BACKGROUND AND OBJECTIVES: Childhood-onset lupus nephritis (LN) carries a worse renal prognosis compared with adults. Controlled treatment trials in children are lacking. We compared renal and patient survival in a cohort of pediatric patients followed over 3 decades. DESIGN, SETTINGS, PARTICIPANTS, & MEASUREMENTS: A retrospective analysis was conducted on 138 patients with childhood-onset systemic lupus erythematosus from 1980 to 2010. The core cohort included 95 with severe LN: 28 progressed to end-stage renal disease (ESRD group) whereas 67 did not (no-ESRD group). Patients were stratified into four "eras" according to the introduction of the primary immuno-suppressive drug: era 1: triple oral therapy with corticosteroids (CS), cyclophosphamide (CYC), and azathioprine (AZA); era 2: intravenous CYC; era 3: mycophenolate mofetil (MMF) ± CYC; era 4: rituximab (RTX) ± CYC ± MMF. RESULTS: Mean age at diagnosis was 12.3 ± 2.9 years with median follow-up of 5 years. Poor renal function (estimated GFR < 60 ml/min per 1.73 m(2)) and nephrotic proteinuria at diagnosis imparted a poor prognosis. Increasing proteinuria correlated with progression of kidney disease. The addition of MMF in era 3 improved 5-year renal survival from 52% to 91% and overall patient survival from 83% to 97%. African-American ethnicity was associated with significant risk for progression to ESRD whereas Hispanic ethnicity conferred an advantage. Infection and cardiovascular disease were the primary causes of patient demise. CONCLUSIONS: Renal and patient survival in childhood-onset LN has improved during the past 3 decades with progressive treatment regimens. Future trials in children are very much warranted.
BACKGROUND AND OBJECTIVES: Childhood-onset lupus nephritis (LN) carries a worse renal prognosis compared with adults. Controlled treatment trials in children are lacking. We compared renal and patient survival in a cohort of pediatric patients followed over 3 decades. DESIGN, SETTINGS, PARTICIPANTS, & MEASUREMENTS: A retrospective analysis was conducted on 138 patients with childhood-onset systemic lupus erythematosus from 1980 to 2010. The core cohort included 95 with severe LN: 28 progressed to end-stage renal disease (ESRD group) whereas 67 did not (no-ESRD group). Patients were stratified into four "eras" according to the introduction of the primary immuno-suppressive drug: era 1: triple oral therapy with corticosteroids (CS), cyclophosphamide (CYC), and azathioprine (AZA); era 2: intravenous CYC; era 3: mycophenolate mofetil (MMF) ± CYC; era 4: rituximab (RTX) ± CYC ± MMF. RESULTS: Mean age at diagnosis was 12.3 ± 2.9 years with median follow-up of 5 years. Poor renal function (estimated GFR < 60 ml/min per 1.73 m(2)) and nephrotic proteinuria at diagnosis imparted a poor prognosis. Increasing proteinuria correlated with progression of kidney disease. The addition of MMF in era 3 improved 5-year renal survival from 52% to 91% and overall patient survival from 83% to 97%. African-American ethnicity was associated with significant risk for progression to ESRD whereas Hispanic ethnicity conferred an advantage. Infection and cardiovascular disease were the primary causes of patient demise. CONCLUSIONS: Renal and patient survival in childhood-onset LN has improved during the past 3 decades with progressive treatment regimens. Future trials in children are very much warranted.
Authors: Stephen D Marks; Sue Patey; Paul A Brogan; Nathan Hasson; Clarissa Pilkington; Patricia Woo; Kjell Tullus Journal: Arthritis Rheum Date: 2005-10
Authors: David Askenazi; Barry Myones; Ankur Kamdar; Robert Warren; Maria Perez; Marietta De Guzman; Anna Minta; M John Hicks; Arundhati Kale Journal: Pediatr Nephrol Date: 2007-03-03 Impact factor: 3.714
Authors: B Namjou; C Gray-McGuire; A L Sestak; G S Gilkeson; C O Jacob; J T Merrill; J A James; E K Wakeland; Q-Z Li; C D Langefeld; J Divers; J Ziegler; K L Moser; J A Kelly; K M Kaufman; J B Harley Journal: Genes Immun Date: 2009-05-14 Impact factor: 2.676
Authors: Gerald B Appel; Gabriel Contreras; Mary Anne Dooley; Ellen M Ginzler; David Isenberg; David Jayne; Lei-Shi Li; Eduardo Mysler; Jorge Sánchez-Guerrero; Neil Solomons; David Wofsy Journal: J Am Soc Nephrol Date: 2009-04-15 Impact factor: 10.121
Authors: Maria Pereira; Eyal Muscal; Karen Eldin; M John Hicks; Anna Carmela P Sagcal-Gironella; Marietta DeGuzman; Scott E Wenderfer Journal: Pediatr Nephrol Date: 2017-07-17 Impact factor: 3.714
Authors: A Boneparth; S E Wenderfer; L Nandini Moorthy; S M Radhakrishna; A C P Sagcal-Gironella; E von Scheven Journal: Lupus Date: 2016-08-20 Impact factor: 2.911