Long-term outcomes with multi-targeted immunosuppressive protocol in children with severe proliferative lupus nephritis

• Published in: Lupus 2016-04, Vol.25 (4), p.399-406
• Main Authors: Aragon, E, Resontoc, L P, Chan, Y H, Lau, Y W, Tan, P H, Loh, H L, Ng, K H, Yap, H K
• Format: Article
• Language: English
• Subjects: Administration, Intravenous; Adolescent; Age Factors; Child; Child, Preschool; Children; Complement component C3; Cyclosporine - administration & dosage; Cyclosporins; Disease-Free Survival; Drug Therapy, Combination; Epidermal growth factor receptors; Female; Follow-Up Studies; Glomerular filtration rate; Hospitalization; Humans; Immunosuppression; Immunosuppressive Agents - administration & dosage; Intravenous administration; Kaplan-Meier Estimate; Kidneys; Logistic Models; Lupus; Lupus nephritis; Lupus Nephritis - diagnosis; Lupus Nephritis - drug therapy; Lupus Nephritis - immunology; Male; Methylprednisolone; Methylprednisolone - administration & dosage; Mycophenolate mofetil; Mycophenolic acid; Mycophenolic Acid - administration & dosage; Nephritis; Preservation; Proteinuria; Pulse Therapy, Drug; Recurrence; Remission; Remission Induction; Risk Factors; Severity of Illness Index; Singapore; Survival; Survival analysis; Systemic lupus erythematosus; Time Factors; Treatment Outcome; Urine
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/0961203315615220

We have previously reported the one-year outcomes of 16 children with severe proliferative lupus nephritis (LN) who were treated using a multi-targeted induction protocol based on intravenous (IV) pulse methylprednisolone (MP), mycophenolate mofetil (MMF) and cyclosporine (CSA). This study examined the long-term renal outcomes of these 16 children, followed up for a median duration of 9.2 years (range 5.8–14.2 years). Primary treatment outcome was complete renal remission. Secondary outcomes included patient and renal survival as well as relapse-free and event-free survival. All patients achieved complete renal remission within 24 months (median 8.7 months, range 4.0–24.0 months). Comparing clinical and laboratory parameters at induction and last follow-up, respectively, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (25.4 ± 8.7 vs 0.4 ± 0.8), serum complement C3 (47 ± 21 vs 107 ± 27 mg/dL), estimated glomerular filtration rate (eGFR) (72 ± 57 vs 109.7 ± 43 ml/min/1.73m2) and urine protein (6.97 ± 7.09 vs 0.2 ± 0.02 g/day/1.73m2) improved significantly (p 1 g/day/1.73m2. Cumulative probability that hospitalization would not be required was 93.8% at one year, and 71.4% at ten years. Our multi-targeted protocol for induction and maintenance therapy in Asian children with severe proliferative LN resulted in good long-term patient survival and renal preservation, with a good safety profile.