Newly onset microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA)
Conditions
Interventions
Candidates of the study subjects take screening tests after giving their consent, and if they are judged to be eligible, they are registered in this study. While awaiting screening test results, methy
Sponsors
Harigai Masayoshi
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients who meet all of the following criteria are included in this study: 1) Patients who give their consent in written form by themselves or their legal representatives 2) Patients who are 18 years or older at giving their consent 3) Patients who are newly dignosed with microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA), consistent with the Chapel Hill consensus conference (CHCC) definition, and who meet the MPA or GPA classification criteria of EULAR/ACR 2022 4) Patients who are positive in MPO-ANCA or PR3-ANCA at diagnosis by either of ELISA, CLEIA, or FEIA
Exclusion criteria
Exclusion criteria: Patients who fall into any of the following criteria are excluded from participating in the study: 1) Patients who initiated treatment for ANCA-associated vasculitis (GC, immunosuppressive agents, or molecular target agents) *1 2) Patients with glomerulonephritis and with eGFR of less than 15ml/min/1.73m2, or with complication of pulmonary alveolar hemorrhage requiring oxygen administration of 2L/min or more 3) Patients with comorbidity of other systemic autoimmune diseases *2 4) Patients with HIV infection, current infection or history of HBV or HCV *3 5) Patients who wish to have a baby, current pregnant, or current breastfeeding 6) Patients with history of malignant tumor within 5 years before giving their consent *4 7) Patients with history of active tuberculosis within a year before giving their consent 8) Patients with history of severe allergy or anaphylaxis by treatment with monoclonal antibodies 9) Patients with comorbidity which may require GC, immunosuppressive agents, biopharmaceuticals, plasmapheresis, or high-dose intravenous immunoglobulin *5 10) Patients who received treatment with biopharmaceuticals targeted B-cell within 6 months before giving their consent (eg. rituximab, belimumab) 11) Patients with history of use of avacopan 12) Patients who cannot receive oral administration of avacopan and prednisolone at the initiation of the study *6 13) Patients with other conditions by which responsible investigator or subinvestigator judge to be inappropriate to conduct this study safely *1. Oral GC administration is allowed up to 7 days including the screening period if prednisolone equivalent of 0.5mg/kg/day or less is administered at another hospital or department before giving their consent. Patients with prior pulmonary lesions treated by antifibrotic agents (nintedanib, pirfenidone) can participate in this study, but with other treatments (eg. immunosuppressive agents) cannot participate in this study. *2. Patients with rheumatoid arthritis without severe internal organ lesion, or patients with scleroderma or Sjogren's syndrome without treatment with GC can participate in this study. *3. Patients with HBs negative, or HBs or HBc positive and HBV-DNA negative can participate in this study under monitoring of HBV-DNA. Patients who achieved sustained virological negative after the treatment with direct acting antivirals (DAA) can participate in this study. *4. Patients with intramucosal carcinoma that is judged to be curatively resected can participate in this study. *5. Patients with well-controlled bronchial asthma without oral GC can participate in this study (inhaled steroids are allowed). Patients with colorectal disease who are using intravenous steroids are excluded from participating in the study. *6. If the drugs can be administered through a gastric tube, it is also considered as the patients can receive them orally.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Proportion of patients who achieve remission at week 26 (including patients who received rescue treatment defined in the protocol and achieve remission at week 26, but excluding patients who achieved remission after the use of the prohibited drugs or treatments or off-protocol treatments such as additional GC administration not specified as the rescue therapy in the protocol). Remission is defined as achievement of BVAS ver.3 score of zero (or 1 or less in case of minimally invasive and persistent) and use of oral prednisolone of 5mg/day or less. | — |
Secondary
| Measure | Time frame |
|---|---|
| - Proportion of patients who achieve oral prednisolone of 0mg/day and remain in remission at week 104 - Proportion of patients who achieve remission without rescue treatmet at week 26 (excluding patients who achieved remission after the use of the prohibited drugs or treatments or off-protocol treatments such as additional GC administration not specified as the rescue therapy in the protocol) - Proportion of patients who achieve oral prednisolone of 0mg/day and remain in remission without rescue treatmet at week 104 - Proportion and time to death, recurrence, and ESKD. Recurrence is defined as new appearance or re-appearance of one or more items of BVAS ver.3 after remission, regardless of re-administration or dose up of prednisolone. - Cumulative dose of GC at week 26 or 104 - Proportion of patients who experienced rescue treatment till week 26 or 104 - Disease activity assessed by BVAS ver.3 at week 26 or 104 - Irreversible disorder assessed by VDI at week 104 - Health-related QOL assessed by SF-36 at week 26 or 104 - Number of severe adverse events and proportion of patients who experienced the severe adverse events till week 26 or 104 - Proportion of patients with new onset of diabetes, hypertension, and dyslipidemia requiring treatment till week 26 or 104 - Proportion of patients with pathological bone fracture and lumber spine bone density at week 104 - Number of infection requiring oral or transvenous administation of antibiotics, antivirals, or antimycotics and proportion of patients who experienced the infection till week 26 or 104 - Number of severe infection requiring oral or transvenous administation of antibiotics, antivirals, or antimycotics and proportion of patients who experienced the severe infection till week 26 or 104 - Number of hepatic disorder of grade 3 or higher and proportion of patients who experienced the hepatic disorder till week 26 or 104 - Number of malignant tumor of grade 3 or higher and proportion of patients who experienced the | — |
Contacts
Public ContactMasayoshi Harigai
International University of Health and Welfare Narita Hospital
Outcome results
None listed