Introduction: 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). Aim: 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. Methods: A cross-sectional, multicentre study covering Nordic patients with MHA and MHB. Arthropathy was evaluated by HEAD-US and HJHS 2.1. Results: 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. Conclusion: 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.
Bibliographical noteFunding Information:
The MoHem study was financially supported by an unrestricted research grant from Bayer HealthCare. HEAD‐US courses have been arranged and financially supported by Pfizer. MB was supported by funds from Stockholm County Council. Thanks to all physiotherapists, research nurses and physicians at the study centres who have contributed to enrolment and data collection.
EB has acted as a paid consultant to Bayer. JA has received research grant from SOBI, Shire/Takeda, Bayer, Octapharma and CSL Behring, and acted as consultant/speaker for SOBI, Shire/Takeda, Bayer, Octapharma, Pfizer and Novo Nordisk. MA has acted as consultant for SOBI, Pfizer, Roche and Bayer, speakers fee by Sobi, Pfizer and Bayer, reimbursement for attending symposium by Bayer and Sobi, and research grant by Swedish Haemophilia Society, FBIS. PAH has acted as a paid consultant to Bayer, Shire, Novo Nordisk, Octapharma, CSL Behring, Pfizer and Sobi including lectures. RJM, JH, AO, MB, TF, VN and GET stated that they had no interests which might be perceived as posing a conflict or bias.
© 2020 John Wiley & Sons Ltd
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Subject classification (UKÄ)
- joint score
- moderate haemophilia A
- moderate haemophilia B