An MRI scale for assessment of haemophilic arthropathy from the International Prophylaxis Study Group

Lundin, Björn; Manco-Johnson, M L; Ignas, Danial M; Moineddin, Rahim; Blanchette, Victor S.; Dunn, Amy L.; Gibikote, Sridhar; Keshava, Shyamkumar N.; Ljung, Rolf; Manco‐Johnson, M. J.; Miller, Scott E.; Rivard, Georges E.; Doria, A.

doi:10.1111/j.1365-2516.2012.02883.x

Cited by 122 publications

(199 citation statements)

References 28 publications

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“…It is of note that some abnormalities are better detected with MRI, such as subchondral cysts, cartilage loss and haemosiderin deposits [25]. To improve consistency in determining joint status, simplified innovative protocols and scoring techniques have been developed for both ultrasound and MRI, that is, the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) and the compatible additive MRI scale, respectively [26,27]. In addition, X-rays scored using the Pettersson scale can still be informative for patients with haemophilia and established joint damage [28].…”

Section: Resultsmentioning

confidence: 99%

Evaluation of early musculoskeletal disease in patients with haemophilia

Seuser¹,

Khayat

Négrier³

et al. 2018

Blood Coagulation &Amp; Fibrinolysis

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Early joint damage in patients with haemarthrosis often escapes diagnosis because of insufficient investigation of biomechanical changes. Arthropathy in haemophilia requires complex assessment with several tools. Considering the increased emphasis on an integrated approach to musculoskeletal (MSK) outcomes, re-evaluation of MSK assessment to address individual patient needs is warranted. To advise on the optimal use of current assessment tools and strategies for tailored MSK evaluation in patients with haemophilia. A panel of experts in haemophilic arthropathy evaluated internationally recognized assessment tools through published literature and personal expertise. Each tool was considered, scored and ranked for their utility in the clinical assessment of MSK damage. Subsequently, a patient evaluation table detailing advice on type and frequency of assessments for different patient populations was constructed. To obtain a complete MSK assessment, multiple tools must be used to ensure each criterion is evaluated. For patients with haemophilia, clinical examination of the joint, disease-specific structure/function scores, and activity/participation scores including quality of life are important, and should be performed on a regular basis according to age and clinical condition. Joint imaging is recommended in the prevention, diagnosis and follow-up of haemophilic arthropathy and should be used in conjunction with joint structure and function scores. An integrated approach to MSK assessment using combinations of tools will allow earlier management of dysfunction and may improve long-term outcomes. This approach could be used in long-term follow-up of all patients independent of age and disease stage, especially in children to prevent arthropathy.

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Section: Resultsmentioning

confidence: 99%

Evaluation of early musculoskeletal disease in patients with haemophilia

Seuser¹,

Khayat

Négrier³

et al. 2018

Blood Coagulation &Amp; Fibrinolysis

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“…Scoring scales for reliable assessment of hemophilia arthropathy have been established. [33][34][35][36] However, MRI application to young children is limited and also expensive. More recently, ultrasound has been used for diagnosis of joint disease in hemophiliacs.…”

Section: Outcome Assessmentmentioning

confidence: 99%

Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens

Oldenburg¹

2015

Blood

328

322

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Prophylactic application of clotting factor concentrates is the basis of modern treatment of severe hemophilia A. In children, the early start of prophylaxis as primary or secondary prophylaxis has become the gold standard in most countries with adequate resources. In adults, prophylaxis is reasonably continued when started as primary or secondary prophylaxis in childhood to maintain healthy joint function. Initial data support that adult patients with already existing advanced joint arthropathy benefit from tertiary prophylaxis with significantly lowered number of bleeds, almost complete absence of target joints, and less time off from work. Current prophylactic regimens, although very effective, do not completely prevent joint disease in a long-term perspective. Joint arthropathy in primary prophylaxis develops over many years, sometimes over a decade or even longer time periods. The ankle joints are the first and most severely affected joints in those patients and thus may serve in outcome assessment as an indicator of early joint arthropathy when followed by ultrasound or magnetic resonance imaging. Optimized outcome and best use of available resources is expected from individualization of therapy regimens, which comprises the individual’s bleeding pattern, condition of the musculoskeletal system, level of physical activity and the pharmacokinetic profile of the substituted coagulation factor, and most recently includes novel products with extended half-lives.

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“…Non-contrasted joint MRI with a T2*-weighted gradient recalled echo sequence in three planes was per- formed on any joint with >10% active or passive joint ROM asymmetry or reduced joint ROM compared to age- and BMI- matched historic controls from The Normal Joint Study [4]. This significance threshold was determined to be clinically significant and not the result of measurement variation by a team of physical therapists at St. Jude and Vanderbilt University.…”

mentioning

confidence: 99%

“…Joint abnormalities were estimated using the International Prophylaxis Study Group (IPSG) MRI score, an additive scale tallying soft tissue and osteochondral changes [4]. MRI of the affected joint was compared with MRI of the unaffected contralateral joint if avail- able.…”

mentioning

confidence: 99%