The prediction of haemophilic arthropathy progression based on MRI findings and clinical characteristics.

IF 3.4 2区 医学 Q2 GENETICS & HEREDITY
Lu Zhang, Jinxia Guo, Shufang Wei, Jiajia Li, Yincong Dou, Tianming Cheng, Yinghui Ge, Tuo Zhang
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Abstract

Objective: To identify magnetic resonance imaging (MRI) and clinical characteristics that are closely associated with the progression of haemophilic arthropathy (HA) after different therapies and to establish a prediction model for HA progression using Cox proportional hazards regression, thus facilitating the development of personalized clinical replacement therapy plans.

Materials and methods: Retrospective clinical and imaging data were collected from HA patients registered at the Henan Provincial Registration Management Center of Haemophilia from December 2010 to May 2023. The inclusion criteria were joints with a history of haemorrhage and initial/posttreatment reevaluation with X-ray and MRI. Joints with severe damage (i.e., a Pettersson score > 6) were excluded. Joint disease progression was defined as a > 1-point increase in the Pettersson score. Progression-free survival (PFS) was the primary outcome. MRI observations revealed joint effusion, synovial hypertrophy, haemosiderin deposition, bone destruction or cystic degeneration at the joint margins, and cartilage destruction. Age, body mass index (BMI), factor VIII (FVIII) activity, activated partial thromboplastin time (APTT), prothrombin time (PT), therapy type, annual joint bleeding rate (AJBR), and the Haemophilia Joint Health Score (HJHS) were also assessed. Subsequently, univariate and multivariate Cox proportional hazards regression models were employed to analyse the clinical and imaging characteristics influencing HA progression. Factors with a P < 0.15 in univariate analysis were subsequently included in the multivariate analysis. The impact of various imaging and clinical characteristics on PFS was assessed via Kaplan‒Meier (K-M) survival curves.

Results: This study included 98 joints across 65 patients. During the follow-up period, 63 joints exhibited progression. Both univariate and multivariate Cox analyses revealed that MRI-detected synovial hypertrophy (MRI-SP) was an independent risk factor for HA progression. Incorporating BMI into the model improved its predictive performance (Model 1: c-index = 0.671, P < 0.01). Spearman's correlation analysis revealed strong correlations between baseline MRI-SP and detected haemosiderin deposition (r = 0.73) as well as AJBRs (r = 0.66). K-M survival curves indicated that patients receiving prophylactic treatment and those with less severe MRI-SP had better progression-free survival.

Conclusion: MRI-detected synovial hypertrophy is an independent risk factor for HA progression. The predictive model, which includes BMI as a covariate for assessing the risk of HA progression, can serve as an auxiliary tool for developing personalized treatment plans for HA patients.

基于MRI表现和临床特征的血友病关节病进展的预测。
目的:识别不同治疗后与血友病性关节病(HA)进展密切相关的磁共振成像(MRI)及临床特征,利用Cox比例风险回归建立HA进展预测模型,为制定个性化的临床替代治疗方案提供依据。材料和方法:回顾性收集2010年12月至2023年5月在河南省血友病登记管理中心登记的HA患者的临床和影像学资料。纳入标准为有出血史的关节,并通过x线和MRI进行初始/治疗后重新评估。严重损伤的关节(即Pettersson评分为bb60)被排除在外。关节疾病进展的定义为Pettersson评分增加bb0.1分。无进展生存期(PFS)是主要终点。MRI观察显示关节积液,滑膜肥大,血黄素沉积,关节边缘骨破坏或囊性变性,软骨破坏。年龄、体重指数(BMI)、因子VIII (FVIII)活性、活化部分凝血活素时间(APTT)、凝血酶原时间(PT)、治疗类型、年度关节出血率(AJBR)、血友病关节健康评分(HJHS)也进行了评估。随后,采用单因素和多因素Cox比例风险回归模型分析影响HA进展的临床和影像学特征。结果:本研究包括65例患者的98个关节。在随访期间,63个关节出现进展。单因素和多因素Cox分析显示,mri检测到的滑膜肥大(MRI-SP)是HA进展的独立危险因素。将BMI纳入模型可提高其预测性能(模型1:c-index = 0.671, P)。结论:mri检测的滑膜肥大是HA进展的独立危险因素。该预测模型包括BMI作为评估HA进展风险的协变量,可作为HA患者制定个性化治疗计划的辅助工具。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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