title: Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis.
abstract: OBJECTIVE
  To compare whole-body MRI (WB-MRI) with clinical examination in the assessment of disease activity in juvenile dermatomyositis (JDM).
  
  
  METHODS
  WB-MR images were obtained from 41 JDM patients and 41 controls using a 1.5 T MRI scanner and short τ inversion recovery sequences. 18 patients had follow-up WB-MRI. Muscle, subcutaneous tissue and myofascial signal abnormalities were scored in 36 muscular groups and on proximal and distal extremities. WB-MRI and clinical assessments were performed concurrently and results compared. Validation procedures included analysis of feasibility, reliability, construct validity, discriminative ability and responsiveness.
  
  
  RESULTS
  WB-MRI revealed distal legs (26/41 patients) and forearm (19/41 patients) muscle inflammation undetected during clinical examination and allowed an accurate assessment of subcutaneous (23/41 patients) and myofascial involvement (13/41 patients). 27 patients showed a patchy distribution of muscle inflammation while in seven the abnormal hyperintense areas tended to be homogeneously distributed. The inter-reader agreement for muscular, subcutaneous and myofascial WB-MRI scores was excellent. Correlations between WB-MRI muscle score and disease activity measures were excellent (Manual Muscle Test: rs=-0.84, Childhood Myositis Assessment Scale: rs=-0.81). WB-MRI score was higher in JDM active patients when compared with the control group (pB<0.0001) and the inactive patients (pB=0.004), and showed an excellent responsiveness (standardised response mean=1.65). Follow-up WB-MRI showed resolution of inflammation in nine patients whereas clinical criteria for remission were satisfied in five.
  
  
  CONCLUSIONS
  WB-MRI provides additional information to clinical evaluation and represents a promising tool to estimate total inflammatory burden, tailor treatment and monitor its efficacy.
authors: Pediatria II, Istituto Giannina Gaslini, , Genova, Italy.
authors: Clara
authors: C
authors: Malattia
authors: Maria Beatrice
authors: MB
authors: Damasio
authors: Annalisa
authors: A
authors: Madeo
authors: Angela
authors: A
authors: Pistorio
authors: Anna
authors: A
authors: Providenti
authors: Silvia
authors: S
authors: Pederzoli
authors: Stefania
authors: S
authors: Viola
authors: Antonella
authors: A
authors: Buoncompagni
authors: Chiara
authors: C
authors: Mattiuz
authors: Agnese
authors: A
authors: Beltramo
authors: Alessandro
authors: A
authors: Consolaro
authors: Angelo
authors: A
authors: Ravelli
authors: Nicolino
authors: N
authors: Ruperto
authors: Paolo
authors: P
authors: Picco
authors: Gian Michele
authors: GM
authors: Magnano
authors: Alberto
authors: A
authors: Martini
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D015331:Cohort Studies; D003882:Dermatomyositis / Q000175:diagnosis* / Q000473:pathology; D005205:Fascia / Q000473:pathology*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007249:Inflammation / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology*; D010808:Physical Examination; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D040521:Subcutaneous Tissue / Q000473:pathology*; D051598:Whole Body Imaging
keywords: Dermatomyositis; Disease Activity; Magnetic Resonance Imaging