Adie's syndrome and mesenterial lymphangioma in a patient with celiac disease and autoimmune hepatitis

Background: Celiac disease associated with autoimmune liver disease has been reported. We report a case of celiac disease associated with autoimmune hepatitis, Adie's syndrome and multicystic mesenterial lymphangioma. No such associations were reported formerly.

Case report: A 16-year old girl with autoimmune hepatitis responded well to prednisolone treatment. Flare up was observed following each 3 deliveries. At her age of 31 she presented with diarrhoea, rapid loss of body weight. Abdominal tumor in infrahepatic retroperitoneal region was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric muticystic lymphangioma. Celiac disease was diagnosed by villous atrophy, anti-endomysium and transglutaminase antibody positivity. Gluten-free diet resulted in a significant improvement and disappearance of symptoms of celiac disease, but not of autoimmune hepatitis.

At her age of 35 symptoms of Adie's syndrome developed: photophobia, dilatation and lack of light reaction of the left pupil, knee- and Achilles-tendon areflexia. No tumor or other neurological abnormality was found. The pilocarpine test was positive. Gall bladder hypomotility and gall stone formation was considered as consequence of autonomic neuropathy proved by standard cardiovascular tests. Axonal lesion of ulnar nerve was also detected by ENG-EMG. Improvement of liver tests was observed during immunosuppressive treatment, but symptoms of Adie's syndrome persisted.

Conclusion: This is the first report of association of celiac disease, autoimmune hepatitis, mesenteric multicystic lymphangioma and Adie's syndrome. Autoimmune pathomechanism could be considered as common background.