A single-center experience of 13 Iranian children with lupus nephritis and pericarditis

Abstract

Although pericarditis with lupus nephritis is a recognized complication of systemic lupus erythematosus (SLE) [1], there are very few data on its manifestations and outcome in children. In this single-center report, we have reviewed outcome for 13 Iranian children with lupus nephritis who developed pericarditis. All patients were under follow up in the Pediatric Nephrology Department of Tehran University of Medical Sciences during the period from 1985 to 2005. The diagnosis of SLE met the criteria of the American Rheumatism Association (ARA) [2]. To characterize disease activity, we used the criteria of the SLEDAI (systemic lupus erythematosus disease activity index) [3]. All patients studied had at least one trans-thoracic echocardiography and all were documented by echocardiography as having pericarditis. Because of the retrospective nature of the study and incomplete records, we were unable to retrieve data about the severity and complications of pericarditis, for example cardiac tamponade, constrictive pericarditis, and purulent pericarditis, but no patient required pericardiocentesis or pericardial surgery. End-stage renal disease (ESRD) was defined as patients who required renal replacement therapy with dialysis and renal transplantation. All patients were initially treated with oral corticosteroids (CS) prescribed at a dose of 2 mg/kg per day, with a daily maximum of 60 mg/day. Additional therapy included intravenous cyclophosphamide (CP), azathioprine (AZA), and mycophenolate mofetil (MMF). Maintenance treatment varied for individual patients: AZA was used to treat patients diagnosed before 2001 and MMF in cases starting with 2001. To assess potential predictive factors for developing pericarditis we reviewed our data registry to find matched controls with the highest levels of similarity in their lupus nephritis, sex, and age classes for each of our pericarditis patients. Nonparametric tests and Kaplan–Meier and Cox proportional regression methods were used where appropriate. Table 1 shows data for the 13 children included in this study. Study population consisted of five (39%) males and eight females. Mean age was 10.8 ± 2.6 years; mean creatinine value at the time of pericarditis was 1.4 ± 0.9 mg/dl. Mean follow up F. Beiraghdar E. Nemati B. Einollahi Nephrology Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran