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Abstract Number: 2476

Role of Whole-Body Magnetic Resonance Imaging in the Assessment of Disease Activity in Juvenile Dermatomyositis. A Pilot Study.

Clara Malattia1, Annalisa Madeo2, Silvia Pederzoli1, Anna Providenti3, Marta Mazzoni2, Agnese Beltramo2, Alessandro Consolaro4, Stefania Viola5, Antonella Buoncompagni6 and A. Martini2, 1PRINTO, Genoa, Italy, 2Istituto G Gaslini, Pediatria II, Reumatologia, Genova, Italy, 3Istituto G Gaslini, Genova, UO Fisioterapia, Genova, Italy, 4Pediatria II, Istituto Giannina Gaslini, Genova, Italy, 5Istituto G. Gaslini, Istituto Giannina Gaslini, Genova, Italy, 6Istituto Giannina Gaslini, Genova, Italy

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Juvenile dermatomyositis and magnetic resonance imaging (MRI)

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Session Information

Session Title: Pediatric Rheumatology: Clinical and Therapeutic Disease III: Childhood Systemic Lupus Erythematosus and Other Vasculidities

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Musculoskeletal MRI represents a valuable non-invasive technique for dectecting muscle inflammation in idiopathic inflammatory myopathies (IIM). So far, all MRI studies in juvenile dermatomyositis (JDM) have focused on the thighs muscles. Whole-body MRI (WB-MRI) is a new technique which allows to screen the entire muscular-skeletal system and gives a complete assessment of the total inflammatory burden in patients with IIM. However, its potential in children with JDM has never been explored so far. Purpose: to evaluate the contribution of WB-MRI examination in the clinical assessment of JDM patients and to investigate its feasibility and validity in the assessment of disease activity.

Methods:

WB-MR images were obtained from 30 JDM patients (12M;18F,median age 8.6 years) and from 30 children (13M; 17F, median age 10.4 years) without inflammatory myopathies (control group), using a 1.5 Tesla and Short Tau Inversion Recovery (STIR) sequences. Signal intensity was scored using a 0-2 point scale in 42 muscular groups; myofascial and subcutaneous tissue inflammation were assessed on the upper and lower extremities using a 0-1 point scale. Validation procedures included the analysis of reliability, construct validity, discriminant validity and sensitivity to change.

Results:

in addition to a symptomatic proximal distribution of inflammation, WB-MRI revealed asymptomatic distal legs muscle inflammation in 19 out of 30 patients(70%) and asymptomatic forearms inflammation in 15 out of 30 patients (50%). Twenty-three patients showed a typical patchy and heterogeneous distribution of muscular inflammation. In 3 patients the abnormal hyperintense areas tended to be diffusely and homogeneously distributed within the muscles. WB-MRI showed inactive disease in 4 patients. Fascial and subcutaneous tissue inflammation were detected in 9 out of 30 (30%) and 18 out of 30 (60%) patients, respectively. WB-MRI scores were significantly increased in active JDM when compared with the inactive JDM group (p=0.02) and the control group (p<0.0001), indicating an excellent discriminant validity of the WB-MRI. The inter- and intra-reader agreement for the muscular, subcutaneous and fascial WB-MRI scores were excellent (intra-class correlation coefficient >0.8). The muscular WB-MRI score showed moderate to excellent correlations with indicators of disease activity such as the Manual Muscle Test (MMT;rs=0.86), the Childhood Myositis Assessment Scale (CMAS;rs=0.85) and physician’s assessment of disease activity (VAS Phys;rs=0.75). WB-MRI score showed a higher responsiveness to change (standardized response mean=0.86) compared to MMT (SRM=0.51), CMAS (SRM=0.28), VAS Phys (SRM=0.63) and CPK (SRM=0.17).

Conclusion:

WB-MRI provides additional information to the clinical assessment by revealing a wider involvement of muscle groups and different patterns of distribution of muscle inflammation. WB-MRI score allows to reliably visualize the extent of the inflammatory process and therefore it represents a promising non-invasive tool to estimate the total disease burden, to adjust treatment to disease severity and to monitor treatment efficacy in JDM.

 


Disclosure:

C. Malattia,
None;

A. Madeo,
None;

S. Pederzoli,
None;

A. Providenti,
None;

M. Mazzoni,
None;

A. Beltramo,
None;

A. Consolaro,
None;

S. Viola,
None;

A. Buoncompagni,
None;

A. Martini,
None.

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