Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
Physical Therapy Sciences, Program in Clinical Health Sciences, University Medical Center Utrecht, Utrecht, The Netherlands.
Haemophilia. 2017 Nov;23(6):934-940. doi: 10.1111/hae.13327. Epub 2017 Sep 5.
Joint bleeds in patients with haemophilia may result in haemophilic arthropathy. Monitoring joint health is essential for identifying early signs of deterioration and allows timely adjustment of treatment.
The aim was to describe changes in joint health over 5-10 years follow-up and identify factors associated with joint health deterioration in patients with haemophilia.
A post hoc analysis was performed from previous cohort studies in patients with moderate/severe haemophilia, ≥16 years. Joint health of ankles, knees and elbows was measured with the Haemophilia Joint Health Score (HJHS) from 2006-2008 (T0) to 2011-2016 (T1). Analyses were performed on patient level (ΔHJHS-total) and joint level (ΔHJHS-joint). Deterioration was defined as ΔHJHS-total ≥4 and ΔHJHS-joint ≥2.
Sixty-two patients (median age 25, 73% severe haemophilia, median [interquartile range] 0.0 [0.0;2.0] joint bleeds between T0 to T1) were included. After median 8 years, HJHS-total deteriorated in 37% and HJHS-joint in 17%. Ankle joints (31%) showed deterioration more often than elbows (19%) and knees (3%). Deterioration of HJHS-total was only associated with severe haemophilia. Deterioration of HJHS-joint was weakly associated with a lower HJHS at baseline and more self-reported limitations in activities, and strongly with more joint bleeds between T0 and T1 and presence of synovitis.
In 37% of patients with moderate/severe haemophilia and low joint bleeding rates, joint health deteriorated over 5-10 years. Ankle and elbow joints showed deterioration most frequently. Factors found in this study help to identify which joints need frequent monitoring in patients with haemophilia with access to early prophylaxis.
血友病患者的关节出血可能导致血友病性关节病。监测关节健康对于发现早期恶化迹象至关重要，并允许及时调整治疗。
本研究旨在描述 5-10 年随访期间关节健康状况的变化，并确定与血友病患者关节健康恶化相关的因素。
对先前中度/重度血友病（≥16 岁）患者的队列研究进行了事后分析。使用血友病关节健康评分（HJHS）从 2006-2008 年（T0）到 2011-2016 年（T1）测量踝关节、膝关节和肘关节的关节健康状况。在患者水平（ΔHJHS-总）和关节水平（ΔHJHS-关节）上进行分析。恶化定义为 ΔHJHS-总≥4 和 ΔHJHS-关节≥2。
共纳入 62 例患者（中位年龄 25 岁，73%为重度血友病，T0 至 T1 期间中位数[四分位间距]0.0[0.0；2.0]关节出血）。中位随访 8 年后，HJHS-总恶化发生率为 37%，HJHS-关节恶化发生率为 17%。踝关节（31%）的恶化发生率高于肘关节（19%）和膝关节（3%）。HJHS-总恶化仅与重度血友病相关。HJHS-关节恶化与基线时较低的 HJHS 评分、更多的活动受限自我报告、T0 至 T1 期间更多的关节出血以及存在滑膜炎呈弱相关，与滑膜炎呈强相关。
在接受低关节出血率早期预防治疗的中度/重度血友病患者中，37%的患者在 5-10 年内关节健康状况恶化。踝关节和肘关节最常出现恶化。本研究发现的因素有助于识别需要频繁监测的关节，以便为血友病患者提供早期预防。
