Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.
Therapeutic Approaches for the Treatment of New Onset and Flared Juvenile Systemic Lupus Erythematosus with Active Renal Disease: An International Multicenter PRINTO Study.
Miettunen, Paivi, Pistorio, Angela, Ravelli, Angelo, Oliveira, Sheila, Alessio, Maria, Cuttica, Ruben, Mihaylova, Dimitrina
To evaluate in a prospective international cohort of juvenile systemic lupus erythematosus (JSLE) with active renal disease (ARD) response to therapy over a 24-month period.
ARD was present in 240 (78.3% female) out of 557 patients with JSLE, age < 18 years (yr): 134 new onset JSLE (N-JSLE) and 106 flared JSLE (F-JSLE). Disease activity parameters and therapeutic approaches were analyzed at baseline, 6, 12 and 24 months, in 4 geographic areas. Response was assessed according to the PRINTO/ACR JSLE criteria, serum creatinine and glomerular filtration rate (GFR). Logistic regression was used to identify potential prognostic indicators at baseline for poor renal outcome at 24 months, defined as end stage renal disease (ESRD) or urinary protein >=3.5 grams/24 hours.
The mean age at disease onset was 13.1 (SD 2.8) yr for N-JSLE and 10.2 (SD 2.9) yr for F-JSLE, with mean disease duration of 0.3 (SD 0.2) yr for N-JSLE and 4.2 (SD 2.9) yr for F-JSLE. A total of 143/240 (59.6%) patients had all 4 time point assessments available. At baseline, new and flared patients had similar renal activity, but N-JSLE had higher SLE activity overall, with more serositis, hematological, mucocutaneous and musculoskeletal manifestations. Corticosteroids were initiated at similar median doses: 1.0 mg/kg/day in N-JSLE and 0.8 mg/kg/day in F-JSLE. Pulse steroids were initiated in 33.6% of N-JSLE and 40.6% of F-JSLE. Cyclophosphamide was the most common (41.2%; 99/240) immunosuppressive medication, followed by azathioprine (25%; 60/240).Mycophenolate mofetil and azathioprine were used more commonly in F-JSLE. Latin American patients received more pulses and higher doses of oral steroids when compared to Western Europe. Use of cyclophosphamide was similar in all 4 regions. Seventy eight % (103/132) of N-JSLE compared to 57.4% (58/101) of F-JSLE (p=0.0007) reached at least PRINTO/ACR 70 level of response at 6 months, which increased to 87.1% in N-JSLE and 77.2% in F-JSLE at 24 months (p=0.12). The median serum creatinine and GFR were within normal range at all time points; all other measures showed statistically significant improvement over time in both groups (p <=0.0002). ESRD developed in 6 patients. Urinary protein >=3.5grams/24 hours was present in 50/240 patients at baseline and in 7/143 patients at 24 months. No predictors for poor renal outcome were identified. Twelve patients (5%) died, 10/12 before 12 months, and 7/12 of sepsis. Corticosteroids were discontinued in 20/143 by 24 months.
Despite higher overall lupus activity at baseline, N-JSLE and F-JLSE improved similarly over 24 months. ESRD was rare. Some differences in therapeutic approaches exist worldwide.
To cite this abstract, please use the following information:
Miettunen, Paivi, Pistorio, Angela, Ravelli, Angelo, Oliveira, Sheila, Alessio, Maria, Cuttica, Ruben, et al; Therapeutic Approaches for the Treatment of New Onset and Flared Juvenile Systemic Lupus Erythematosus with Active Renal Disease: An International Multicenter PRINTO Study. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :2009