Autonomic failure and proximal skeletal myopathy in a patient with primarySjogren syndrome

Autonomic failure and proximal skeletal myopathy are rare features of the S jogren syndrome (SS), We describe a 51-year-old woman with primary SS who h ad development of esophageal dysmotility, urinary retention, severe orthost atism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well to corticosteroid therapy, Altho ugh dysfunction of the peripheral nervous system has a prevalence rate of 2 0% in patients with SS, most commonly the nerve dysfunction is a sensory de ficit, and autonomic neuropathy is less frequent. Autonomic neuropathy due to SS may be underreported, The cause of our patient's myopathy remains und etermined. We speculate that the myopathy was due to either a form of polym yositis or an immune-mediated neuropathy with muscle involvement.