Until recently the majority of myopathies involving multisystem disorders have been described in adults, but in whom the disease process has been in place for a number of years resulting in obvious damage to the intestinal smooth muscle coats, mostly with increased fibrosis. With modern physiological recording methods and histological techniques available in young children, myopathies associated with systemic disorders have been increasingly recognized. It is important to identify these conditions, as medical treatment may be available for them which can result in symptomatic improvement and even prevention of progression of the disease process, if it is put in place before fibrosis of the muscle coat has occurred.
Scleroderma, systemic lupus erythematosus and even dermatomyositis may all affect the muscle coats of the bowel. However, the best understood of these is scleroderma, a systemic disease characterized by the excessive deposition of collagen by fibroblasts in the skin and in many internal organs. It is associated with prominent and often severe alteration of the microvasculature, the autonomic nervous system and the immune system.22 Gastrointestinal involvement with symptoms of clinical relevance occur in approximately 50% of patients with the systemic form of scleroderma. The esophagus is the most commonly affected part of the gastrointestinal tract, followed by the rectum and then the small intestine. The lesions of the muscle coat are similar throughout the gut with atrophy and fragmentation of the muscle coats, collagen infiltration and fibrosis in a later stage of the disease.
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