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Abstract Number: 1272

Whole-Body Versus Thigh Magnetic Resonance Imaging In The Assessment Of Juvenile Dermatomyositis

Clara Malattia1, Annalisa Madeo2, Marta Dellepiane3, Diliana Beleva3, Stefania Viola4, Alessandro Consolaro5, Nicolino Ruperto1 and Alberto Martini6, 1PRINTO, Genoa, Italy, 2Istituto G Gaslini, Pediatria II, Reumatologia, Genova, Italy, 3Istituto G. Gaslini, Pediatria 2, Genoa, Italy, 4Istituto G. Gaslini, Istituto Giannina Gaslini, Genova, Italy, 5Pediatria II, Istituto Giannina Gaslini, Genova, Italy, 6Istituto Giannina Gaslini, Pediatria II, Reumatologia, Paediatric Rheumatology International Trials Organisation (PRINTO) Coordinating Center, Genoa, Italy, Genoa, Italy

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Disease Activity, Juvenile dermatomyositis and magnetic resonance imaging (MRI)

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Session Information

Session Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects II: Pediatric Systemic Lupus Erythematosus, Pediatric Vasculitis and Pediatric Myositis

Session Type: Abstract Submissions (ACR)

Background/Purpose: MRI is a promising tool to assess disease activity in juvenile dermatomyositis (JDM). So far all MRI JDM studies focused on the thigh musculature. Whole-body(WB)-MRI screens the entire body with the advantage to evaluate larger areas of muscles and subcutaneous tissue. Aim of the study was to compare WB-MRI and thigh-MRI (T-MRI) in the assessment of disease activity and in predicting treatment efficacy in JDM.

Methods: WB-MRI were obtained from 43 JDM patients and 43 controls. Muscle abnormalities were scored by 2 independent readers in 36 muscular groups while perifascicular and subcutaneous inflammation were assessed on 8 sites (arm, forearm, thigh and lower leg). Two different readers separately scored both thighs for muscles, subcutaneous and perifascicular oedema. WB- and T-MRI scores were compared in terms of reliability, construct validity and predictive value.

Results: WB-MRI revealed myofascial and subcutaneous inflammation of areas other than the thigh in 8 (18.6%) and 10 (23.2% ) patients. Concordance between WB- and T-MRI myofascial and subcutaneous scores was moderate (rs=0.59 and rs=0.69 respectively) while the concordance for muscle inflammation was excellent (rs=0.97). Inter-reader agreement was excellent for both T- and WB-MRI scores (ICC:0.96 and 0.98). Both scores showed excellent correlations with Manual Muscle Test (rs=-0.82 for T-MRI; rs=-0.84 for WB-MRI) and Childhood Myositis Assessment Scale (rs=-0.83 for T-MRI;CMAS rs=-0.81 for WB-MRI). WB- and T-MRI muscle scores were significantly higher in JDM active patients when compared with control group (pB<0.0001 for both the scores) and inactive patients (T-MRI pB=0.0022, WB-MRI pB=0.0037). Responsiveness to change was higher for WB-MRI score (SRM=1.65) compared to that of T- MRI score (SRM=1.04). The ability of WB and T-MRI to predict treatment efficacy was tested only in patients with disease duration ≤ 2 months (N=21) who started treatment with prednisone alone (N=4) or in combination with methotrexate (N=15) or cyclosporine (N=2). Eleven patients (52.4%) met the PRINTO criteria for improvement at 3-months follow-up. WB-MRI muscular score (median value: 61.2) and T-MRI score (7.2) were higher in non-responders compared to responders (34.5; p=0.001 and 5; p=0.01, respectively). WB-MRI muscle score > 57 was predictive of a poor response to treatment, as evaluated by ROC curve analysis (AUC:0,9). Non-responders showed higher WB myofascial MRI score (1.5) compared to responders (0; p=0.04); no significant difference in myofascial T-MRI score and in subcutaneous involvement were found between responders and non-responders. Seven out of 8 patients (87.5%) with diffuse homogeneous pattern of inflammation at WB-MRI were non responders; viceversa 10 out of 13 patients with the typical patchy distribution of muscle inflammation were responders (p=0.02).

Conclusion:

WB-MRI provides a complete assessment of total inflammatory burden and was more accurate than T-MRI in identifying myofascial and subcutaneous inflammation and in predicting treatment efficacy.


Disclosure:

C. Malattia,
None;

A. Madeo,
None;

M. Dellepiane,
None;

D. Beleva,
None;

S. Viola,
None;

A. Consolaro,
None;

N. Ruperto,
None;

A. Martini,
None.

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