نفريت لوپوسي در كودكان: مطالعه كلينكوپاتولوژي 20 مورد در اهواز

Lupus Nephritis in Children: A clinicopathological study of 20 cases in Ahwaz

Objectives: Systemic lupus erythematous (SLE) is a multi-systemic disease of unknown aetiology that may affect any organ system. Lupus nephritis (LN) remains an important problem in patients with SLE. Clinically significant renal involvement in SLE is more common in children than in adults. The aim of this study was to evaluate the clinical course, histopathology and the efficacy and safety of high-dose pulse cyclophosphamide in children with LN. Subjects and Methods: Retrospectively, 20 children under 16 years of age with LN at the Ahwaz Abouzar childrenʹs hospital were studied from 1995 to 2006. All patients under went percutaneous renal biopsy and were followed up for at least 36 months. Histopathological evaluation of the biopsies were performed or reconfirmed at Shiraz University of Medical Sciences. The clinical and serologic parameters at the time of renal biopsy were recorded. Based on renal biopsy (WHO class: fication for LN), the patients were treated with the following regimens: one patient (class I) with low-dose prednisolone, 7 (class II, III) with high-dose of prednisolone, diuretics and anti-hypertensive drugs, 12 (class IV) with high-dose prednisolone and 13 received intermittent intravenous cyclophosphamide pulses (monthly for 6 months and then every 3 months) followed by mycophenolate mofetil (MMF) as maintenance therapy. Results: The sample consisted of 13(65%) girls and 7(35%) boys. The mean age at the time of diagnosis of SLE was 10.2 (ranging 5- 13) years. Eighteen patients (90%) were more than 8 years old. Sixty percent of the patients were presented as nephritic-nephrotic syndrome. Based on biopsy results, there was 1 patient (5%) with class I, 5 (25%) with class II, 2 (10%) with class III, 12 (60%) with class IV and none with class V. Eighty five percent of cases went in remission, one was hemodialytic and 2 died due to renal failure and central nervous system involvement. Among 12 cases with class IV, 11 responded to prednisolone and intravenous cyclophosphamide pulses. There was no evidence, short-term or long-term side effects. Conclusion: It seems that intravenous pulse cyclophosphamide induces remission of clinical and renal disease in the majority of early diagnosed children with severe LN. Furthermore, it appears that MMF is an appropriate drug for maintenance therapy. Further studies to confirm the long-term efficacy and safety of cyclophosphamide pulse therapy on larger numbers of patients are prudently needed.