Comparison of Tofacitinib and Methotrexate in the Maintained Treatment of GPA

Inclusion Criteria:

1. Patients with newly diagnosed or relapsing Granulomatosis with polyangiitis met thecriteria of 1990 ACR and 2012 Chapel Hill criteria
2. Patients in disease flare have achieved remission using a treatment combiningcorticosteroids and IV cyclophosphamide
3. Remission is defined as a Birmingham Vasculitis Activity/ Wegener's granulomatosis (BVAS/WG) score of 0 and receiving 10 mg/day of oral prednisone (or equivalent) atleast 2 weeks
4. Age 18 to 75 years
5. Written informed consent obtained before taking part in the study

Exclusion Criteria:

1. Severe GPA defined as potentially organ- or life-threatening disease (i.e. alveolarhaemorrhage, heart failure caused by myocarditis or pericarditis, progressiveneurological symptoms, deaf, blindness, et al.)
2. Serum creatinine>120umol/L or proteinuria>1.0g/d
3. Failure to response after treatment with methotrexate or cyclophosphamide previously
4. Receipt of a JAKi therapy previously
5. Co-existence of another systemic autoimmune disease
6. Secondary vasculitis (following neoplastic disease, an infection or antithyroid drugs)
7. Malignancy or history of malignancy
8. Infection by HIV, HCV, HBV or tuberculosis
9. Severe uncontrolled cardiovascular, pulmonary, liver, gastrointestinal, endocrine,hematological, neurological, or psychiatric diseases that are not related to systemicvasculitis
10. Allergic to any of the medication (cyclophosphamide, corticosteroids, tofacitinib,methotrexate)
11. Blood dyscrasias including confirmed: Hemoglobin <9 g/dL or Hematocrit <30%; Whiteblood cell count <3.0 x 109/L; Absolute neutrophil count <1.5 x 109/L; Platelet count <100 x 109/L; Alanine transaminase or aspartate aminotransferase or totalbilirubin>1.5 upper normal limit; Estimated glomerular filtration rate<60ml/min/1.73m2
12. Any medical or psychiatric disorder which, in the investigator's opinion, may preventthe administration of treatment and patient follow-up according to the protocol,and/or which may expose the patient to a too greater risk of an adverse effect.
13. Incapacity or refusal to understand or sign the informed consent form.
14. Pregnancy, breastfeeding.