Arthropathy Hemophilic, Arthropathy of Knee, Hemophilia, MRI
Conditions
Brief summary
The aim of this study is to assess role of MRI in detecting synovial, cartilaginous , osseous abnormalities ، bleeding inside knee joint and to use a system for assessing HA as support for therapeutic regimes and for monitoring response to therapy .
Detailed description
Hemophilia is a mostly inherited genetic disorder caused by a complete or partial deficiency of coagulation factors VIII or IX, which impairs the body's ability to form blood clots-a process necessary to stop bleeding. This results in prolonged bleeding after injury, easy bruising, and an increased risk of internal bleeding, particularly within joints or the brain. Hemophilic arthropathy (HA), caused by recurrent hemarthrosis, is the most common musculoskeletal manifestation of hemophilia and one of the most disabling complications of the disease. It can lead to severe pain, deformity, destruction of the joint, debilitating arthritis, and permanent joint damage. Clinical assessment plays only a minor role in detecting the early stages of HA, as early clinical signs are often nonspecific and of limited value in quantifying disease involvement. Conventional radiographs mainly detect bone lesions that appear in advanced HA, but they cannot identify early changes such as synovial hypertrophy or focal cartilage destruction. Computed tomography (CT) is highly sensitive for detecting bone changes, but it provides limited information on soft tissue involvement and requires a relatively high dose of ionizing radiation. By contrast, magnetic resonance imaging (MRI) offers superior soft tissue contrast, allowing detailed evaluation of hemophilic joints and visualization of synovial and cartilaginous abnormalities. T1-weighted imaging: evaluates bone marrow, joint anatomy, and subchondral changes. T2 fat-suppressed imaging: useful for detecting synovitis and joint effusion. Gradient echo (GRE) or susceptibility-weighted imaging (SWI): detect chronic blood products (hemosiderin), with SWI being more sensitive and able to identify earlier blood deposits. Several scoring systems exist for the assessment of hemophilic arthropathy, including the European scoring system and the International Prophylaxis Study Group (IPSG) MRI scale. The IPSG system provides more detailed scoring and greater sensitivity for early disease, including subtle synovial changes. For radiographs, the most widely used system is the Pettersson radiographic scoring system. In this study, the investigators will use the IPSG MRI scoring system and the Pettersson X-ray scoring system to evaluate hemophilic arthropathy, with the aim of improving grading, management, and follow-up.
Interventions
None listed
Sponsors
Assiut University
Study design
Observational model
OTHER
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Adult patient ( more than 18 years old ) diagnosed as Haemophilia with history of bleeding in knee joint more than once
Exclusion criteria
* \*contraindications for MRI such as claustrophobia , metallic foriegn body carriers , cardiac pacemaker * patient refused the exam * Pregnant female patients
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| International Prophylaxis Study Group (IPSG) MRI Score for Hemophilic Arthropathy | After completion of MRI scanning session (within the same study visit). Overall Study Duration: 2 years (for recruitment, imaging, and data analysis). | Assessment of knee hemophilic arthropathy severity using the International Prophylaxis Study Group (IPSG) MRI scoring system. This scoring system evaluates early and advanced joint changes such as synovial hypertrophy, hemosiderin deposition, and cartilage/bone damage. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Pettersson X-ray Score for Hemophilic Arthropathy | After completion of X-ray scanning session (within the same study visit). Overall Study Duration: 2 years (including recruitment, imaging, and data analysis). | Assessment of knee hemophilic arthropathy severity using the Pettersson X-ray scoring system. This system evaluates joint space narrowing, osteoporosis, subchondral cysts, erosions, and bone deformities to quantify the degree of arthropathy. |
| Agreement Between MRI and X-ray Scoring Systems in Hemophilic Arthropathy | After completion of MRI and X-ray scanning sessions (within the same study visit). Overall study duration: 2 years (including recruitment, imaging, and data analysis). | To evaluate the agreement between the International Prophylaxis Study Group (IPSG) MRI score and the Pettersson X-ray score in assessing the severity of knee hemophilic arthropathy. This outcome will determine the level of concordance between the two imaging modalities in detecting joint changes. |
Contacts
Primary Contactkerolos wagdy maurice morid, principal investigator
Outcome results
None listed