Longitudinal assessment of disease burden in juvenile systemic lupus erythematosus: A multicenter study of activity and damage scores

IntroductionJuvenile Systemic Lupus Erythematosus (jSLE) is a rare pediatric rheumatic disease characterized by systemic inflammation that can lead to organ damage. Compared to adults, it often has a more severe course in children. Both disease activity and treatments may result in temporary or permanent damage.ObjectivesTo evaluate risk factors associated with damage occurrence in patients with jSLE.MethodsThis multicenter, retrospective study included patients with jSLE followed for at least 12 months. Low-dose corticosteroid therapy was defined as prednisolone 0.01-0.03 mg/kg/day (max 7.5 mg/day). The annual cumulative steroid dose was calculated by dividing the total steroid intake by 365.25 times the number of follow-up years. Collected data included SLEDAI and SDI scores at initial and final visits, laboratory parameters, and flare characteristics.ResultsA total of 158 patients (86.7% female) from 17 centers were included. Median age at diagnosis was 13.8 years, with a median follow-up of 35 months. Organ damage was present in 14 patients at diagnosis and in 23 at final visit. Damage types included proteinuria, cognitive dysfunction (each 3.2%), and others such as cataracts, erosive arthritis, avascular necrosis, optic atrophy, and vertebral collapse. Patients with damage had significantly higher SLEDAI scores at both time points, delayed transition to low-dose steroids, and a lower rate of achieving Lupus Low Disease Activity State (LLDAS) (<i>p</i> = .006).ConclusionPersistent disease activity and delayed control are major contributors to organ damage in jSLE. Early and sustained disease suppression is critical to prevent long-term complications.

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Journal
Lupus
Published
2025-09-29
DOI
https://doi.org/10.1177/09612033251386091
Primary Topic
Systemic Lupus Erythematosus Research
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article
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article

Longitudinal assessment of disease burden in juvenile systemic lupus erythematosus: A multicenter study of activity and damage scores

Betül Sözeri, Yasin Karalı, Sıla Atamyıldız Uçar, Hafize Emine Sönmez et al.
Lupus
Systemic Lupus Erythematosus Research
article

Longitudinal assessment of disease burden in juvenile systemic lupus erythematosus: A multicenter study of activity and damage scores

Betül Sözeri, Yasin Karalı, Sıla Atamyıldız Uçar, Hafize Emine Sönmez, Kübra Öztürk, Belde Kasap Demir, Nuray Aktay Ayaz, Tolga Kasap, Mustafa Çakan, Semanur Özdel, Selçuk Yüksel, Görkem Oğuz, Gülşah Kavrul Kayaalp, Hatice Adıgüzel Dundar, Nihal Şahin, Sara Şebnem Kılıç, Figen Çakmak, Gülçin Otar Yener, Hatice Kübra Dursun, Ferhat Demir, Serkan Türkuçar, Dorukcan Alkan, Eray Tunce, Hande Ilgaz Tüzen, Duygu Aydın, Burcu Bozkaya Yücel
article en

Abstract

IntroductionJuvenile Systemic Lupus Erythematosus (jSLE) is a rare pediatric rheumatic disease characterized by systemic inflammation that can lead to organ damage. Compared to adults, it often has a more severe course in children. Both disease activity and treatments may result in temporary or permanent damage.ObjectivesTo evaluate risk factors associated with damage occurrence in patients with jSLE.MethodsThis multicenter, retrospective study included patients with jSLE followed for at least 12 months. Low-dose corticosteroid therapy was defined as prednisolone 0.01-0.03 mg/kg/day (max 7.5 mg/day). The annual cumulative steroid dose was calculated by dividing the total steroid intake by 365.25 times the number of follow-up years. Collected data included SLEDAI and SDI scores at initial and final visits, laboratory parameters, and flare characteristics.ResultsA total of 158 patients (86.7% female) from 17 centers were included. Median age at diagnosis was 13.8 years, with a median follow-up of 35 months. Organ damage was present in 14 patients at diagnosis and in 23 at final visit. Damage types included proteinuria, cognitive dysfunction (each 3.2%), and others such as cataracts, erosive arthritis, avascular necrosis, optic atrophy, and vertebral collapse. Patients with damage had significantly higher SLEDAI scores at both time points, delayed transition to low-dose steroids, and a lower rate of achieving Lupus Low Disease Activity State (LLDAS) (<i>p</i> = .006).ConclusionPersistent disease activity and delayed control are major contributors to organ damage in jSLE. Early and sustained disease suppression is critical to prevent long-term complications.

LupusVol. 34(13)
Çanakkale Onsekiz Mart Üniversitesi (TR), Bursa Uludağ Üni̇versi̇tesi̇ (TR), Izmir University (TR), Izmir Kâtip Çelebi University (TR), Acıbadem University (TR), Dr. Behçet Uz Çocuk Hastalıkları Hastanesi (TR), Eskişehir City Hospital (TR), Memorial Ankara Hospital (TR), Zeynep Kamil Hospital (TR), Ümraniye Eğitim ve Araştırma Hastanesi (TR), Samsun University (TR), Sağlık Bilimleri Üniversitesi (TR), Herttoniemi Hospital (FI), Istanbul Memorial Hospital (TR), Izmir Bozyaka Eğitim ve Araştırma Hastanesi (TR), Hampshire Constabulary (GB), İstanbul Başakşehir Çam ve Sakura Şehir Hastanesi, Pamukkale University (TR), Kocaeli Üniversitesi (TR), Istanbul Medeniyet University (TR), Istanbul University (TR), University of Health Sciences Antigua (AG)
Openalex Percentile: Top 61%
Systemic Lupus Erythematosus Research
DOISourceOpenAlex
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