title: SPINART study: validation of the extended magnetic resonance imaging scale for evaluation of joint status in adult patients with severe haemophilia A using baseline data.
abstract: BACKGROUND
  Most previous joint imaging scales for haemophilia have focused on earlier disease stages observed in younger patients.
  
  
  OBJECTIVE
  We sought to demonstrate that the 45-item extended magnetic resonance imaging (eMRI) scale is a valid instrument for measuring joint status in adults with severe haemophilia A.
  
  
  METHODS
  Six scale categories (effusion/haemarthrosis, synovial hypertrophy, hemosiderin, erosion, subchondral cysts and cartilage loss) in two domains (soft tissue [range = 0-9] and osteochondral [range = 0-36]) were evaluated for each joint. eMRI scores were derived using baseline data from a randomized, controlled, parallel-group clinical trial (SPINART). Quantitative analysis of linearity included assessments of the relationship between scores derived from the eMRI scale vs. the 17-point International Prophylaxis Study Group (IPSG) MRI scale and Colorado Adult Joint Assessment Scale (CAJAS).
  
  
  RESULTS
  Patient eMRI scores correlated with age (r = 0.58), consistent with the expectation of arthropathy progression and more severe joint changes in older patients. eMRI scores demonstrated excellent between-reader agreement for overall patient score (intraclass correlation coefficient [ICC] = 0.88) and outstanding agreement for knee evaluations (ICC = 0.95). There was a strong linear relationship of the eMRI score with the CAJAS (r = 0.7). Adding individual bone evaluations for each joint increased the sensitivity of the instrument to detect change at low scores. There was minimal ceiling effect (5% maximum scores for all evaluated joints) compared with the IPSG MRI scale (20%). Internal reliability was good (overall Cronbach's alpha = 0.75).
  
  
  CONCLUSIONS
  Overall, the eMRI scale represents a valid and reliable measure of joint status in adolescents and adults with severe haemophilia A.
authors.affiliation: Bayer, Whippany, NJ, USA.
authors.forename: W
authors.initials: W
authors.lastname: Hong
authors.affiliation: ICON Medical Imaging, Warrington, PA, USA.
authors.forename: D
authors.initials: D
authors.lastname: Raunig
authors.affiliation: Department of Clinical Sciences, Lund University, Department of Diagnostic Radiology, Lund, Sweden.|Center for Medical Imaging and Physiology, Skåne University Hospital, Lund, Sweden.
authors.forename: B
authors.initials: B
authors.lastname: Lundin
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006395:Hemarthrosis / Q000175:diagnosis*; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007592:Joint Diseases / Q000000981:diagnostic imaging*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D055815:Young Adult
keywords: arthropathy; haemophilia; joint; magnetic resonance imaging; rating scale; recombinant factor VIII