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NPCM is rare, and MRI may help its differentiation from other inflammatory lesions. However, the presence of active infection outside CNS and some imaging characteristics should point to this diagnosis.

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Background:Juvenile dermatomyositis (JDM) is a rare multisystem connective tissues disorder of unknown aetiology. Assessment of disease activity is a chalange in clinical practice.Objectives:To compare whole-body MRI (WB-MRI) with clinical examination in the assessment of disease activity in JDM.Methods:We included consecutive JDM patients followed in the rheumatology unit. All patients were submitted to clinical and laboratory evaluation. WB-MRI images were obtained using a 1.5 T MRI scanner and short T inversion recovery sequences (STIR). Muscle, peripheral inflammation and subcutaneous inflammations signal abnormalities were scored in 42 muscular groups. Muscle inflammation was classified as: 0 = absent; 1 = Mild to moderate/involvement less than 50% of muscle extension and 2 = Accentuated/greater than 50%. Peripheral and subcutaneous inflammations were classified as: 0 = absent; 1 = present; and on proximal and distal extremities. WB-MRI and clinical assessments were performed concurrently and results compared. Evaluation was repeated after 12 months. Statistics was performed according to the nature of the variable.Results:WB-MRI revealed muscle inflammation in 6 (31.6%) at study entry. We observed grade 2 muscle inflammation of the right and left scapular girdle (1/19 patients), right and left pelvic girdle (2/19 patients) and right and left tight (1/19 patients). Grade 1 inflammation was observed in peripheral right and left arm (2/19 patients), peripheral right and left thigh (1/19 patients). Grade 1 subcutaneous inflammation was observed in right and left thigh (1/19 patients) and left leg (1/19 patients). Additionally we observed sacroiilitis (1/19 patients), spinal cord infarction (21%) and osteonecrosis (5.2%). All patients were treated with standardized treatment. After 12 months 13/19 (68.4%) patients repeated the WB-MRI. Five (38.4%) patients had new/worsening of muscle and subcutaneous inflammation, one (7.7%) patient had tibial medullary infarction. Correlations between WB-MRI muscle score and disease activity measures were excellent (Manual Muscle Test: r=−0.88, Childhood Myositis Assessment Scale: rs=−0.81). Patients with subcutaneous inflammation developed clinically evident subcutaneous calcifications during follow-up.Conclusion:WB-MRI provides additional information to clinical evaluation and represents a promising tool to determine the grade of muscle inflammation to additional peripheral and subcutaneous tissue inflammation.References:[1] Malattia C, Damasio MB, Madeo A, et al. Ann Rheum Dis 2014;73:1083–1090Disclosure of Interests:None declared

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