Clinical and radiological assessment of joints in people with haemophilia in Assam, Northeast India: a cross-sectional study

Abstract Background Despite the availability of factor replacement therapy, including prophylaxis, to treat and prevent bleeding, haemophilic arthropathy continues to be the most common complication of haemophilia and significantly impairs the quality of life in people with haemophilia (PwH). Regular periodic assessment of joint status in PwH is essential to identify early arthropathic changes and prevent the development or progression of haemophilic arthropathy. Kinematic and kinetic assessment are preferable and MRI is the gold standard for diagnosing haemophilic arthropathy, but availability is limited in developing countries. HJHS and ultrasound in conjunction with HEAD-US have been shown to effective in assessing changes indicating arthropathy. Aim This study is designed to identify which radiological assessment tool(s) works best when integrated with clinical assessment tools to examine joint health in PwH, with the aim of establishing a cheap, effective, multimodal approach to joint assessment in clinical practice in low resource settings which can then help to guide treatment. Methods A cross-sectional observational study was carried out among PwH attending a clinic in the department of medicine at Assam Medical College and Hospital, Northeast India, over a one-year time period. The elbow, knee and ankle joints of participants were assessed clinically and functionally using HJHS and FISH and radiologically by Pettersson (X-ray) and HEAD-US (ultrasound) scoring. The resulting data was analysed statistically to ascertain correlation between these parameters. Results Sixty-seven PwH were enrolled in the study, with a mean age of 21.69±8.24 years (median 21 years); 38 (57.72%) had severe factor deficiency. The majority (71.70%) presented with joint bleeds; 40 (59.70%) had the knee as a target joint, followed by the elbow (11; 16.41%) and ankle (6; 8.95%). Mean ABR (annual bleed rate), AJBR (annual joint bleed rate) and ATJBR (annual target joint bleed rate) values had a positive correlation with increased severity of factor deficiency (p <0.001). FISH score increased with factor activity level; HJHS, HEAD-US and Pettersson scores decreased with factor activity level. Significant positive correlation was established between Pettersson score, age and ABR. The degree of association was greatest between HJHS and HEAD-US at 60.62%; between HJHS and Pettersson it was 57.74%. Statistically significant negative correlation was established between FISH and HEAD-US. Conclusion A combined multimodal approach using the HJHS, FISH and HEAD-US scoring systems can provide a cheap, quick, more reliable and accurate option for assessing haemophilic joints, with minimal inter and intra observer reliability. This can support the optimisation of management of joint damage in PwH in resource-constrained settings.