Primary Sjogren's syndrome presenting with generalized autonomic failure

Eur J Neurol. 2004 Sep;11(9):635-8. doi: 10.1111/j.1468-1331.2004.00846.x.


A 64 year-old woman developed Raynaud's phenomenon and dry eyes/mouth. Laboratory examination revealed positive Schirmer's test, rheumatoid factor and anti-nuclear antibody, and lymphocytic sialoadenitis on salivary gland biopsy. These features strongly suggested the diagnosis of primary Sjogren's syndrome. Three years later, she gradually developed generalized autonomic failure without apparent sensory neuropathy on nerve conduction study. She had systolic pressure fall of 51 mmHg on head-up tilt test, cardiovascular supersensitivity to diluted norepinephrine infusion, cardiac denervation in [123I]-MIBG scintigraphy, impaired R-R variability, decreased sweating and prolonged colonic transit time. Autoimmune autonomic ganglionopathy was mostly responsible for her autonomic failure.

Publication types

  • Case Reports

MeSH terms

  • Cardiovascular Diseases / etiology
  • Cardiovascular Diseases / physiopathology
  • Digestive System / physiopathology
  • Female
  • Humans
  • Middle Aged
  • Pupil / physiology
  • Raynaud Disease / complications
  • Raynaud Disease / physiopathology
  • Regional Blood Flow / physiology
  • Shy-Drager Syndrome / etiology
  • Shy-Drager Syndrome / physiopathology*
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / physiopathology*
  • Skin / blood supply
  • Urologic Diseases / etiology
  • Urologic Diseases / physiopathology
  • Vasomotor System / physiopathology