractory	Progressive disease unresponsive to glucocorticoids and cyclophosphamide

Figure 3 Biochemical changes over time in a patient with PR3-ANCA demonstrating rapid decrease in serum creatinine, ANCA titer and C-reactive protein following initiation of immunosuppression.

Abbreviations: ANCA, antineutrophil cytoplasm antibody; PR3-ANCA, proteinase-3 antineutrophil cytoplasm antibody.

Table 2 Dose modification of pulsed intravenous CYP as used in the CYCLOPS trial⁴⁴

Pulsed CYC dose reductions for renal function and age
Age (years)	Creatinine (μmol/L)
	<300	300–500
<60	15 mg/kg/pulse	12.5 mg/kg/pulse
60–70	12.5 mg/kg/pulse	10 mg/kg/pulse
>70	10 mg/kg/pulse	7.5 mg/kg/pulse

See www.vasculitis.org/protocols/CYCLOPS.pdf

Table 3 Suggested schema for the management of ANCA-associated vasculitis, adapted and updated from Pallan et al⁹⁸

Disease severity	EUVAS definition	Induction therapy	Maintenance therapy
Localized	Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms	Methotrexate and steroids	Low-dose steroids plus azathioprine or leflunomide or methotrexate (+ trimethoprim-sulphamethoxazole also added in WG)
Early systemic	Any, without organ-threatening or life-threatening disease	Methotrexate or cyclophosphamide and steroids	Low-dose steroids plus azathioprine or methotrexate
Generalized	Renal or other organ threatening disease, serum creatinine <500 μmol/L (5.6 mg/dL)	Cyclophosphamide (or rituximab) and steroids	Low-dose steroids plus azathioprine Mycophenolate mofetil as second-line agent
Severe	Renal or other vital organ failure, serum creatinine >500 μmol/L (5.6 mg/dL)	Cyclophosphamide and steroids plus plasma exchange (rituximab instead of CYC)	Low-dose steroids plus azathioprine Mycophenolate mofetil as second-line agent
Refractory	Progressive disease unresponsive to glucocorticoids and cyclophosphamide	Deoxyspergualin, antithymocyte globulin, or rituximab	No consensus

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