Özet
Amaç
Üçüncü basamak bir pediatrik romatoloji merkezinde çocukluk çağı başlangıçlı sistemik lupus eritematozus (çSLE) hastalarının klinik spektrumunu, organ tutulum paternini, tedavi stratejilerini ve hastalık şiddetini karakterize etmektir.
Yöntem
Bu retrospektif çalışmaya, Sistemik Lupus Uluslararası İşbirliği Klinikleri kriterlerine göre çSLE tanısı almış ve Ağustos 2019 ile Eylül 2025 arasında takip edilen 18 yaş altı hastalar dahil edildi. Hastalık aktivitesi Sistemik Lupus Eritematozus Hastalık Aktivite İndeksi 2000 (SLEDAI-2K) ile değerlendirildi ve lupus nefriti Uluslararası Nefroloji Derneği/Böbrek Patolojisi Derneği kriterlerine göre sınıflandırıldı.
Bulgular
Tanı anındaki ortanca yaş 13 yıldı [çeyreklik aralık (IQR): 11-15]. Mukokutanöz bulgular baskındı ve 29 hastada (%54,7) akut veya subakut kutanöz lupus döküntüsü mevcuttu. Diğer yaygın özellikler arasında eroziv olmayan artrit (%43,4, n=23), oral ülserler (%13,2, n=7) ve alopesi (%11,3, n=6) yer aldı. Takip sırasında 31 hastada (%58,5) böbrek tutulumu meydana geldi. Yirmi beş hastaya (kohortun %47,1’i) böbrek biyopsisi yapıldı ve tüm olgularda histopatolojik olarak lupus nefriti doğrulandı. Biyopsi yapılan hastalar arasındaki dağılım şu şekildeydi: 11’inde Sınıf II (%20,7), 11’inde Sınıf IV (%20,7), 2’sinde Sınıf I (%3,7) ve 1’inde Sınıf V (%1,8). Tüm hastalara (%100) hidroksiklorokin verildi. Elli hastaya (%91,3) sistemik kortikosteroidler uygulandı. Mikofenolat mofetil en sık kullanılan immünosüpresif ajandı (%43,4, n=23), bunu siklofosfamid (%41,5, n=22) izledi. Hastalık aktivitesi başlangıçta yüksekti (ortanca SLEDAI-2K skoru: 16, IQR: 8-20), ancak son değerlendirmede önemli bir iyileşme gösterdi (ortanca SLEDAI-2K: 1,5, IQR: 0-4; p<0,001).
Sonuç
Bu çalışma, sık görülen mukokutanöz ve kas-iskelet sistemi tutulumu ve belirgin renal ve nörolojik bulgularla birlikte çSLE’nin heterojen bir tablo sergilediğini vurgulamaktadır. Olumlu sonuçlar için erken tanı ve uygun tedavi esastır.
Anahtar Kelimeler:
Çocukluk çağı, hastalık şiddeti, lupus eritematozus, organ tutulumu
Kaynaklar
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