Gastrointestinal Complications of Dermatomyositis

Gastrointestinal manifestations of DM in adults have included dysphagia, esophageal reflux, esophageal dysmotility, delayed gastric emptying, decreased intestinal motility, and rectal incontinence [1, 2]. The most common gastrointestinal symptom in juveni

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Gastrointestinal manifestations of DM in adults have included dysphagia, esophageal reflux, esophageal dysmotility, delayed gastric emptying, decreased intestinal motility, and rectal incontinence [1, 2]. The most common gastrointestinal symptom in juvenile DM is dysphagia, as a result of pharyngeal and upper esophageal involvement [3]; however, occasionally ulceration and perforation of the gastrointestinal tract [4, 5], and pneumatosis intestinalis [6, 7] have been reported. Although dysphagia results from inflammation and atrophy of esophageal muscle, ulceration is the consequence of vasculitis and thromboses of both esophageal and small bowel blood vesels [1, 5, 8, 9]. Dysphagia occurs in approximately 25–50% of DM patients. According to various clinical studies in a large group of patients with IIMs, dysphagia has been present in 29% of cases in Australia [10], 30% in Brazil [11], 31–43% in Canada [12, 13], 45% in the USA [14], 47% in Bulgaria [15], and 52% in Sweden [16]. Low frequency of dysphagia has been reported in Singapore – 11% of IIM patients [17] (Table 12.1). The dysphagia can be of two types: proximal or distal [18]. Proximal dysphagia is caused by the involvement of striated muscle of the pharynx or proximal esophagus. This involvement correlates with the severity of the muscle disease, and is responsive to steroids. Distal dysphagia is related to the involvement of non-striated muscle, and seems to be more common in patients with overlap with SSc or another connective tissue disease. Two different types of abnormalities are encountered in cricopharyngeal (CP) sphincter muscle [19]: (1) the duration of CP-sphincter pause is shortened, or (2) it is prolonged during water swallowing. These two findings are interpreted respectively as hyperreflexic and hyporeflexic sphincter behavior. In the first case, hyperreflexic sphincter behavior may indicate the inflammation and/or edema in the muscle during the acute stage of PM/ DM. However, in chronic disease course it may be related with muscle fibrosis [20]. In the second case, sphincter hyporeflexes could be related with the muscle weakness and the low muscle tonous of CP sphincter. Dysphagia generally signifies a rapidly progressive course, and is associated with the presence of pulmonary disease. It may also be associated with a poor prognosis. In some patients, involvement of the muscles of the upper esophagus, the cricopharyngeal muscle, and other muscles in the hypopharynx may produce difficulty in swallowing and dysphonia (nasal speech and hoarseness] [21]. The difficulty in swallowing has been L.A. Dourmishev and A.L. Dourmishev, Dermatomyositis: Advances in Recognition, Understanding and Management, © Springer-Verlag Berlin Heidelberg 2009

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12 Gastrointestinal Complications of Dermatomyositis

Table 12.1 Degree of dysphagia in DM [19] Grade 1 No complaint and no clinical findings related to oropharyngeal dysphagia Grade 2 Dysphagia is suspected from the patient’s complaints; however, the clinical examination does not support the