Department of Haematology, Oslo University Hospital, Oslo, Norway.
Research Institute of Internal Medicine, Oslo University Hospital, Oslo, Norway.
Haemophilia. 2021 Mar;27(2):e253-e259. doi: 10.1111/hae.14245. Epub 2021 Feb 6.
Detection of early arthropathy is crucial for the management of haemophilia, but data on moderate haemophilia are limited. Therefore, we evaluated joint health and treatment modalities in Nordic patients with moderate haemophilia A (MHA) and B (MHB).
To explore and compare the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) and Haemophilia Joint Health Score (HJHS) to detect early arthropathy in moderate haemophilia.
A cross-sectional, multicentre study covering Nordic patients with MHA and MHB. Arthropathy was evaluated by HEAD-US and HJHS 2.1.
We assessed 693 joints in 118 patients. HEAD-US scores (medians [interquartile ranges]) were as follows: elbows 0 points (0-0), knees 0 (0-0) and ankles 0 (0-1). Respectively, by HJHS: elbows 0 (0-1), knees 0 (0-1) and ankles 0 (0-1). Cartilage (14%) and bone (13%) were most commonly affected by HEAD-US. Frequent HJHS findings were crepitus on motion in knees (39%), and loss of flexion (23%) and extension (13%) in ankles. HEAD-US correlated strongly with HJHS (elbows r = .70, knees r = .60 and ankles r = .65), but 24% had discordant scores. Joints with HJHS zero points, 5% captured HEAD-US ≥1 point. Moreover, 26% had HJHS findings without HEAD-US pathology. Notably, 31% of knees had crepitus on motion and normal HEAD-US.
Overall, the joints attained low scores implying good joint health. HEAD-US correlated strongly with HJHS. In 5%, HEAD-US detected subclinical pathology. Crepitus on motion was frequently reported despite normal HEAD-US, thus not necessarily reflecting arthropathy. HEAD-US therefore improves the joint assessment in moderate haemophilia.
早期关节病的检测对血友病的治疗至关重要，但有关中度血友病的数据有限。因此，我们评估了北欧中度血友病 A（MHA）和 B（MHB）患者的关节健康状况和治疗方式。
探讨和比较超声早期关节病检测（HEAD-US）和血友病关节健康评分（HJHS）在中度血友病中的早期关节病检测作用。
这是一项涵盖北欧 MHA 和 MHB 患者的横断面、多中心研究。采用 HEAD-US 和 HJHS 2.1 评估关节病。
我们评估了 118 例患者的 693 个关节。HEAD-US 评分（中位数[四分位间距]）如下：肘部 0 分（0-0），膝关节 0 分（0-0），踝关节 0 分（0-1）。相应地，HJHS 评分：肘部 0 分（0-1），膝关节 0 分（0-1），踝关节 0 分（0-1）。HEAD-US 最常影响软骨（14%）和骨骼（13%）。HJHS 常见发现包括膝关节运动时出现弹响（39%），踝关节活动度丧失（23%）和背屈（13%）。HEAD-US 与 HJHS 相关性较强（肘部 r=0.70，膝关节 r=0.60，踝关节 r=0.65），但 24%的关节评分不一致。HJHS 评分 0 分的关节中，5%的关节 HEAD-US 评分≥1 分。此外，26%的关节存在 HJHS 发现但无 HEAD-US 病理学表现。值得注意的是，31%的膝关节运动时有弹响，但 HEAD-US 正常。
总体而言，这些关节的评分较低，提示关节健康状况良好。HEAD-US 与 HJHS 相关性较强。5%的关节中，HEAD-US 检测到亚临床病变。尽管 HEAD-US 正常，但运动时仍常出现弹响，因此不一定反映关节病。因此，HEAD-US 可改善中度血友病的关节评估。
