Holmes-Adie syndrome, autoimmune hepatitis and celiac disease: a case report

World J Gastroenterol. 2006 Mar 7;12(9):1485-7. doi: 10.3748/wjg.v12.i9.1485.


A 35-year-old female patient presented with the following symptoms of Holmes-Adie syndrome: photophobia,enlargement of the left pupil unresponsive to light, Achilles areflexia. The pilocarpine test was positive. No tumor or other neurological abnormality was found. She had a 19-year history of autoimmune hepatitis. Flares up were observed following each 3 deliveries. At age of 31 she presented with diarrhea and weight loss. Abdominal tumor was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric multicystic lymphangioma.Simultaneously, celiac disease was diagnosed.Gluten-free diet resulted in a significant improvement of celiac disease,but not of autoimmune hepatitis. Autonomic neuropathy was proven by standard cardiovascular tests. The patient was a homozygous carrier for HLA DQ2 antigen characteristic for celiac disease and heterozygous for HLA DR3 B8 frequent in autoimmune liver diseases. Our novel observation on association of Holmes-Adie syndrome with autoimmune hepatitis and celiac disease is suggestive for a common immunological background for all three entities present in a patient with mesenteric multicystic lymphangioma.

Publication types

  • Case Reports

MeSH terms

  • Adie Syndrome / complications*
  • Adie Syndrome / diagnosis
  • Adie Syndrome / immunology
  • Adult
  • Celiac Disease / complications*
  • Celiac Disease / diagnosis
  • Celiac Disease / immunology
  • Female
  • Hepatitis, Autoimmune / complications*
  • Hepatitis, Autoimmune / diagnosis
  • Hepatitis, Autoimmune / immunology
  • Humans
  • Lymphangioma, Cystic / complications
  • Lymphangioma, Cystic / diagnosis
  • Peritoneal Neoplasms / complications
  • Peritoneal Neoplasms / diagnosis