Age-related severity and distribution of haemophilic arthropathy of the knee, ankle and elbow among Chinese patients with haemophilia

Haemophilia. 2020 Jan;26(1):129-135. doi: 10.1111/hae.13858. Epub 2019 Oct 27.

Abstract

Introduction and aims: Age-related severity and distribution of haemophilic arthropathy (HA) among Chinese patients with haemophilia using the Haemophilia Early Detection with Ultrasound (HEAD-US) system have not been extensively studied.

Methods: In our study, 89 patients with moderate and severe haemophilia were recruited. A total of 534 joints (knees, ankles and elbows on both sides included) were evaluated using musculoskeletal ultrasound (MSKUS) and scored using the HEAD-US system.

Results: Prevalence and average number of HA were 39.1% and 0.7, 90.6% and 3.2, 94.1% and 4.5, and 100% and 4.3 for ages ≤10, 11-20, 21-30 and 31-40 years, respectively. Prevalence and mean number of knee, ankle and elbow arthropathies also increased with age, although joint damages progressed in unparallel patterns. A significant difference in synovium subscores was observed between patients aged <10 and >10 years. An increasing tendency was observed in cartilage and subchondral bone subscores along with age before 30 years. No significant difference in mean joint scores was found between patients receiving on-demand therapy and those receiving on-demand to low-dose prophylactic therapy.

Conclusions: Haemophilic arthropathy developed in early childhood and progressed mainly before 30 years of age among Chinese patients with haemophilia, although in different ways among the knee, ankle and elbow.

Keywords: Haemophilia Early Detection with Ultrasound scoring scale; haemophilic arthropathy; musculoskeletal ultrasound; prevalence; prophylaxis.

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Age Factors
  • Ankle / diagnostic imaging
  • Ankle / pathology*
  • Asian People*
  • Child
  • Child, Preschool
  • Elbow / diagnostic imaging
  • Elbow / pathology*
  • Hemophilia A / complications*
  • Hemophilia A / drug therapy
  • Humans
  • Joint Diseases / complications*
  • Joint Diseases / diagnostic imaging
  • Knee / diagnostic imaging
  • Knee / pathology*
  • Prevalence
  • Severity of Illness Index
  • Synovial Membrane / diagnostic imaging
  • Synovial Membrane / pathology
  • Young Adult