Autoimmune cholangitis presenting as pancreatic malignancy in a man with hand morphea

Abstract

We report a 63 years old man with hand morhea who was admitted because of 6 weeks history of weight loss, weakness, low grade fever and jaundice. Physical examination revealed jaundice, hand morphea and a thin patient. Laboratory tests revealed aneamia, increased serum transaminase levels and an every day increasing of alkaline phosphatase, gamma-glutamyl transpeptidase and direct bilirubin, with a 3-fold increasing of Ca 19.9. The U/S, C.T and MRI of the abdomen were in favor of malignacy in the head of pancreas althought ERCP was within normal limits. Serologic tests for microbial infections were negative but immunoserologic tests, revealed ANA +1/160 (type fine speckled), AMA (-), SMA (-),centromere antibody (-), cryoglobulins (-), RF=1/320, C3=34 ui/ml, C4<6 ui/ml.

Liver biopsy revealed autoimmune cholangitis and the patient was started on 0,5 mg/kg methylprednisolone. He recovered totally within 2 months and he is still alive in exellent health with normal clinical and laboratory profile. It is known that scleroderma can co-exist with other autoimmune phenomena. We add to this that also a localized scleroderma (morphea) can co-exist with autoimmune cholangitis presenting as pangreatic malignancy.

Althought in this case the clinical, serological and radiological findings, exept ERCP, were in favor of neoplasia, immunoserologic tests and liver biopsy offered the true diagnosis before unnessescary laparotomy was performed.

Keywords: autoimmune cholangitis, morphea, Ca19.9, ERCP, pancreatic malignancy, (autoimmune pancreatitis).