[Angioimmunoblastic lymphadenopathy with dysproteinemia and sclerosing cholangitis]

A 28-year-old man with recurrent swelling of both upper eyelids was found to have increased values in several liver function tests (GOT 162 U/l, GPT 356 U/l, gamma-GT 643 U/l, bilirubin 3.0 mg/dl, alkaline phosphatase 925 U/l). Abdominal ultrasonography demonstrated lymph node enlargements up to 3 cm, dilated intra- and extrahepatic bile ducts, as well as a cyst of 3 cm size in the pancreatic tail. Endoscopic retrograde cholangiopancreatography and punch biopsy of the liver revealed sclerosing cholangitis. In addition to the eyelid swellings the patient also had protrusion of the left eyeball, blood eosinophilia (800/microliter) and marked increase in polyclonal IgG (6930 mg/dl) with lymphadenopathy suggesting the diagnosis of angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD, lymphogranulomatosis X), confirmed by lymphocyte surface marker analysis. However, histological examination of a lymph node was more suggestive of a T-zone lymphoma. Treatment with ursodeoxycholic acid (250 mg three times daily) and prednisolone (initially 2 mg/kg daily) quickly led to normal biochemical values and regression of the eye changes. In addition, treatment with interferon alpha-2b (initially 3 mill. U daily for 10 days) was begun. The abnormalities in the bile ducts disappeared 6 months later. The patient has been in full remission for 25 months (prednisolone dosage reduced to 12.5/7.5 mg alternating daily and interferon alpha-2b 3 mill. U three times weekly). This response makes AILD with secondary involvement of the bile ducts the most likely diagnosis.