Table 7

Inclusion Criteria for studies of maintenance treatment

Study ID

Inclusion criteria

Exclusion criteria

Jayne 2003

Diagnosis of WG, MPA or renal limited vasculitis.

Renal involvement, other threatened loss of function of vital organ, or both.

ANCA positivity. ANCA negative patients enrolled with biopsy evidence of vasculitis.

Cytotoxic drug in previous year.

Other multi-system autoimmune disease, hepatitis B antigenaemia, hepatitis C, HIV infection, cancer, pregnancy.

Age: <18 or > 75 years SCr >500 μmol/L

Stegeman 1996

RPGN on biopsy plus lung disease compatible with WG.

Disease limited to respiratory tract.

Meeting Chapel Hill consensus but not criteria 1

Previous reactions to co-trimoxazole.

GFR < 30 ml/min

Long term treatment with antibiotics

Metzler 2007

18 to 75 years of age.

Diagnosis of generalized WG

Successful induction therapy with prednisolone and CPA.

Bone marrow insufficiency (leukopenia < 4000/μl, Hb < 10 g/dl, thrombocytopaenia > 100,000/μl) serum creatinine > 1.3 mg/dl (115 uM), malignancies, hepatitis B or C or HIV positivity, pregnancy or breast feeding, inadequate contraception, chronic liver disease or alcohol abuse, active gastric ulcer, lack of compliance, further coexisting autoimmune diseases or treatments interfering with the MTX/LEF medication.

WGET 2005

Patients with BVAS/WG score of 3 or more.

Stratified to severe (life-threatening manifestations including RPGN, alveolar haemorrhage or neuropathy) or limited (skin, joints, sinus or mild renal abnormalities).

At least 2 of 5 modified criteria of the American College of Rheumatology for classification of Wegener's Granulomatosis.

Either new or established disease

None stated

Pagnoux 2008

Patients over 18 years old. Newly diagnosed Wegener's Granulomatosis and Microscopic Polyangiitis. Successful remission induction.

use of steroids for more than 1 months prior to CPA therapy, co-existence of another systemic disease, cancer (unless in remission for more than 3 years), HIV, Hep B or C virus infection, contraindication to study drugs, pregnancy, absence of contraception in pre-menopausal women, mental or physical disabilities abrogating ability to consent. Patients not entering remission were not randomised.

Hiemstra 2009


Newly diagnosed patients with WG, MPA or renal-limited vasculitis.

ANCA positivity.

Age 18 to 75 years

Any cytotoxic drug within previous year.

Co-existence of another systemic autoimmune disease, e.g. SLE. Hepatitis B or Hepatitis C infection HIV positivity.

Failure to achieve remission after 6 months of CPA therapy.

Failure to control progressive disease with induction protocol.


Pregnancy or inadequate contraception.

End stage renal failure unless active extra-renal disease requires treatment Inability for informed consent

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

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