Autonomic failure and proximal skeletal myopathy in a patient with primary Sjögren syndrome.

Abstract:

:Autonomic failure and proximal skeletal myopathy are rare features of the Sjögren syndrome (SS). We describe a 51-year-old woman with primary SS who had development of esophageal dysmotility, urinary retention, severe orthostatism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well to corticosteroid therapy. Although dysfunction of the peripheral nervous system has a prevalence rate of 20% in patients with SS, most commonly the nerve dysfunction is a sensory deficit, and autonomic neuropathy is less frequent. Autonomic neuropathy due to SS may be underreported. The cause of our patient's myopathy remains undetermined. We speculate that the myopathy was due to either a form of polymyositis or an immune-mediated neuropathy with muscle involvement.

journal_name

Mayo Clin Proc

journal_title

Mayo Clinic proceedings

authors

Sorajja P,Poirier MK,Bundrick JB,Matteson EL

doi

10.4065/74.7.695

keywords:

subject

Has Abstract

pub_date

1999-07-01 00:00:00

pages

695-7

issue

7

eissn

0025-6196

issn

1942-5546

pii

S0025-6196(11)65070-2

journal_volume

74

pub_type

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