EULAR recommendations- vasculitis (Medium and small vessel vasculitis)

Medium and small vessel vasculitis- Wegener’s, Microscopic polyangiitis, Churg-strauss, PAN, Cryoglobulinaemia.

1. Management is to be in expert centres
• Its rarity makes management in normal settings suboptimal
• Specialised services may be required

2. ANCA must be done (both indirect IF and ELISA)

3. Positive biopsy in strongly supportive of vasculitis
• Fibrinoid necrosis
• Pauci-immune GN (segmental necrosis, extracapillary proliferation)
• Granuloma
• Biopsy esp helpful in ANCA negative pt

4. Clinic visits- structured assessment is required (ie checklist- clinical, urine, laboratory)
• To avoid missing the multi-organ involvement

5. Treatment is based on severity
• Localised
• Early systemic (any, without organ threatening or life threatening disease)
• Generalized (renal or other vital organ failure, serum creat >500umol/l)
• Severe (renal or other vital organ failure, serum creat >500umol/l)
• Refractory (unresponsive to steroids/cyclophos)

6. WG/MPA
• Induction:- Cyclophosphamide (oral 2mg/kg/d max 200mg/d) and prednisolone (1mg/kg/d max 60mg/d)
• Pulse IV Cyclophos- higher remission rate with lower A/E but higher rate of relapse
1. EUVAS regime- 15mg/kg (max 1.2g) 2 weekly IV cyclophosphamide for 3 pulses, then 3 weekly for 3-6 pulses. (dose adjusted for age and renal function)

7. PAN/CSS
• Induction:- Cyclophosphamide + steroids- better remission vs steroid alone.
• IV versus oral cyclophosphamide
• Lower A/E and equal efficacy in PAN pt

8. Cyclophosphamide therapy
• Mesna
• PCP prophylaxis (480mg dly or 960mg 3 times a week)

9. Non organ threatening or non life threatening ANCA associated vasculitis
• MTX (oral/IV) and steroids- acceptable less toxic alternative

10. Plasma exchange in pt with RPGN (with serum creatinine > 500umol/l)

11. Maintenance therapy- Steroids + Azathioprine, lefllunomide or MTX

12. Patients who failed remission or relapse despite on maximum doses of standard therapy –
• MMF
• Anti TNF (infliximab)
• RTX
• IVIG
• ATG

13. Cryoglobulinemia
• Mixed essential- treat as small vessel vasculitis
• RTX in hepatitis associated cryoglobulinemic vasculitis may be of benefit

14. HepC associated cryoglobulinaemic vasculitis- anti viral therapy

15. HepB associated PAN- antiviral therapy + steroids + plasma exchange