Autoimmune autonomic ganglionopathy associated with Sjögren's syndrome presenting with recurrent abdominal distension

BMJ Case Rep. 2018 Oct 2:2018:bcr2017223785. doi: 10.1136/bcr-2017-223785.

Abstract

A 65-year-old woman with Sjögren's syndrome presented with recurrent abdominal distension, constipation, weight loss, orthostatic dizziness, loss of sweating and incomplete emptying of the bladder. Gastrointestinal dilatation but no evidence of malignancy or obstruction was found on CT of the abdomen, oesophagogastroduodenoscopy or colonoscopy. Postvoiding residual urine volume was increased. Antiganglionic acetylcholine receptor antibody was positive. We diagnosed as autoimmune autonomic ganglionopathy. The patient responded to corticosteroid treatment. One year after treatment, she continued to have mild gastrointestinal symptoms, but overall condition was stable without further intervention.

Keywords: neurogastroenterology; sjogren’s syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoantibodies / immunology*
  • Autoimmune Diseases of the Nervous System / complications*
  • Autoimmune Diseases of the Nervous System / drug therapy
  • Autoimmune Diseases of the Nervous System / immunology*
  • Diagnosis, Differential
  • Female
  • Ganglia, Autonomic / immunology*
  • Gastric Dilatation / drug therapy
  • Gastric Dilatation / etiology*
  • Gastric Dilatation / physiopathology
  • Glucocorticoids / therapeutic use
  • Humans
  • Prednisolone / therapeutic use
  • Recurrence
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / physiopathology

Substances

  • Autoantibodies
  • Glucocorticoids
  • Prednisolone