Detection and evaluation of haemophilic arthropathy: Which tools may be considered more reliable

Abstract

Introduction Progressive arthropathy is the main cause of morbidity in patients with severe haemophilia. Diagnostic imaging can detect even subclinical arthropathy and impact prophylactic treatment. However, in most clinical settings the regular joint evaluation and follow‐up are based on clinical evaluation and patient's personal reporting of problems, while diagnostic imaging is not regularly employed. Aim The aim of our prospective study was to assess how ultrasound (US), clinical examination, patient's subjective assessment and certain laboratory biomarkers correlate with magnetic resonance imaging (MRI) for detection and evaluation of haemophilic arthropathy in order to determine which tool is the most reliable. Methods The study included 30 patients with severe haemophilia (age range 16‐49 years). MRI (IPSG), US (HEAD‐US), clinical examination (HJHS 2.1) and patient's subjective assessment of elbows, knees and ankles were performed; additionally, blood samples for laboratory analysis were taken (s‐25‐OH vitamin D, s‐ferritin, s‐C‐terminal telopeptide of type I collagen, s‐N‐terminal propeptide of type I procollagen and s‐cartilage oligomeric matrix protein). MRI results were used as a reference standard for joint status. Pearson's r was used to assess correlation of other methods with MRI. Results The correlation with MRI was the highest for US (r = .92), considerably higher than for clinical evaluation (r = .62) and patient's subjective assessment (r = .66). There was no correlation between the presence or degree of haemophilic arthropathy and any of the laboratory biomarkers. Conclusion The results of our study warrant the inclusion of US into the regular follow‐up of patients with severe haemophilia, where the equipment and staffing permit.

Clinical assessment and point of care ultrasonography: How to diagnose haemophilic synovitis

“…31 Plut et al found that the negative predictive value of physical examination was 40% for joint damage compared with MRI. 19 These high published percentages of subclinical synovitis could be related to the fact that synovitis does not usually show joint tension, nor is it painful. 3,32 It appears that synovial hypertrophy is more frequently subclinical in the elbows than in the knees and ankles.…”

“…De la Corte‐Rodriguez et al demonstrated that 18/167 (10.7%) patients with no history of haemarthrosis and with HJHS 0 had subclinical synovitis in HEAD‐US 31 . Plut et al found that the negative predictive value of physical examination was 40% for joint damage compared with MRI 19 . These high published percentages of subclinical synovitis could be related to the fact that synovitis does not usually show joint tension, nor is it painful 3,32 …”

“…Although the complete assessment was performed, only the variable synovitis (score 0–2) was used for this analysis. This ultrasound protocol was performed because it is recommended by expert groups to be used at the point of care 1,6 and because it has a high detection capability of joint changes in haemophilia 19–21 . Particularly for synovial hypertrophy, ultrasound has been shown to have a sensitivity of 95%, 19 89%, 20 92%, 21 depending on the series, compared with MRI.…”

Joint Health Markers in Hemophilia: The State of the Art

“…endothelial specific isoform of type XVIII collagen (COL-18N), tissue inhibitor of metalloproteinase (TIMP-1), degraded type II collagen (C2M) and inflammatory markers, e.g. VEGF, interleukin-6 (IL-6), neutrophil extracellular traps (NETs) showed statistically significant correlations with the degree of HA, while bone markers did not show this correlation [7][8][9][10][11][12][13][14][15]. Kjeld et al previously showed that the cartilage marker COL-18N was significantly associated with the annual bleeding rate (ABR), a parameter directly associated with the degree of HA on X-rays [16].…”

Challenges in biomarker research in haemophilic arthropathy

“…

With great interest, we read the original article 'Detection and evaluation of haemophilic arthropathy: Which tools may be considered more reliable' by Plut et al 1

…”