Çocuklarda sistemik lupus eritematozusun klinik özellikleri ve uzun dönem sonuçları

Year 2021, Volume: 9 Issue: 2, 78 - 83, 31.08.2021

Elif Çelikel , Zahide Ekici Tekin , Fatma Aydın , Tuba Kurt , Melike Kaplan , Cüneyt Karagöl , Müge Sezer , Nilüfer Tekgöz , Serkan Coşkun , Banu Acar , Nilgün Çakar

https://doi.org/10.21765/pprjournal.982781

Abstract

Amaç: Sistemik lupus eritematozus (SLE), otoantikorların varlığı ve çoklu organ sistemi tutulumu ile karakterize, yaygın bir multisistemik otoimmün hastalıktır. Bu çalışmanın amacı, çocuklarda SLE'nin klinik belirtilerini, laboratuvar bulguları, klinik seyrini ve prognozunu tanımlamaktır.

Yöntemler: 18 yaşından önce tanı almış SLE'li hastaları retrospektif olarak incelendi.. Klinik ve laboratuvar verileri, ilk kabulden çalışmanın başlamasına kadar geçen sürede hastaların klinik ve laboratuvar verileri kaydedildi.

Bulgular: Otuz beş çocuk ve adölesan SLE (cSLE) hastanın %85.7'si kadındı. Ortalama hastalık başlangıç ​​yaşı 12 (4-17 arası) yıl ve medyan takip süresi 5 (1-14) yıldı. En sık görülen klinik özellik artrit (%65.1) idi, bunu konstitusyonel semptomlar (%48.6), malar döküntü (%31.4), ışığa duyarlılık (%5.7), alopesi (%5.7) ve oral ülserler (%5.7) izliyordu. Böbrek tutulumu hastaların 4/5'ini (%80) oluşturuyordu. Hematüri ve proteinüri en sık başvuru bulgusuydu (sırasıyla %48.5 ve %45.7). 27 hastaya böbrek biyopsisi yapıldı. WHO sınıflamasına göre: 1 hastada sınıf V, 10 hastada sınıf IV, 4 hastada sınıf III ve 12 hastada sınıf II nefrit vardı. Çocukların %20'sinde nörolojik semptomlar gelişti. Takip süresi boyunca bir hasta öldü. Son takipte hiçbir hastada böbrek yetmezliği gelişmedi, ancak 4'ünde (%11.4) proteinüri devam etti. Hastaların sekel dışında tüm nörolojik bulguları remisyondadır.

Sonuç: Hastalarımızda klinik sonuç olumluydu. Böbrek tutulumu yaygındır, ancak en azından kısa vadede son dönem böbrek hastalığına ilerleme nadirdir.

Keywords

Çocuklar, İlk başvuru, Prognoz, Sistemik lupus eritematozus

Supporting Institution

Yok

Project Number

Yok

Clinical characteristics and long-term outcomes of systemic lupus erythematosus in children

Abstract

Background: Systemic lupus erythematosus (SLE) is a common multisystemic autoimmune disease characterized by the presence of autoantibodies and multiorgan system involvement. The aim of this study was to describe the presenting clinical manifestations, laboratuary findings, clinical course and prognosis of SLE in children.

Methods: We performed a retrospective study patients with SLE, diagnosed before the age of 18 years. Clinical and laboratory data were collected from initial admission to study initiation.

Results: Thirty-five children and adolescents SLE (cSLE) were recorded, 85.7% of female. The median age at disease onset was 12(range 4-17) years, and median follow up duration was 5 (1-14) years. The most common clinical feature was arthritis (65.1%), followed by constitutional symptoms (48.6%), malar rash (31.4%), photosensitivity (5.7%), alopecia (5.7%) and oral ulcers (5.7%). Renal involvement accounted 4/5 of the patients (80%). Hematuria and proteinuria were the most frequent presenting findings (48.5% and 45.7% respectively). Renal biopsy was performed in 27 patients. According to WHO classification: 1 patient had classV nephritis, 10 had class IV, 4 had class III and 12 had class II nephritis. 20% of children developed neurologic symptoms. One patient died during the follow-up period. At the last follow up none of the patients had renal failure but, proteinuria persisted in 4 of them (11.4%). All neurological findings of patients are in remission apart from sequelae.

Conclusion: Clinical outcome was favorable in our patients. Renal involvement is common but progression to end stage kidney disease, at least in the short term, is rare.

Keywords

Children, Initial presentation, Prognosis, systemic lupus erythematosus

Project Number

Yok

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