Lupus Nefritli Çocuklarda İntravenöz Siklofosfamid Tedavisinin Etkinliğinin Değerlendirilmesi

Yıl 2017, , 77 - 83, 01.04.2017

Işık Odaman Al Sevinç Emre Ilmay Bilge Alev Yılmaz Bağdagül Yavaş Aksu

https://doi.org/10.5222/j.child.2017.077

Öz

Amaç: Sistemik lupus eritematozus seyrinde renal tutulum önemli morbidite ve mortalite nedenidir. Lupus nefriti tedavisinde siklofosfamid yaygın olarak kullanılmaktadır. Bu çalışmadaki amacımız, çocuklardaki lupus nefritinde intravenöz siklofosfamid tedavisinin etkinliğini değerlendirmektir.Gereç ve Yöntem: İstanbul Tıp Fakültesi Çocuk Nefrolojisi Bilim Dalı tarafından 1991-2011 yılları arasında intravenöz siklofosfamid tedavisi alan 28 lupus nefritli hasta retrospektif olarak değerlendirildi. Tüm hastalarda öykü, başvuru sırasındaki bulgu ve belirtiler, laboratuvar tetkikleri, renal biyopsi sonuçları, tedavi ve prognoza ait veriler kaydedildi.Bulgular: Başvuru sırasında, 27 hastada %96.5 proteinüri, 4 hastada %14.3 makroskopik hematüri, 15 hastada %53.5 hipertansiyon, 11 hastada %39.2 ise böbrek yetersizliği saptandı. En sık saptanan ekstrarenal klinik bulgular artrit/artralji %67.9 , konstitüsyonel bulgular %64.3 ve cilt bulguları %60.7 idi. Hastalarımızda histopatolojik bulgu olarak en sık Class 4 %53.5 , daha sonra sırasıyla Class 2 %21.6 , Class 3 %10.7 ve Class 5 %3.5 lupus nefriti saptandı. Çalışma grubunda toplam 62.7±49 aylık 6-204 ay izlem boyunca 21 hastada %75 renal remisyon sağlandı. On dokuz hasta %67.8 hem renal hem de ekstrarenal bulgular açısından remisyonda idi. Son dönem böbrek yetersizliği gelişme oranı %14.2 ve mortalite oranı %3.5 olarak belirlendi. Lupus nefriti vakalarında SDBY ve ölüm başlangıçtaki serum kreatinin yüksekliği ile istatistiksel olarak ilişkili bulundu p: 0.047 . Çalışma grubundaki hastaların %39.2’sinde intravenöz siklofosfamide bağlı yan etki görülürken, en sık görülen yan etki nötropeni %21.4 idi.Sonuç: Bu çalışmamızda, pediyatrik lupus nefriti vakalarında intravenöz siklofosfamid tedavisinin minimal yan etki ile renal ve ekstrarenal remisyon sağlanmasında etkin ve güvenilir olduğu gösterilmiştir. Başlangıçtaki serum kreatinin yüksekliği kötü prognostik faktör olarak bulunmuştur

Anahtar Kelimeler

Sistemik lupus eritematozus, çocuk, siklofosfamid, lupus nefriti

Evaluation the Effectiveness of Intravenous Cyclophosphamide Treatment in Children with Lupus Nephritis

Öz

Objective: Renal involvement in systemic lupus erythematosus is an important cause of morbidity and mortality. Cyclophosphamide is widely used in the treatment of lupus nephritis. The aim of this study is to evaluate the effectiveness of intravenous cyclophosphamide treatment in children with lupus nephritis.Material and Method: In this study, 28 patients with lupus nephritis treated by intravenous pulse cyclophosphamide between the years 1999, and 2011 in Istanbul Faculty of Medicine, Department of Pediatric Nephrology were investigated retrospectively. Clinical history, presenting symptoms and findings, laboratory analysis, results of renal biopsy, treatment methods and prognosis were evaluated.Results: Proteinuria was determined in 27 96.5% , macroscopic hematuria in 4 14.3% , hypertension in 15 53.5% , and renal failure in 11 patients 39.2% as initial presentations. The most frequent extrarenal clinical findings were arthritis/arthralgia 67.9% , constitutional 64.3% and skin findings 60.7% . The most frequent histopathological finding of lupus nephritis patients in our study group was Class 4 lupus nephritis 53.5% , followed by Class 2 21,6% , Class 3 10,7% and Class 5 3.5% nephritis respectively. Renal remission was obtained in 21 75% patients throughout 62.7±49 months of follow up period in study group. Nineteen patients 67.8% were in remission in terms of both renal and extrarenal findings. Morbidity ratio ESRF was 14.2%, and mortality rate was 3.5% in our study group. ESRF and death in the study group were statistically related to increased serum creatinine levels during the initial presentation p: 0.047 . Side effects of intravenous cyclophosphamide were detected in 39.2% of the patients, while most frequent side effect was neutropenia 21.4% . Conclusion: In this study, intravenous cyclophosphamide treatment was determined as effective and reliable with minimum side effects in order to obtain renal and extrarenal remission in patients with pediatric lupus nephritis. The presence of high serum creatinine during the initial presentation was found as a poor prognostic factor

Anahtar Kelimeler

Systemic lupus erythematosus, child, cyclophosphamide, lupus nephritis

Kaynakça

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