Short-term outcomes of severe lupus nephritis in a cohort of predominantly African–American children

Abstract

Renal involvement is one of the major determinants of the outcome in patients with systemic lupus erythematosus. Although African-American ethnicity has been suggested to be a poor prognostic factor in severe lupus nephritis in adult patients, information on outcomes of African-American children with this disease is still very limited. We retrospectively studied the patients diagnosed with severe lupus nephritis by renal biopsy at Le Bonheur Children's Medical Center from January 1990 to December 2003. All patients were below the age of 18 years at the time of biopsy. Clinical features assessed included age, gender, race, estimated glomerular filtration rate (GFR), presence of hypertension, gross hematuria, degree of proteinuria, complement 3 and 4 levels, serum albumin, renal histology and dose of oral prednisone. Forty-four patients were studied: 82% were African-American and 89% were female. Mean age at biopsy was 14.2+/-3 years (median 15.0 years; range 4.7 years to 17.0 years). Renal biopsies were assessed according to the WHO classification. Twenty-seven percent, 43%, and 30% were in class III, IV and V, respectively. At presentation, 55% had hypertension and 23% had a history of macroscopic hematuria. The patients had varying degrees of proteinuria, including 18% with nephrotic syndrome. Eighteen percent had moderate renal insufficiency with estimated GFRs less than 50 ml/1.73 m2 body surface area per minute. All the patients were treated with corticosteroids. Sixty-eight percent also received cyclophosphamide and 20% received either mycophenolate mofetil (MMF) or azathioprine (AZA). Two patients developed end stage renal disease and required chronic dialysis within 12 months of biopsy. At the 12-month follow-up visit, 23% of patients had complete remission and 48% had partial remission. The mean estimated GFR had increased from 96.0 ml/1.73 m2 per minute to 124 ml/1.73 m2 per minute (P=0.03). Mean serum creatinine levels decreased from 1.62 mg/dl to 0.91 mg/dl (P=0.03). Complement 3 levels increased from 54.3 mg/dl to 90.3 mg/dl (P<0.01). Mean serum albumin levels also increased from 2.8 mg/dl to 3.6 mg/dl (P<0.01) and urine protein-to-creatinine ratio decreased from 5.8 to 1.0 (P<0.01). The average prednisone dose decreased from 0.96 mg/kg per day to 0.41 mg/kg per day (P=0.64). In our center, with predominantly African-American children, patients with lupus nephritis presented similarly to those in other studies with predominantly Caucasian patients, and short-term renal outcomes were not different.

Three Decades of Progress in Treating Childhood-Onset Lupus Nephritis

“…Class V, membranous nephropathy, was as likely to be associated with progression as was class IV, and these patients were treated similarly. The clinical distinction of these two LN classes remains controversial, and our experience is consistent with other reports from nonwhite pediatric populations (35,36).…”

Predictors of Relapse and End Stage Kidney Disease in Proliferative Lupus Nephritis

“…Prior estimates of treatment resistance in children are more difficult to find as most of these studies report only long-term survival estimates. Treatment resistance was observed in 13% of a cohort of (n ϭ 77) Korean children with lupus nephritis and in 29% of a group of predominately (n ϭ 44) African American children with lupus nephritis (3,9). Treatment resistance was observed only among African Americans in our cohort.…”

“…The prevalence and significance of disease relapse and impact of available treatments in pediatric patients with lupus nephritis are poorly understood. Most published studies in pediatrics are small, single-center reports, and outcome data are often difficult to generalize to populations that are demographically diverse (2,(7)(8)(9)(10). This retrospective study entails a predominantly African American cohort of pediatric patients with lupus nephritis and a high prevalence of proliferative disease.…”

A prospective study on the use of mycophenolate mofetil in children with cyclosporine-dependent nephrotic syndrome

“…Because of its potent immunosuppressive action, MMF has emerged a new agent for treatment of patients with lupus nephritis, steroid-dependent and steroid-resistant NS without serious adverse effects such as nephrotoxicity [4][5][6][7][8][9][10][11]. However, there is no consensus on the best way to monitor its efficacy and tailor its dose because of little data in children with glomerular disease.…”