idiopathic retroperitoneal fibrosis
Conditions
Interventions
glucocorticoid+Pirfenidone group:glucocorticoid+Pirfenidone
Sponsors
Beijing Friendship Hospital Affiliated to Capital Medcal University
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: 1. Presence of a new-onset or recurrent IRF New cases were defined as finding soft tissue masses around the abdominal aorta and iliac artery on imaging (CT / MRI), with or without involvement of adjacent structures such as ureter and inferior vena cava, excluding [5] of tumors (such as lymphoma, sarcoma, etc.), drugs, methyl sulfide, methyldopa, peroride, ermic alkaloids), infection (e. g., tuberculosis), radiotherapy, trauma, major surgery, systemic connective tissue disease (e. g., SLE, tuberous arteritis). A recurrent is defined when the patient meets any one of the following: Disease-related aggravation of hydronephrosis or imaging findings of an enlarged mass (20% greater maximum diameter), with or without IRF-related symptoms; The IRF-related symptoms appeared, associated with a 50% increase in CRP / ESR, but with no hydronephrosis or imaging mass enlargement of <20%; Note: A 50% elevated CRP / ESR alone patient without IRF-related symptoms could not be considered as a relapse. 2. Age: 18 years old 3. Fully informed consent, voluntarily participated in the trial, and signed the informed consent form
Exclusion criteria
Exclusion criteria: 1. Suffering from other rheumatic immune diseases, including but not limited to rheumatoid arthritis, spinal arthritis, systemic lupus erythematosus, polymyositis, dermatomyositis, systemic sclerosis, sarcoidosis, etc.; 2. Patients with uncontrolled heart failure, arrhythmia, severe pulmonary hypertension, unstable coronary atherosclerotic heart disease, serious gastrointestinal diseases, malignant tumors, and neuropsychiatric diseases, which the researchers consider are not suitable for patients to participate in the trial; 3. Patients with a previous tumor history within 5 years prior to enrollment. Patients with cervical carcinoma in situ or cutaneous squamous epithelial tumors undergoing radical resection within 3 years and evaluated with no evidence of recurrence can be enrolled; 4. Patients with serious viral, bacterial, fungal or parasitic infections within 4 weeks before enrollment and not considered unsuitable by the investigator to participate in the trial; 5. Female patients during pregnancy or lactation, or female patients who have a pregnancy plan or cannot voluntarily take effective contraceptive measures within one year; 6. A history of severe liver damage or end-stage liver disease; 7. A history of end-stage renal disease or kidney transplant requiring dialysis; 8. A history of alcohol abuse or drug abuse in the past two years; 9. History of photosensitive dermatitis; 10. History of peptic ulcer disease within 6 months; 11. People who need fluvoxamine (a drug for depression or compulsive mental disorder); 12. Patients receiving the following drugs: Treatment with any biological agents within 12 weeks before enrollment, including but not limited to tozizumab, infliximab, adalimumab, etanercept, ababil, and golimuumab; Patients who were treated with other B cell clearance therapy for 24 weeks before enrollment, including but not limited to rituximab, bellimimumab, or intravenous immunoglobulin infusion; Patients treated with any abiotic DMARDs or immunosuppressive agent within 12 weeks prior to enrollment, including, but not limited to, cyclophosphamide, mycophenolate mofetil, methotrexate, ailamod, etc. Patients receiving leflunomide, those who did not receive 11 days of 8g tid, or who were stopped for less than 2 years, should be excluded; Patients who had received tamoxifen within 12 weeks prior to enrollment; 13. Patients who have used the study drugs with unclear efficacy in other clinical trials within 12 weeks prior to enrollment; 14. Serious routine blood tests are abnormal: Hemoglobin content was 3 normal upper limit; Alanine aminotransferase (ALT)> 3 upper limit of normal; Total bilirubin (TBIL)> 1.5 upper limit of normal; Alkaline phosphatase (ALP)> 2.5 normal upper limit;If IgG4-related IRF and hepatobiliary system activity performance: Asparpartate aminotransferase (AST)> 10 normal upper limit; Alanine aminotransferase (ALT)> 10 normal upper limit; Total bilirubin (TBIL)> 5 upper limit of normal; Alkaline phosphatase (ALP)> 5 normal upper limit; 16. The glomerular filtration rate (eGFR) is <30 mL/minute / 1.73 m2.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Changes in the percentage of the imaging mass reduced from baseline at week 48;The incidence of adverse events at week 48.; | — |
Secondary
| Measure | Time frame |
|---|---|
| Imaging remission rate of pirfenidone for IRF at week 48;Symptom improvement rate at week 48;Rate of improvement of inflammatory indicators at week 48;Blood creatinine improvement rate of renal function improvement at week 48;Extraction rate of a ureteral stent or nephrostomy; | — |
Countries
China
Contacts
Public ContactYanying Liu
Beijing Friendship Hospital Affiliated to Capital Medcal University
Outcome results
None listed