childhood-onset, complicated, steroid-resistant nephrotic syndrome
Conditions
Interventions
Rituximab and methylprednisolone pulse therapy
Sponsors
Japanese Study Group of Kidney Disease in Children
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1.Diagnosed as idiopathic nephrotic syndrome (INS) according to the ISKDC criteria. 2.The first onset of INS is under 18 years of age, and 2 years of age or older at assignment. 3.Patients who have received these 1) and 2) within 1 year prior to assignment. 1) CNI(cyclosporine or taclorimus) over four months 2) Methylprednisolone pulse therapy (more than three times to 12 times) 4. Urinary protein to creatinine ratio is over 2.0 g/gCr and Serum albumin is under 3.0 g/dL 5. Patients in whom 5/microL or more CD20-positive cells are observed in the peripheral blood. 6. Patients who can be hospitalized overnight on the first day of rituximab administration. 7. Written informed consent.
Exclusion criteria
Exclusion criteria: 1.History of nephritic-NS, such as IgA nephropathy prior to assignment or in whom secondary NS is suspected. 2.Patients who have been detected gene abnormality(NPHS2,WT1)or in whom gene abnormality(NPHS2,WT1)is suspected. 3.History of receiving rituximab within 1 years prior to assignment. 4.History of receiving any kinds of monoclonal antibody therapy. 5.Having received a new immunosuppressant within 4 weeks prior to assignment. 6.Patients meeting either one of the following infection: 1)Presence or history of severe infections within 6 months prior to assignment. 2)Presence or history of opportunistic infections within 6 months prior to assignment. 3)Presence of active tuberculosis. 4)Patients with a history of tuberculosis or in whom tuberculosis is suspected. 5)Presence or history of active Hepatitis B or Hepatitis C or hepatitis B virus carrier. 6)Presence of HIV infection. 7.Presence or history of angina pectoris, cardiac failure, myocardial infarction, or serious arrhythmia (findings observed under Grade 4 of the CTCAE v4.0-JCOG. 8.Presence or history of auto-immune diseases or vascular purpura. 9.Presence or history of malignant tumor. 10.History of organ transplantation. 11.History of drug allergies to methylprednisolone, acetaminophen, or d-chlorpheniramine maleate. 12.Uncontrollable hypertension. 13.Deteriorated kidney function, e.g. eGFR<45 mL/min./1.73m2. 14.Having received a live vaccine within 4 weeks prior to assignment. 15.Patients showing either one of the following abnormal clinical laboratory value: 1)WBC <3,000/microL. 2)neutrophil <1,500/microL. 3)PLT <50,000/microL. 4)ALT >2.5 x upper limit of normal value. 5)AST >2.5 x upper limit of normal value. 6)Positive for HBsAg, HBsAb, HBcAb and HCVAb. 7)Positive for HIV antibody. 16. Patients who do not agree with contraception during the study period. 17.Women during pregnancy or breast-feeding. 18.Judged inappropriate for this study by the physicians.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Complete remission rate at month 6 | — |
Secondary
| Measure | Time frame |
|---|---|
| Time to complete remission, Partial remission rate, CKD rate, Urinary protein to creatinine ratio, Estimated glomerular filtration rate, B cell depletion period | — |
Countries
Japan
Contacts
Public ContactMayumi Sako
National Center for Child Health and Development Division for Clinical Trials
Outcome results
None listed