Background Despite modern treatment approaches Juvenile Dermatomyositis (JDM) remains a serious and potentially life threatening disease. There are few studies which have documented mortality among large series of cases of JDM treated with modern therapeutic approaches. The Juvenile Dermatomyositis National Registry and Repository, UK and Ireland (JDRR) was established in 2000 to facilitate res...

CONTEXT One of the major current challenges related to juvenile idiopathic inflammatory myopathy is the search for highly sensitive and specific non-invasive methods for diagnosis as well as for follow-up. OBJECTIVES The aim of our study was to describe typical magnetic resonance imaging findings and to investigate the usefulness of this method in detecting active muscle disease in juvenile d...

PURPOSE OF REVIEW To present scientific evidence on clinical and molecular effects of exercise in adult and juvenile idiopathic inflammatory myopathies focusing on recent studies. RECENT FINDINGS In patients with inclusion body myositis (IBM), one small, open study recently for the first time reported on improved muscle strength and functional capacity after a twice-a-day home exercise progra...

Dermatomyositis is an idiopathic inflammatory myopathy, with skin manifestations. A less frequently recognized but clinically important complication of dermatomyositis is lipodystrophy (LD), and associated metabolic abnormalities that increasingly recognized as complications of juvenile dermatomyositis. Lipodystrophy is less frequently reported in adult onset dermatomyositis.In this paper we ar...

BACKGROUND Calcinosis cutis is usually seen in long standing and untreated cases of juvenile dermatomyositis. CASE CHARACTERISTICS 7-year-old girl with severe calcinosis cutis who developed cutaneous ulceration, rash and myopathy. OBSERVATION Myopathic changes in EMG, muscle edema in MRI, elevated muscle enzymes and Jo-1 positive antibodies. OUTCOME Treatment with prednisolone and methotr...

Juvenile dermatomyositis (JDMS) is a chronic inflammatory condition characterized by muscle weakness and a distinctive rash caused by underlying vasculopathy. Long-term complications include subcutaneous and muscular calcification, contractures and in some cases the gradual development of a second connective tissue disease. Early aggressive treatment with systemic immunosuppressants and other a...

a change in proinflammatory plasma cytokine profile of patients with juvenile dermatomyositis correlating with disease activity Juvenile dermatomyositis ( JDM) is a rare autoimmune disorder, affecting mainly muscles and skin. The mainstay of treatment is high dose corticosteroids, combined with other immunosuppressive drugs. In about 30% of patients, the disease cannot be controlled despite mul...

The clinical profile of 7 children and their follow-up is described. There was female preponderance with mate to female ratio of 1:6. The median age of onset was 6 years. All the patients had skin rash, muscle weakness and abnormal enzyme profile. Muscle biopsy was performed in 6 and was abnormal in all of them. The electromyogram (EMG) was performed in 6 and was found abnormal in five. All the...

Calcinosis is rarely observed in juvenile-onset amyopathic dermatomyositis in contrast to juvenile-onset dermatomyositis. A 6-year-old female presented with several 0.5 to 2 cm-sized painless grouped masses on both knees for 3 years. The patient also presented with multiple erythematous scaly patches and plaques on both elbows, knuckles, buttock, ankles and cheeks. Her mother had similar skin l...

Background Intravenous Immunoglobulin (IVIG) is widely used in paediatric inflammatory disease. The Department of Health, UK recently produced guidelines for IVIG use and a demand management plan in response to global IVIG shortages, recommending use in "selected" patients with juvenile dermatomyositis (JDMS), as a "last resort" in systemic juvenile idiopathic arthritis (sJIA), and use not supp...