Intravenous cyclophosphamide combined with steroids in pediatric onset severe lupus nephritis

Prayong Vachvanichsanong,Edward Mcneil,Pornsak Dissaneewate

doi:10.1007/s11255-012-0331-9

Prayong Vachvanichsanong, Edward Mcneil + Show 1 more

Open Access

https://doi.org/10.1007/s11255-012-0331-9

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Abstract

BackgroundIntravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN) for many years. Because of the wide variety of manifestations of the condition and the long-term nature of the disease, outcomes vary widely.ObjectiveTo evaluate and compare the immediate and long-term results of IVCY in pediatric onset severe LN and between patients with normal and abnormal initial renal function.MethodsPatients aged <18 years who attended the Department of Pediatrics, Prince of Songkla University, diagnosed with severe LN, and who were given a 36-month IVCY course, were included. Comparison of overall survival between the two groups was assessed using Kaplan–Meier survival curves.Results108 patients with a mean age of 12.6 ± 2.7 years were studied, with a mean follow-up time of 5.7 ± 4.3 years. 48 patients completed the IVCY course. 36 patients had abnormal renal function and 72 patients had normal renal function at the start of therapy. Both groups responded well initially to treatment; proteinuria reduced to normal levels after 1 and 2 treatments in the normal and abnormal groups, respectively, while creatinine clearance returned to normal levels after 8 treatments in the abnormal group. Overall survival was not different between the two groups; however, the abnormal renal function group had a higher crude mortality rate than the normal group (13/36 vs 10/72, p value = 0.02). At the time of analysis, some patients who had completed their IVCY course still required other therapy to control their disease activity.ConclusionThree years of IVCY treatment provided similar outcomes in both normal and abnormal renal function groups. Immediate outcomes were favorable but long-term remission was not promising.

Highlights

Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN) for many years
Initial studies of IVCY therapy in severe lupus nephritis showed quite good outcomes in both adults and children; many subsequent studies with longer patient follow-up found that their results were not as good as the initial studies had promised with many patients relapsing and developing unfavorable, long-term side effects [9,10,11,12,13]
The objectives of this study were to evaluate and compare the outcomes of IVCY treatment in childhood onset severe lupus nephritis in a large group of Thai children and between patients with normal and abnormal initial renal function. This retrospective study examined the medical records of patients diagnosed with Systemic lupus erythematosus (SLE) based on the American Rheumatism Association 1982 revised criteria [15], with LN WHO class IV or who had severe glomerulonephritis, defined by rising serum creatinine or heavy proteinuria, who had not responded to corticosteroid therapy or corticosteroids combined with oral cyclophosphamide

Summary

Introduction

Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN) for many years. Intravenous cyclophosphamide (IVCY) is the recommended treatment in severe lupus nephritis. Initial studies of IVCY therapy in severe lupus nephritis showed quite good outcomes in both adults and children; many subsequent studies with longer patient follow-up found that their results were not as good as the initial studies had promised with many patients relapsing and developing unfavorable, long-term side effects [9,10,11,12,13]. A preliminary report from our institution involving 16 children treated with IVCY showed satisfactory outcomes with minimal complications; the duration of remission was not evaluated due to the short follow-up period of that study [14]

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