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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2009  |  Volume : 20  |  Issue : 3  |  Page : 424-428
Prognosis and predictors of convulsion among pediatric lupus nephritis patients

1 Nephrology and Urology Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran
2 Nephrology Unit of Medical Pediatric Center of Tehran Medical University, Tehran, Iran
3 Nephrology and Urology Research Center, Baqiyatallah University of Medical Sciences, Tehran; Dr Taheri Medical Research Group, Tehran, Iran
4 Neurology Department, Tabriz University of Medical Sciences, Tabriz, Iran
5 Nephrology and Urology Research Center, Baqiyatallah University of Medical Sciences, Tehran; Research Center for Chemical Injuries, Tehran, Iran

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In this study, we aimed to analyze features and outcome of convulsion in pediatric lupus nephritis patients. We retrospectively reviewed data of 14 Iranian children with lupus nephritis who developed seizures and compared them with a group of the same number of well matched pe­diatric lupus nephritis patients. Higher serum creatinine levels and higher frequencies of anemia and lymphopenia were observed in the convulsion group. Multivariable logistic regression analysis re­vealed that the only risk factor for development of convulsion in pediatric lupus patients with ne­phritis was lymphopenia. Survival analysis showed that convulsion had no impact on patient and renal function outcomes in our pediatric lupus nephritis subjects. In conclusion, we found that lympho­penia is a predictive factor for convulsion occurrence in our patients and special attention to neuro­logical status assessment may be needed in this situation.

Keywords: Pediatric, Childhood, SLE, Lupus nephritis, Convulsion, Neurological manifestations

How to cite this article:
Beiraghdar F, Maddani A, Taheri S, Sharifi-Bonab MM, Esfahani T, Panahi Y, Einollahi B. Prognosis and predictors of convulsion among pediatric lupus nephritis patients. Saudi J Kidney Dis Transpl 2009;20:424-8

How to cite this URL:
Beiraghdar F, Maddani A, Taheri S, Sharifi-Bonab MM, Esfahani T, Panahi Y, Einollahi B. Prognosis and predictors of convulsion among pediatric lupus nephritis patients. Saudi J Kidney Dis Transpl [serial online] 2009 [cited 2021 May 12];20:424-8. Available from: https://www.sjkdt.org/text.asp?2009/20/3/424/50773

   Introduction Top

Systemic lupus erythematosus (SLE) is a multi­organ disorder of autoimmune origin with sig­nificant morbidity and mortality. [1],[2] Approxi­mately 25% of all cases of SLE occur in the first two decades of life. [3] Childhood onset SLE has variable clinical manifestations and an un­predictable natural history and frequently have more severe disease at presentation and worse outcomes. [4],[5],[6]

Even with current improved survival of lupus patients, complications from disease process or from its concomitant therapy currently contri­bute to the major causes of morbidity and mor­tality in these patients. [7] Poor prognosis is asso­ciated with diffuse proliferative (WHO class IV) nephritis and persistent central nervous system disease. [8] In this study, we retrospectively ana­lyzed our data to find potential factors which can predict occurrence of seizures in the manage­ment process of pediatric patients with lupus nephritis.

   Materials and Methods Top


In this single center report, we retrospectively reviewed data of 14 Iranian children with lupus nephritis who developed seizures during their follow up at Pediatric Nephrology Department of Tehran University of Medical Sciences during the period from 1985 to 2005. The diagnosis of systemic lupus erythematosus met the criteria adopted by the American Rheumatism Asso­ciation (ARA). [9] To characterize disease activity, we used the criteria of SLEDAI (Systemic Lupus Erythematosus Disease Activity Index). [10]

Unfortunately, because of the retrospective na­ture of the study, we were unable to retrieve data about the features and severity of seizures in these patients. In all cases, however seizures were not life threatening or associated with perma­nent complications.

We extracted the following data from our data registry: age, gender, creatinine at diagnosis, pathological class of nephritis, CH50 comple­ment percentages, activity index, chronicity index, having malar rash, having oral ulcers, musculo­skeletal involvement, pericarditis, hematuria, nephrotic syndrome, anemia, lymphopenia, hy­pertension, treatment modalities, end stage re­nal disease requiring renal replacement therapy and death.

Treatment strategies

In our institution, the initial therapy was orally administered corticosteroids (CS) prescribed at a dose of 2 mg/kg per day to all patients, with a daily maximum of 60 mg/day; the decision to add an immunosuppressant depended on the in­dividual patient's clinical condition. Additional therapy with immunosuppressants including in­travenous cyclophosphamide (CP), azathioprine (AZA) and mycophenolate mofetil (MMF) was also included in the induction regimen in patients with worse clinical course. Hydroxychloroquine was used regarding skin manifestations and com­plications.

Maintenance treatment varied for individual patients and was adjusted according to the clini­cal course of each patient. AZA was used for patients presenting before 2001 and MMF after 2001.

Statistical analysis

Software SPSS v. 13.0 (SPSS corp. IL, USA) was used for all data analyses. Paired t test and non parametric χ2 and Mann-Whitney U tests were used where appropriate. Multivariate logistic regression was used for analyzing independent effects of individual variables on occurrence of convulsion. Kaplan Meier method was used for survival analysis. P< 0.05 was considered signi­ficant.

   Results Top

Of overall 60 pediatric lupus nephritis patients diagnosed and followed at our clinic, 14 (23%) were found to have convulsions during their follow up period. Of these, 11 (79%) were female and 3 (21%) were male. Mean age was 11.1 ± 2.2 years. 12 (86%) patients had class IV; 1 (7%) had class III and another patient had class V lupus nephritis on renal biopsy. All patients had histories for hematuria and proteinuria. 6 (43%) had malar rash, 3 (21%) had oral ulcer, 10 (71%) arthritis, 7 (50%) pericarditis, 10 (71%) nephrotic syndrome, 13 (93%) anemia, 12 (86%) lympho­penia, and 10 (71%) hypertension. Mean time period from initiation of disease symptoms to documentation of nephritis was 2.6 (1-9) months.

[Table 1] shows differences in patients with and without convulsion. Anemia, lymphopenia and higher mean initial serum creatinine level was significantly more frequently observed in patients presenting with seizures.

Independent impact of potential risk factors for development of seizures in our study sub­jects was evaluated by multivariable logistic re­gression analysis revealing lymphopenia as the only risk factor [Table 2].

Most patients received a combination therapy with cyclosporine and or cyclophosphamide. Because of limited sample size and variant types of treatments, meaningful analysis was not possible in our cases and controls. One patient among cases and three from the controls died, survival analysis was not different among the two groups [Figure 1] and [Figure 2].

   Discussion Top

Neurological involvement in SLE has been recognized since Olser's description of the di­sease. [11] Similar to the earlier reports 23% of our patients had nervous system involvement. [12] In this study, we observed that pediatric lupus ne­phritis patients with evidence of CNS involve­ment had no different renal and patient out­comes. This finding is in contrast to the previous studies which proposed neurological manifes­tation as a poor prognostic factor for patients with SLE. [13],[14] It was also reported that neuro­logical disease has a tendency to develop with more severe renal disease. [13] All of our patients had lupus nephritis and this association there­fore cannot be evaluated in our study.

Because of the small sample size it was not possible to have any meaningful analysis of the treatment regimens, nevertheless most of the patients had received cyclophosphamide and or cyclosporine. Although in Bivariate analysis, higher creatinine levels at entrance, having ane­mia and lymphopenia were detected as potential risk factors for convulsion, multivariate logistic analysis after adjustment for other potential risk factors revealed lymphopenia as the only risk factor for development of convulsion in pedia­tric lupus nephritis patients [Table 2]. This fin­ding is in agreement with previous reports de­monstrating lymphopenia as a predictive factor for occurrence of convulsion in SLE patients. [13]

In conclusion, our study, similar to previous reports lymphopenia was the only predictive factor for convulsion in pediatric lupus nephritis patients. Future studies with larger sample size seem necessary for confirming our findings.

   References Top

1.Klein-Gitelman M, Reiff A, Silverman ED. Sys­temic lupus erythematosus in childhood. Rheum Dis Clin North Am 2002;28(3):561-77, vi-vii. Review.  Back to cited text no. 1    
2.Ardoin SP, Schanberg LE. The management of pediatric systemic lupus erythematosus. Nat Clin Pract Rheumatol 2005;1:82-92.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.King KK, Kornreich HK, Bernstein BH, et al. The clinical spectrum of systemic lupus erythe­matosus in childhood. Arthritis Rheum 1977;20 (2 Suppl):287-94.  Back to cited text no. 3    
4.Meislin AG, Rothfield N. Systemic lupus ery­thematosus in childhood. Analysis of 42 cases, with comparative data on 200 adult cases follo­wed concurrently. Pediatrics 1968;42:37-49.  Back to cited text no. 4    
5.Tucker LB, Menon S, Schaller JG, et al. Adult­and childhood-onset systemic lupus erythema­tosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol 1995; 34:866-72.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Font J, Cervera R, Espinosa G, et al. Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteris­tics in adults. Ann Rheum Dis 1998; 57:456-9.  Back to cited text no. 6    
7.Bongu A, Chang E, Ramsey Goldman R. Can morbidity and mortality of SLE be improved? Best Pract Res Clin Rheumatol 2002;16:313-32.  Back to cited text no. 7    
8.Supavekin S, Chatchomchuan W, Pattaragarn A, et al. Pediatric systemic lupus erythematosus in Siriraj Hospital. J Med Assoc Thai 2005;88 Suppl 8:S115-23.  Back to cited text no. 8  [PUBMED]  
9.Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25: 1271-7.  Back to cited text no. 9  [PUBMED]  
10.Liang MH, Socher SA, Larson MG, et al. Reliability and validity of six systems for the clinical assessment of disease activity in SLE. Arthritis Rheum 1989;32:1107-18.  Back to cited text no. 10  [PUBMED]  
11.Osler W. Classics: On the visceral complica­tions of erythema exudativum multi-forme. Am J Med Sci 1976;271(1):106-17.  Back to cited text no. 11    
12.Benseler SM, Silverman ED. Neuropsychiatric involvement in pediatric systemic lupus erythe­matosus. Lupus 2007;16(8):564-71.  Back to cited text no. 12    
13.Gibson T, Myers AR. Nervous system involve­ment in systemic lupus erythematosus. Ann Rheum Dis 1975;35(5):398-406.  Back to cited text no. 13    
14.Estes D, Christian CL. The natural history of sys­temic lupus erythematosus by prospective ana­lysis. Medicine (Baltimore) 1971;50(2):85-95.  Back to cited text no. 14    

Correspondence Address:
Yunes Panahi
Research Center for Chemical Injuries, Baqiyatallah Hospital, Mollasadra St. Tehran
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PMID: 19414945

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