Rituximab Effective Difficult Steroid Resistant Nephrotic Syndrome

Dr Sidharth Kumar Sethi MD, FISN, FIPNA Journal Club

Efficacy and Safety of Treatment with Rituximab for Difficult Steroid-Resistant and Dependent Nephrotic Syndrome: Multicentric Report Ashima Gulati, Aditi Sinha, Stanley C. Jordan, Pankaj Hari, Amit K. Dinda, Sonika Sharma, Rajendra . Srivastava, Asha Moudgil, and Arvind Bagga Divisions of Pediatric Nephrology and Genetics, and Department of Pathology, AIIMS, New Delhi, India; Nephrology and Transplant Immunology, Cedars-Sinai Medical Center, Los Angeles; and Nephrology and Kidney Transplant, Children’s National Medical Center, Washington, DC

Nephrotic syndrome- why occurs? ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Pediatr Nephrol (2009) 24:1433–1438

Evidence in support of B cell disease ,[object Object],[object Object],[object Object]

Pro-B Pre-B1 Pre-B2 Immature B Mature B Plasma MHC II CD19 CD22 CD34 Ig  VpreB,  5 CD10 CD20

Rituximab and B cells ,[object Object],[object Object],[object Object],[object Object]

Mechanism of Actions ,[object Object],[object Object],[object Object],[object Object],[object Object]

Rituximab and Cell death ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Course of Nephrotic Syndrome 40% 10-15% ISKDC. J Am Soc Nephrol 8: 769–776, 1997 Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children. Clin J Am Soc Nephrol 4: 1593–1600, 2009

Difficult to manage ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Pediatr Nephrol 24: 1525–1532, 2009; Pediatr Nephrol 22: 215-221, 2007

Rituximab ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Semin Arthrit Rheum 36: 71–81, 2006 Pediatr Nephrol 24: 1433–1438, 2009

Rituximab in Nephrotic Syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Material and Methods ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Definitions ,[object Object],[object Object],[object Object],[object Object],[object Object]

Methods ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Rituximab Therapy in SRNS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Rituximab therapy in SDNS ,[object Object],[object Object],[object Object],[object Object]

Rituximab not used ,[object Object],[object Object],[object Object],[object Object],[object Object]

Rituximab Therapy ,[object Object],[object Object],[object Object],[object Object],[object Object]

Concomitant therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Follow-Up ,[object Object],[object Object],[object Object]

Results ,[object Object],[object Object],[object Object]

SRNS patients ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

[object Object],[object Object],[object Object],[object Object]

Longer follow up ,[object Object],[object Object],[object Object],[object Object]

SRNS- Retreatment ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

SDNS patients ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

SDNS patients ,[object Object],[object Object],[object Object]

B-Cell (CD19) Depletion ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Side Effects ,[object Object],[object Object],[object Object],[object Object],[object Object]

Discussion ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

SDNS patients ,[object Object],[object Object],[object Object],[object Object]

Sustained remission in 19 patients (86.3%),

[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Critical Appraisal ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Side Effects ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Limitations ,[object Object],[object Object],[object Object],[object Object],[object Object]

Conclusions ,[object Object],[object Object],[object Object],[object Object]

Rituximab Effective Difficult Steroid Resistant Nephrotic Syndrome

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