Table 8

Interventions for studies of maintenance treatment

Study ID



Study Outcomes

Hiemstra 2009


Mycophenolate mofetil 2 G/d for 42 months

azathioprine 2 mg/kg/d for 42 months

1. Time to first relapse

2. relapse rate

3. Rate of side-effects and intolerance

4. cumulative doses (AZA, CS, MMF),

5. AUC for BVAS, SF-36 or VDI

6. Evolution of titres of ANCA and


Pagnoux 2008

All patients received identical remission induction therapy. Pulse MP 15 mg/kg for 3 days. Oral prednisolone 1 mg/kg/d for 3 weeks, tapered to 12.5 mg at 6 months. Pulse CPA 0.6 G/m2, 3 doses at 2 week intervals then every 3 weeks until remission, 3 further consolidation doses at 3 week intervals.

Azathioprine 2 mg/kg/d Maintenance therapy continued for 12 months then withdrawn over 3 months.

Trimethoprim-sulfamethoxazole 320/1600 daily recommended for WG patients for additional 2 years.

Methotrexate 0.3 mg/kg/week, increasing every week by 2.5 mg to 25 mg/week. Folinic Acid 25 mg or Folic Acid 5 mg given 48 hours after Methotrexate.

Maintenance therapy continued for 12 months then withdrawn over 3 months. Trimethoprim-sulfamethoxazole 1600/320 daily recommended for WG patients for additional 2 years.

Primary end point:

1. adverse reaction causing death or

leading to discontinuation of the study drug.

Secondary end points:

2. any adverse event,

3. severe adverse event,

4. relapse,

5. relapse free survival,

6. event free survival,

7. quality of life.

WGET 2005

Etanercept 25 mg twice weekly by subcutaneous injection Immunosuppression as for control group.

Twice weekly placebo injection

Severe disease: Cyclophosphamide 2 mg/kg/d.

Replaced with methotrexate if in remission at 3 to 6 months.

Limited disease: Methotrexate 0.25 mg/kg/week to maximum of 25 mg/week. 12 months after remission, MTX dose cut by 2.5 mg each month.

Prednisolone was given to patients with severe and limited disease starting at 0.5 to 1.0 mg/kg/d.

Tapered to 0 mg at 6 months if no relapse.

Patients in remission with creatinine > 2 mg/dl received Azathioprine 2 mg/kg/d, decreased after 12 months in remission by 25 mg each month.

1. Sustained remission. BVAS/WG score 0 for at least 6 months.

2. Number and rate of flares during treatment, percentage of patients with sustained low level of disease activity (BVAS/WG < 3 for at least 6 months), percentage of patients with a remission, cumulative area under the curve for the BVAS/WG, adverse events, quality of life.

Metzler 2007

Leflunomide. Loading dose of 100 mg/d for 3 days, followed by 20 mg/d from day 4 to end of week 4. Then increased to 30 mg/d thereafter.

Prednisolone as per methotrexate group.

Methotrexate: 7.5 mg/week weeks 1 to 4. 15 mg/week for weeks 5 to 8. 20 mg/week after week 8.

Prednisolone allowed at a dose of 10 mg/d or less.

In the absence of disease activity, the dose was tapered by 2.5 mg/month to 5 mg, then by 1 mg/month.

1. Number of major and minor relapses.

2. Disease Extent Index (DEI), BVAS, patient self assessment of quality of life (SF-36), cANCA titre, ESR, CRP.

Stegeman 1996

Co-trimoxazole 960 mg twice daily


1. Death

2. Remission

Jayne 2003

CPA 1.5 mg/kg/d from remission.

Switched to AZA 1.5 mg/kg/d 12 months after study entry.

Immunosuppression as for control group.

After remission induction, AZA 2 mg/kg/d with Prednisolone 10 mg/d.

BOTH GROUPS: Remission induction with oral CPA 2 mg/kg/d and prednisolone 1 mg/kg/d tapered to 0.25 mg/kg/d by 12 weeks. From 12 months both groups received AZA 1.5 mg/kg/d and prednisolone 7.5 mg/d.

1. Relapse by 18 months

2. Side effects including leucopenia and infections

Walters et al. BMC Nephrology 2010 11:12   doi:10.1186/1471-2369-11-12

Open Data