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Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis
  1. Clara Malattia1,2,
  2. Maria Beatrice Damasio3,
  3. Annalisa Madeo1,
  4. Angela Pistorio4,
  5. Anna Providenti1,
  6. Silvia Pederzoli1,
  7. Stefania Viola1,
  8. Antonella Buoncompagni1,
  9. Chiara Mattiuz3,
  10. Agnese Beltramo1,
  11. Alessandro Consolaro1,
  12. Angelo Ravelli1,2,
  13. Nicolino Ruperto1,
  14. Paolo Picco1,
  15. Gian Michele Magnano3,
  16. Alberto Martini1,2
  1. 1Pediatria II, Istituto Giannina Gaslini, Genova, Italy
  2. 2Dipartimento di Scienze Pediatriche, Università degli Studi di Genova, Genova, Italy
  3. 3UO Radiologia, Istituto Giannina Gaslini, Genova, Italy
  4. 4Servizio di Epidemiologia e Biostatistica, Istituto Giannina Gaslini, Genova, Italy
  1. Correspondence to Dr Clara Malattia, Pediatria II, Istituto Giannina Gaslini, Largo G. Gaslini 5, Genova 16147, Italy; claramalattia{at}ospedale-gaslini.ge.it

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.

  • Dermatomyositis
  • Magnetic Resonance Imaging
  • Disease Activity

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