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Rheumatology (Oxford, England) · 2026

Organ damage profiles and associated factors in juvenile versus adult-onset SLE: a matched analysis from the Japanese ANSWER-SLE cohort.

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Kachi Shion, Tsuji Hideaki, Onishi Akira, Ishimura Kaori, Miyake Hirofumi, Esa Natsuko, Hiramatsu Yuri, Katsushima Masao, Oka Hideki, Iwata Shigeru, Yamamoto Wataru, Ohmura Koichiro, Fujii Takao, Nozaki Yuji, Hashimoto Motomu, Morinobu Akio

Аннотация

OBJECTIVES: To elucidate differences in organ damage in patients with systemic lupus erythematosus (SLE) between juvenile-onset (<18 years) and adult-onset (≥18 years) using the Systemic Lupus International Collaborating Clinics Damage Index (SDI).

METHODS: Among patients in the multicenter registry, ANSWER-SLE (2019-2023), total-SDI and organ domains of SDI were compared between juvenile- and adult-onset patients after 1:2 matching based on sex and current age. Factors associated with total SDI and organ domains of SDI were analyzed by regression models in the total population.

RESULTS: A total of 615 patients were included. Among them, 83 juvenile- and 166 adult-onset patients were matched. Juvenile-onset showed higher total-SDI (juvenile-onset vs. adult-onset, 2.17 vs. 1.16) and higher damage in the ocular, nervous system, renal, cardiovascular, and musculoskeletal domains than adult-onset. In the multivariable negative binomial regression analysis of the total cohort, younger age at onset (incidence rate ratio [IRR] 0.99, p=0.005) and older current age (IRR 1.03, p<0.001) were independently associated with higher total SDI scores. These associations persisted in the matched cohort (IRR 0.96 and IRR 1.03, respectively). In multivariable analyses of organ-specific damage adjusted for sex, current age, and age at onset, younger age at onset was associated with a higher SDI in the cataract, angina/coronary artery bypass, valvular disease, avascular necrosis, skin ulceration, and premature gonadal failure in the total cohort, and additionally with renal domain in the matched cohort.

CONCLUSIONS: Juvenile-onset SLE is associated with organ damage compared to adult-onset SLE, with prominent ocular, neurological, renal, cardiovascular, and musculoskeletal systems.