Sibel BALCI, Rabia Miray KIŞLA EKİNCİ, Ferhat Can PİŞKİN, Engin MELEK, Bahriye ATMIŞ, Dilek DOĞRUEL, Derya Ufuk ALTINTAŞ, Aysun KARABAY BAYAZIT

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Amaç: Avasküler nekrozis (AVN), çocukluk çağı başlangıçlı sistemik lupus eritematozusun (jSLE) yıkıcı bir komplikasyonudur. Tek merkezden yapılan bu çalışmanın amacı jSLE hastalarında AVN sıklığını ve bu hastaların klinik özelliklerini belirlemektir. Gereç ve Yöntem: Amerikan Romatoloji Koleji’nin sınıflama kriterlerine göre tanı almış 58 jSLE hastası bu geriye dönük çalışmaya dahil edildi. jSLE hastalarının hastalık aktiviteleri SLE hastalık aktivite indeksi baz alınarak (SLEDAI-2K) ölçüldü. Organ hasarı Amerikan Romatoloji Koleji’nin SLE hastalarında uyguladığı hasar indeksinin çocukluklardaki versiyonu kullanılarak son muayenede belirlendi. Bulgular: Elli sekiz jSLE hastasının %86,2 (50)’si kız hastaydı. Ortalama bazal SLEDAI-2K skoru 21,47±8,96 idi. En az bir organda hasarı olan hasta sayısı 24 (%41,4) idi, bunlardan 5 tanesinde (%8,6) AVN vardı. AVN olan ve olmayan hastalar arasında çeşitli değikenlere göre istatistiksel anlamlı bir farklılık saptanmadı. Sadece bazal kompleman 3 (C3) düzeyi AVN hastalarında, AVN olmayan hastalara göre anlamlı olarak düşüktü. Sonuç: Bazal düşük C3 düzeyi jSLE hastalarında AVN gelişimini tahmin etmede ön görücü olabilir. jSLE hastalarında AVN gelişimi için muhtemel risk faktörlerini inceleyen çok merkezli çalışmalara ihtiyaç vardır.

Anahtar Kelimeler:

Çocukluk çağı başlangıçlı sistemik lupus eritematozus, avasküler nekrozis, kompleman 3, metilprednizolon

The frequency of avascular necrosis in juvenile systemic lupus erythematosus

Purpose: Avascular necrosis (AVN) is a debilitating complication of juvenile systemic lupus erythematosus (jSLE). The aim of this study was to evaluate the frequency and clinical characteristics of patients with AVN in jSLE from a single center. Material and Methods: Fifty-eight jSLE patients diagnosed according to the American College of Rheumatology classification criteria were included in this retrospective study. Disease activity of jSLE patients was measured by SLE Disease Activity Index-2K (SLEDAI-2K), organ damage was determined by the pediatric version of the systemic lupus international collaborating clinics/American College of Rheumatology damage index (pedSDI) at last visit. Results: Among 58 jSLE patients, the female patients accounted for 86.2% (n=50). Mean baseline SLEDAI-2K score was 21.47±8.96. The number of patients with at least one damage item of pedSDI was 24 (41.4%), in which 5 patients (8.6%) had AVN. There were no statistical differences between the groups except baseline complement 3 (C3) level was significantly lower in patients with AVN. Conclusion: Baseline low C3 level might be a predictor for AVN development in jSLE patients.There is unmet need for multicenter studies investigating possible risk factors of AVN in jSLE patients.

Keywords:

Juvenile systemic lupus erythematosus, avascular necrosis, complement 3, methylprednisolone,

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