While classical proximal muscle weakness is typical, it is also important the child undergoes thorough investigation for other organ involvement, such as of the gastrointestinal tract, including a speech and swallowing assessment or respiratory system (typically a high-resolution computed tomography scan). The degree of muscle change on MRI can be quantified on T2 STIR images and has been shown to correlate with disease activity. The investigation of a child suspected of having an inflammatory myopathy should include magnetic resonance imaging (MRI) of muscles in a specialist centre with experience of interpretation of such images. Assessment of other organ systems, including respiratory, gastrointestinal, speech and language is also critical. The tool has been partially validated in a large JDM population and demonstrated good reliability, content and construct validity, and responsiveness. The Cutaneous Assessment Tool (CAT) assesses skin disease in myositis by considering activity in 10 lesions, damage in four lesions and a combination of activity and damage in seven lesions. It has good reliability and scores correlate with periungual nailfold capillary changes. The Disease Activity Score (DAS) for JDM includes assessment of skin and muscle involvement and distribution of rash. Two skin score systems for the assessment of skin involvement in JDM have been proposed. In addition, careful assessment of the skin is mandatory, and this should include nail fold capillary examination. Clinical examination should include formal assessment of muscle strength using a validated tool, such as the Childhood Myositis Assessment Scale (CMAS) or manual muscle testing of the standardized eight groups of muscles (MMT8). Īlthough the criteria for diagnosis still used for many studies are still those of Bohan and Peter these criteria have been shown to be no longer reflective of modern day paediatric practice, since many paediatricians do not choose to perform electromyography on children, and some centres no longer routinely perform muscle biopsy. The increasingly common model of providing care through networks of specialists who share expertise and knowledge should improve the care of these complex patients, although delay in access to specialist care remains an issue. A recent literature review including studies from the past 15 years described a handful of patients in whom unusual physical findings such as splenomegaly or lymphadenopathy were noted on diagnosis, and in whom a primary malignancy was found within 12 months of diagnosis.Īssessment of suspected cases of juvenile inflammatory myositisĬhildren with suspected inflammatory myositis are ideally cared for in specialist centres where there is easy access to an experienced multidisciplinary team, including paediatric rheumatologists and neurologists, specialist nurses, physiotherapists, speech therapists, dermatologists, muscle histopathologists and also access to imaging and other investigations. In the UK JDM Cohort study (currently n = 336 patients) there have been no neoplasms reported in association with childhood myositis in a 12-year data collection period, although long-term follow up of these patients will be important to validate this finding. Unlike the adult idiopathic inflammatory myopathies (IIM), JDM is generally thought not to be a paraneoplastic phenomenon. The mean age of onset is 7 years, although about 25% of patients with JDM present under the age of 5 years. JDM has an incidence of approximately 3.2 per million children per year in the UK with differences between ethnic groups, and is at least three times more common in girls than boys. Although historically many clinical studies concentrated on muscular features, the cutaneous manifestations of JDM can be serious and difficult to treat and may progress to ulcerative disease and or subcutaneous calcification, impacting seriously on quality of life in the long term. Polymyositis and inclusion body myositis are rare in children. Juvenile dermatomyositis (JDM) is a rare but serious systemic autoimmune condition of childhood primarily affecting proximal muscles and skin.
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