Do Hyunsue, Yoon Taejun, Park Yong-Beom, Ha Jang Woo, Ahn Sung Soo, Lee Sang-Won
Department of Internal Medicine, Division of Rheumatology, Kangwon National University School of Medicine, Chuncheon, Gangwon-do, Republic of Korea.
Department of Medical Science, BK21 Plus Project, Yonsei University, College of Medicine, Seoul, Republic of Korea.
Medicine (Baltimore). 2025 Jan 31;104(5):e41271. doi: 10.1097/MD.0000000000041271.
The aim was to investigate the ability of serum sulfatide levels at diagnosis to reflect the cross-sectional activity and further longitudinally predict the occurrence of end-stage kidney disease (ESKD) during the follow-up period in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), regardless of kidney involvement at diagnosis. This study included 67 patients first diagnosed with AAV with available clinical data, including Birmingham Vasculitis Activity Score (BVAS), erythrocyte sedimentation rate, C-reactive protein, and blood samples at diagnosis. Serum sulfatide levels were assessed using stored serum samples at the time of diagnosis. The median age of the 67 patients (40.3% men and 59.7% women) was 61.0 years. During follow-up, 10 (14.9%) patients progressed to ESKD, and 4 (6.0%) died. Serum sulfatide levels significantly correlated with Five-Factor Score (r = -0.242), erythrocyte sedimentation rate (r = -0.315), and renal manifestation of the BVAS items (r = -0.296), but not BVAS at diagnosis. The cutoff of serum sulfatide levels at diagnosis for ESKD progression was 332.5 pg/mL. However, no significant cutoff of serum sulfatide levels for all-cause mortality was obtained. Patients with serum sulfatide levels ≤ 332.5 pg/mL at diagnosis exhibited both significantly higher frequency of ESKD progression (22.7% vs 0%, P = .012) and lower ESKD-free survival rate than those without (P = .011). This study highlighted the clinical usefulness of measuring serum sulfatide levels at the time of diagnosis as a biomarker to predict ESKD progression in patients with AAV regardless of kidney involvement at diagnosis.
目的是研究抗中性粒细胞胞浆抗体相关性血管炎(AAV)患者诊断时血清硫苷脂水平反映横断面活动度以及进一步纵向预测随访期间终末期肾病(ESKD)发生的能力,无论诊断时是否存在肾脏受累情况。本研究纳入了67例首次诊断为AAV且有可用临床数据的患者,包括伯明翰血管炎活动评分(BVAS)、红细胞沉降率、C反应蛋白以及诊断时的血样。使用诊断时储存的血清样本评估血清硫苷脂水平。67例患者(男性占40.3%,女性占59.7%)的中位年龄为61.0岁。随访期间,10例(14.9%)患者进展为ESKD,4例(6.0%)死亡。血清硫苷脂水平与五因素评分(r = -0.242)、红细胞沉降率(r = -0.315)以及BVAS项目的肾脏表现(r = -0.296)显著相关,但与诊断时的BVAS无关。诊断时ESKD进展的血清硫苷脂水平临界值为332.5 pg/mL。然而,未获得全因死亡率的血清硫苷脂水平显著临界值。诊断时血清硫苷脂水平≤332.5 pg/mL的患者ESKD进展频率显著更高(22.7%对0%,P = 0.012),且无ESKD生存率低于无该情况的患者(P = 0.011)。本研究强调了在诊断时测量血清硫苷脂水平作为预测AAV患者ESKD进展的生物标志物的临床实用性,无论诊断时是否存在肾脏受累情况。