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

EULAR recommendations: Vasculitis (Large vessel vasculitis)

Large vessel vasculitis (LVV)- Takayasu and GCA:-

1. Thorough clinical and imaging assessment of the arterial tree when a diagnosis of Takayasu is suspected-
• MRA/PET could assist in diagnosis and document of extent of involvement but has its limitations (not widely available, operator dependant).
• Conventional angiogram is gold standard

2. In Giant cell arteritis,
• Temporal artery biopsy should be performed
• 1cm tissue length is required
• skip lesions may lead to false negative HPE
• Don’t delay treatment while waiting for biopsy. Treat first.
• If CRP/ESR is not elevated, think of another diagnosis
• USG of the temporal artery looking for vessel wall oedema was 88% sensitive and 97% specific in diagnosing GCA

3. Start steroids early and at high dose for induction of remission of LVV
• Prednisolone- 1mg/kg (max 60mg) daily
• Maintain for 1 month then taper
• Taper should not be in the form of EOD therapy which is a/w higher relapse rate
• At 3 months, steroid dose should be at 10-15mg/d
• Steroid duration could be for several years
• Must give bone protection during this period

4. Immunosuppressive agents should be considered in LVV as adjunctive therapy
• Methotrexate (20-25mg weekly)
• Azathioprine (2mg/kg/d)
• Cyclophosphamide (in steroid resistant Takayasu)

5. Monitoring of LVV- clinical and inflammatory markers

6. Use low dose aspirin in all GCA pt

7. Reconstructive surgery for Takayasu should be performed during the quiescent phase at expert centres

Equivalent anti-inflammatory doses of different oral corticosteroids

This table takes no account of mineralocorticoid effects, nor does it take account of variations in duration of action

Prednisolone 5mg

is equivalent to betamethasone 750 mcg

is equivalent to cortisone acetate 25 mg

is equivalent to dexamethasone 750 mcg

is equivalent to deflazacort 6mg

is equivalent to hydrocortisone 20mg

is equivalent to methylprednisolone 4mg

is equivalent to traimacinolone 4mg