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. 2025 Jul 7;14(13):4792.
doi: 10.3390/jcm14134792.

Comparison of Magnetic Resonance Imaging Scales for Assessment of Interval Changes of Arthropathy in Boys with Severe Hemophilia

Ningning Zhang  1 , Manuel Carcao  2 , Danial M Ignas  3 , Brian M Feldman  4   5   6 , Pamela Hilliard  7 , Rahim Moineddin  8 , Ann Marie Stain  9 , Paul Babyn  10 , Victor S Blanchette  2 , Andrea S Doria  11   12
Affiliations

Comparison of Magnetic Resonance Imaging Scales for Assessment of Interval Changes of Arthropathy in Boys with Severe Hemophilia

Ningning Zhang et al. J Clin Med. .

Abstract

Background/Objectives: The variety of magnetic resonance imaging (MRI) scales available to measure soft tissue and osteochondral changes in joints of persons with hemophilia poses challenges in evaluating published clinical/research studies. To evaluate the value of four MRI scales [(i) the 17-point International Prophylaxis Study Group [IPSG] additive scale; (ii) and (iii) the compatible IPSG progressive (P) and additive (A) scales; and (iv) the Denver progressive scale] to assess joint change in boys with hemophilia participating in a prospective two-year prophylaxis study. Methods: Boys with severe hemophilia A (ages, 7-16 years) followed at the Hospital for Sick Children, Toronto, Canada had MRI evaluations of six index joints (ankles, knees, elbows) at study entry and exit. Musculoskeletal (MSK) outcomes included in the study were the Colorado Child Physical Examination (PE) scale; the Pettersson (X-ray) scale; and the aforementioned 4 MRI scales. Results: Very strong (r ≥ 0.80) correlations were observed between the IPSG 17-point, the IPSG progressive (P) and the Denver MRI scales, and moderate (r = 0.40-0.59) to strong (r = 0.60-0.79) correlations for the IPSG 17 point and the IPSG additive (A) MRI scales. Very weak (r = 0.20-0.39) or no correlations were observed between soft tissue MRI scores and the swelling item of the Child PE scale. Conclusions: All four MRI scales demonstrated relative comparability of their construct validities for assessing mild/moderate hemophilic arthropathy. The 17-point IPSG additive scale is recommended as a reference standard in future long-term studies of young boys with hemophilia receiving factor and non-factor-based preventive therapies.

Keywords: arthropathy; children; hemophilia; magnetic resonance imaging (MRI); prophylaxis.

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Conflict of interest statement

Dr. Andrea Doria in the past three years has had the following relationships relevant to the disclosures of this manuscript: Advisory boards of the International Myositis Assessment & Clinical Studies Group (Chair, not for profit), OMERACT SIG in MRI in JIA and OMERACT Technical Advisory Group (Co-Chair, not for profit), and MIT Sloan Physicians’ Group (Co-Chair & Co-Founder, not for profit) and research support from The Terry Fox Foundation (Research Grant), NovoNordisk Health Care (Research Grant), the PSI Foundation (Research Grant), the Society of Pediatric Radiology (Research Grant), and the Garron Family Cancer Centre (Research Grant). Dr Victor Blanchette had received honoraria for participation in educational events and advisory boards sponsored by Sanofi-Genzyme and Takeda. He was a member of the Data Safety Monitoring Boards for Takeda. He received funding from Roche, Sanofi and Takeda for investigator-initiated studies. He was Chair of a non-profit International Prophylaxis Study Group that is funded by Educational grants from Bayer HealthCare, Biomarin, Novo Nordisk, Pfizer, Roche, Sanofi-Genzyme, Sobi and Takeda to the Hospital for Sick Children Foundation in Toronto, Canada. Dr. Blanchette passed away during the finalization stage of the manuscript review, but was the driving force for the completion of this work. Manuel Carcao reports receiving research support from Novartis, Novo Nordisk, Pfizer, Roche, Sanofi and Takeda and honoraria for speaking/participating in advisory boards from Bayer, LFB, Novo Nordisk, Pfizer, Roche, Sanofi and Takeda. He has no stocks or any equity in any pharmaceutical company and is not part of any company board. None of the authors had any financial interests in the conduct of this study profit.

Figures

Figure 1
Figure 1
Ten-year-old boy (at baseline MRI) with severe hemophilia A, without prior left knee bleed history and no inhibitory antibodies. Baseline (a) and follow-up (b) MRI (a) examinations of the left knee show reversible soft tissue changes (effusion/hemarthrosis) of the knee. (a) The baseline sagittal multiplanar gradient-recalled (MRGR) MR image demonstrates an International Prophylaxis Study Group (IPSG) MRI score of 1: soft tissue domain = 1 (effusion/hemarthrosis = 1, arrows); osteochondral domain = 0. (b) The follow-up MR images fail to show any evidence of soft tissue or osteochondral tissue changes. Follow-up IPSG MRI score = 0; soft tissue domain = 0; osteochondral domain = 0. A physiological amount of fluid is seen within the left knee joint.
Figure 2
Figure 2
Eight-year-old boy (at baseline MRI) with severe hemophilia A, and history of 9 prior left ankle bleeds and no inhibitory antibodies. Baseline (a) and follow-up (b) MR images show reversible soft tissue changes (effusion/hemarthrosis) and irreversible osteochondral change (cartilage loss) of the ankle. (a) The baseline sagittal multiplanar gradient-recalled (MPGR) MR image of his ankle shows moderate effusion (short arrows), and mild hemosiderin deposition/synovial hypertrophy, International Prophylaxis Study Group (IPSG) MRI score = 4 (effusion/hemarthrosis = 2, synovial hypertrophy = 1, hemosiderin deposition = 1). (b) The corresponding follow-up MRI shows slight increase in hemosiderin deposition (arrows) compared with the baseline MRI superposed to minimal effusion. It also demonstrates minimal focal loss in cartilage height anteriorly at the tibiotalar joint level (arrowheads). IPSG MRI score = 8; soft tissue domain = 7; osteochondral domain = 1 (effusion/hemarthrosis = 1, synovial hypertrophy = 3, hemosiderin deposition = 3, cartilage loss = 1).
Figure 3
Figure 3
Thirteen-year-old boy with severe hemophilia A, with history of 1 prior right elbow bleed and no inhibitory antibodies. Baseline coronal (a) and sagittal (b) multiplanar gradient-recalled (MPGR) MR images of the right elbow show a small effusion/hemarthrosis (thick arrows) and superimposed marked synovial hypertrophy and hemosiderin deposition (thin arrows), and early erosive changes and cartilage loss (arrowheads, (b)). International Prophylaxis Study Group (IPSG) score = 9: soft tissue domain = 7 (effusion/hemarthrosis = 1, synovial hypertrophy = 3, hemosiderin deposition = 3); osteochondral domain = 2 (surface erosions = 1, cartilage loss = 1). Follow-up coronal (c) and sagittal (d) MPGR MR images show persistent joint cartilage loss associated with hemosiderin deposition (arrowheads, (d)). Subchondral cysts have developed in the distal humerus (thin arrows, (c)) in the interim. IPSG score = 10: soft tissue domain = 7 (effusion/hemarthrosis = 1, synovial hypertrophy = 3, hemosiderin deposition = 3); osteochondral domain = 3 (surface erosions = 1, subchondral cysts = 1, cartilage loss = 1).
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