Assessment Of Haemophilic Arthropathy Research Articles

ASSESSMENT OF HAEMOPHILIC ARTHROPATHY IN PATIENTS WITH SEVERE HEMOPHILIA USING THE HEMOPHILIA EARLY ARTHROPATHY DETECTION WITH ULTRASOUND (HEAD-US) SCORE AND THE HEMOPHILIA JOINT HEALTH SCORE (HJHS) BY DIFFERENT CAREGIVERS

Objective: There are few studies on the assessment of hemophilic arthropathy using the hemophilia early arthropathy detection with ultrasound (HEAD-US) and hemophilia joint health score (HJHS) scores.This study aimed to examine how radiologists and rheumatologists could evaluate hemophilic arthropathy in individuals with severe hemophilia using HEAD- US and HJHS scores simultaneously. Material and Methods: Between 2021 and 2022, 168 joints from 28 individuals with severe hemophilia A and B were investigated at six-month intervals (TP1 and TP2). The HJHS scores of all patients were recorded. The HEAD-US in each hemophilic patient's six joints (elbow, knee, and ankle) were evaluated by radiologists and rheumatologists. Results: The ankle assessment by HEAD-US showed the highest rate (34%) of synovitis by radiologist evaluation at TP1 in patients with an HJHS score of 0. The knee assessment by HEAD-US showed the highest rate (56%) of synovitis and bone damage by rheumatologist evaluation at TP1 in patients with an HJHS score of 0. HEAD-US ankle and knee examinations revealed the highest rate of synovitis (34%) by radiologist evaluation at TP2 in patients with an HJHS score of 0. The HEAD-US knee assessment revealed the highest rate of synovitis (44%) by rheumatologist evaluation at TP2 in patients with an HJHS score of 0. In the assessment of elbow and knee joints, there was a moderate to good correlation between HJHS and HEAD-US scores by different caregivers at different time points (p< 0.05). Conclusion: Our findings indicate that clinicians play a crucial role in the early diagnosis of subclinical hemophilic arthropathy, with HEAD-US scoring conducted by rheumatologists similar to radiologists in severe hemophilia patients without pathology in HJHS scoring.

Role of Musculoskeletal Ultrasound in Detection of Hemophilic Arthropathy

Abstract Background Hemophilia is an X-linked congenital bleeding disorder, with hemophilia A comprising the majority of cases and hemophilia B. Von Willebrand disease (VWD) is a bleeding disorder of variable severity, caused by deficiency of von Willebrand factor (VWF) especially type III. The severe form of the bleeding disorders is characterized by spontaneous hemoarthrosis which occurs mainly in large synovial joints and results in hemophilic arthropathy (HA) that is characterized by soft tissue changes such as synovial hypertrophy and effusion as well as cartilage and bony damage., MRI is the gold standard, however ultrasound (US) was proven to be a sensitive tool in detecting synovial hypertrophy with results comparable to MRI, as well as differentiate between hemoarthrosis and arthritis-metiated joint pain, together with having more advantage to the MRI, making it an excellent tool in assessing disease activity. Objective To describe the value of MSK ultrasound in assessment of intra-articular findings in hemophilic arthropathy (HA) in the pediatric age group. Methods A descriptive, cross-sectional study that included 120 joints in 20 patients with bleeding disorders, (moderate or severe hemophilia A/B or vWD). Evaluated joints included elbows, knees and ankle joints on both sides for every patient. One additional patient was evaluated for a hip joint affection. The abnormal US findings among the involved joints were compared to the sonographically-normal contralateral side. We excluded patients with recent joint surgeries, those who have any other rheumatological disease. Results Out of the 120 joints examined, 28 joints showed US abnormal findings. The knee was the most common involved joint (75%), followed by the ankle (30%) then the elbow (15%). The most frequent US lesion was synovial hypertrophy (82%), followed by joint effusion (78.6%), synovial hyperemia (53%) and lastly comes the ostochondral lesions (7.1%). Other juxta-articular findings were reported as myositis, tenosynovitis and intra-/intermuscular hematoma. Hemoarthrosis was found in 6 out of 16 knee joints, 2 out of 7 ankles, yet no elbow nor hip joint bleedings were reported. There was a significant correlation between the incidence of synovial thickening, bone and cartlage damage and bleeding incidence and increasing age. A high statistical significant difference was found between the normal and abnormal joints (by US) and especially the knee and ankle joints. Conclusion MSK US has now emerged as a promising imaging modality for the early detection and management of HA, and for the evaluation of hemarthrosis and painful MSK episodes in hemophilic patients. It has a high efficacy in assessment of the soft tissue abnormalities such as synovial hypertrophy, vascularity and joint effusion, as well as late-onset degenerative changes. Global standardization of the different US scoring systems and clear definitions of the US pathologies in HA are needed with high-level MSK US training practice as well, for accurate assessment of HA using US.

A new and simplified comprehensive ultrasound protocol of haemophilic joints: the Universal Simplified Ultrasound (US-US) protocol

To present a new protocol to optimise ultrasound (US) assessment of haemophilic arthropathy. Ultrasound of haemophilic arthropathy joints was performed using three different ultrasound protocols, namely, the Toronto-Vellore Comprehensive Ultrasound (TVC-US) protocol, the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US), and the newly developed Universal Simplified Ultrasound (US-US) protocol. Synovial hypertrophy, haemosiderin deposition, effusion, erosion, and cartilage loss were evaluated in 20 joints. The reliability and diagnostic efficiency of these protocols was compared using magnetic resonance imaging (MRI). The correlation between the TVC-US and US-US protocols for synovial hypertrophy was excellent: kappa significance (KS) was 1, but was substantial (KS=0.65) with the HEAD-US protocol. For effusion, both the TVC-US and the HEAD-US protocols had substantial correlation with the US-US protocol (KS=0.7 and 0.6 respectively). The correlation for erosion and cartilage loss was excellent between the TVC-US and the US-US with MRI (KS=1), but poor (KS=0) with the HEAD-US protocol. The US-US protocol also had good interobserver agreement (KS=1). The accuracy of the US-US protocol is comparable to the TVC-US protocol and MRI and is superior to the HEAD-US protocol in the assessment of haemophilic arthropathy.

Assessment of haemophilic arthropathy through balance analysis: a promising tool

The purpose of this study was to develop a tool able to distinguish between subjects who have haemophilic arthropathy in lower limbs and those who do not by analyzing the centre of pressure displacement. The second objective was to assess the possible different responses of haemophiliacs and healthy subjects by creating a classifier that could distinguish between both groups. Fifty-four haemophilic patients (28 with and 26 without arthropathy) and 23 healthy subjects took part voluntarily in the study. A force plate was used to measure postural stability. A total of 276 centre of pressure displacement parameters were calculated under different conditions: unipedal/bipedal balance with eyes open/closed. These parameters were used to design a Quadratic Discriminant Analysis classifier. The arthropathy versus non-arthropathy classifier had an overall accuracy of 97.5% when only 10 features were used in its design. Similarly, the haemophiliac versus non-haemophiliac classifier had an overall accuracy of 97.2% when only 7 features were used. In conclusion, an objective haemophilic arthropathy in lower limbs evaluation system was developed by analyzing centre of pressure displacement signals. The haemophiliac vs. non-haemophiliac classifier designed was also able to corroborate the existing differences in postural control between haemophilic patients (with and without arthropathy) and healthy subjects.

A systematic review of ultrasound imaging as a tool for evaluating haemophilic arthropathy in children and adults.

The purpose of this study was to semi-quantitatively assess the evidence on the value of ultrasound (US) for assessment of haemophilic arthropathy (HA) in children and adults based on the following questions: (1) Does early diagnosis of pathological findings, using available US techniques, impact the functional status of the joint? (2) Do current available US techniques have the ability to accurately detect pathological changes in target joints in haemophilic patients? (3) Does treatment (prophylaxis) improve US evidence of haemophilic arthropathy in children and adults? (4) Is there any association between various US scoring systems and other clinical/radiological constructs? Of the 6880 citations identified searching databases such as MEDLINE, Embase, CENTRAL and Web of Science, 20 articles investigating either the diagnostic accuracy of US and/or US scanning protocols and scoring systems for assessment of HA met the inclusion criteria for the study. Of these, 14 articles evaluating the diagnostic accuracy of US were assessed by two independent reviewers for reporting quality using the Standards for Reporting of Diagnostic Accuracy (STARD) tool and for methodological quality using the Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool. Using STARD, 1/14 studies (7%) was scored as of high reporting quality and 8/14 (57%), of moderate quality. Assessment with QUADAS-2 reported 2/14 (14%) studies as having high methodological quality and 6/14 (43%) as having moderate quality. There is fair evidence (Grade B) to recommend US as an accurate technique for early diagnosis of HA, to demonstrate that US scores correlate with clinical/US constructs and to prove an association between US findings and functional status of the joint. However, there is insufficient evidence (Grade I) to conclude that US-detectable findings in HA are sensitive to changes in therapy.

Assessment of Hemophilic Arthropathy by Ultrasound: Where Do We Stand?

Joint hemorrhages represent the most common type of bleeding episode in persons with hemophilia, and recurrent hemarthrosis triggers chronic arthropathy, which is the most frequent chronic complication in these patients. In recent years, in the frame of a comprehensive care approach, a growing attention has been given to the periodic assessment of the joint status in hemophilia patients with the aim to identify early arthropathic changes and to prevent the development of a clinically overt arthropathy. Besides clinical examination, X-ray and magnetic resonance imaging (MRI) are currently used to evaluate joint status and to monitor the disease progression in hemophilia. Considering the limitations of X-ray and MRI, growing interest has been given to ultrasound (US) as a possible tool to assess joint status and identify early arthropathic changes in hemophilia patients. In the present review, we summarize major literature evidence on the use of joint US for the evaluation of markers of disease activity (joint effusion and synovial hypertrophy) and of degenerative damages (osteochondral changes) in patients with hemophilia. On the whole, being able to identify the presence of intra- or extra-articular fluid, US examination is the fastest and most reliable technique to identify acute conditions, such as hemarthrosis. In addition, the information on joint involvement provided by US in the patient follow-up may influence treatment decisions on a personalized basis. The use of US as part of a routine clinical examination by hemophilia experts may optimize the diagnostic workflow, avoiding additional costs and long waiting lists for patients referred to imaging departments. In the frame of a comprehensive care approach, US might represent a strategy to early detect and monitor synovial hypertrophy and osteochondral changes in hemophilia, thus extending the clinical examination and helping identify joints to be studied with a second-level examination such as MRI.

Ultrasonographic characteristics of haemophilic arthropathy in the knee joints

To investigate the characteristics of ultrasonography in haemophilic arthropathy in knee joints. The knee joints (n=135) of 68 patients with haemophilia A (n=59) or haemophilia B (n=9) were consecutively evaluated with ultrasonography and scored according to MELCHIORRE ultrasound score. The correlations between ultrasound score and the number of knee joint bleeding episodes, age, and World Federation Haemophilic orthopedic (WFHO) score were studied. 68 male patients with median age 20 (3-48) years old were enrolled in this study. The prevalences of hydrops articuli, fibrotic septa, synovial hypertrophy, synovial hypertrophy with angiogenesis, haemosiderin deposition were 39.2% (53/135), 3.0% (4/135), 77.0% (104/135), 49.6% (67/135), 37.8% (51/135), respectively; and the prevalences of cartilage damage, bone erosion, bone remodeling, osteophytes were 63.7% (86/135), 48.1% (65/135), 3.0% (4/135), 13.3% (18/135) respectively. The average ultrasound score was 5.21[95% CI (4.50-5.93)]. The ultrasound score of knees joints according to age 0-6, 7-18 and > 18 years old groups were 0.63[95% CI (0.15-1.10)], 2.88[95% CI (1.90-3.85)], 7.83[95% CI (7.07-8.60)], respectively; and the difference among groups was statistically significant (P<0.01). The significant difference of ultrasound score was not found among moderate, mild and severe haemophilia (F=0.90, P=0.914). A significant positive correlation between ultrasound and WFHO scores was demonstrated (r=0.745, P<0.01). The ultrasound score increased significantly with the increase of number of knee bleed Episodes (F=49.926, P<0.01). The main characteristics of ultrasonography of haemophilic arthropathy in knee joints were identified as followings: synovial hypertrophy, cartilage damage, bone erosion, hydrops articuli, and haemosiderin deposition. Ultrasonography was a fast, effective, safe, and available inspection method in assessment of haemophilic arthropathy.

Hemophilic Arthropathy: A Review of Imaging and Staging

Hemophilic arthropathy (HA) contributes the greatest morbidity and cost in the hemophilic population. Imaging plays a crucial role in accurately monitoring the disease process in all phases and evaluating treatment. It has been shown that prophylactic factor VIII replacement therapy, if given early in life, can prevent or delay the progression of HA and reduce the frequency of hemarthroses. This review evaluates the role of the different imaging modalities used in the staging of HA. It is noteworthy that a new refined consensus magnetic resonance imaging scale for assessment of HA has recently been published which together with introduction of semiquantitative scoring systems in ultrasound will help to provide a more tailored approach to the management of HA in the future.

An attempt to improve the clinical scale for assessment of haemophilic arthropathy in children

Of the methods used to evaluate haemophilic arthropathy, clinical joint score can only detect advanced and not minor changes, which usually occur in younger patients. In addition, the currently used clinical scores are poorly correlated with the MRI and X-rays scales. In an attempt to address these shortcomings, a modification of Stockholm clinical scale was designed in which elements of clinical information were included. This new scale was applied in 165 joints of 40 patients with haemophilia A and B and the results were statistically analyzed for its capacity to detect early joints alterations. Furthermore, the adjusted score was compared with Pettersson's radiological score and Denver's MRI score, which can detect early signs of arthropathy. The adjusted scale (a) revealed the earliest abnormalities of haemophilic arthropathy and its correlation with the Pettersson and Denver scores is better than those of Stockholm's scale, (b) provided prediction of the Denver score and (c) was simple and safe to be performed and it could easily be repeated.