A Multisystem Illness Involving Pancreas, Bile Ducts, and Salivary Glands

Venu Julapalli, MD; Daniel Ball, MD; Ravi Shivshankar, MDSeries Editor: Richard Goodgame, MD

Disclosures

June 17, 2005

Case Presentation

The patient is a 46-year-old man with a complicated history of biliary tract, pancreatic, and salivary gland disease.

Prior to the onset of this illness the patient was healthy. He had no excessive alcohol or tobacco use, no substance abuse, no travel, and had a stable family and finances. There was no relevant family history of serious diseases. Three years ago, the patient abruptly developed epigastric and right upper quadrant abdominal pain, nausea, and vomiting. He had persistent symptoms over the next few weeks, during which time he visited his local emergency department several times. After 1 month he developed jaundice. This began a series of interactions with multiple physicians and hospitals that is best summarized as 3 problems, as described below:

Biliary disease: Cholestatic jaundice developed 3 years ago and endoscopic retrograde cholangiopancreatography (ERCP; Figure 1 and 2) showed multiple biliary strictures suggestive of sclerosing cholangitis.

Liver biopsy showed periductular neutrophilic infiltrates, bile plugging (Figure 3), and mild periportal fibrosis around the bile ducts (Figure 4) without hepatitis or cirrhosis. These findings supported the diagnosis of bile duct obstruction, as may be seen with sclerosing cholangitis.

In the following year, the patient underwent multiple attempts at balloon dilation and stenting of dominant strictures in the biliary tree. Multiple brushings and biopsies of the ducts were performed, and no malignancy was found. During this period he lost 40 pounds. Two years ago all attempts at instrumentation of the biliary strictures was abandoned. Two months ago he was referred to our hospital. An ERCP was performed (Figure 5 and 6). Results showed a high-grade stricture in the distal common bile duct, marked dilation of the biliary system, and multiple intra- and extrahepatic ductal irregularities. Brushings and biopsies of the stricture were negative for malignancy.

Pancreatic disease: Although the patient had pancreatic-type pain at the onset of his illness 3 years ago, the initial imaging studies did not show impressive changes in the pancreas. The biliary abnormalities attracted more attention, but the abdominal pain never resolved. Two years ago, he had worsening pancreatic-type pain that prompted an abdominal MRI (Figure 7). Results of MRI showed a diffusely enlarged pancreas with a small halo of pancreatic edema.

Given the common bile duct stricture and 40-pound weight loss, a pancreatic malignancy was suspected. Pancreatic biopsy was performed (Figures 8-10), and results showed pancreatic fibrosis and mild inflammation that was nonspecific. No malignancy was found.

One year ago the patient underwent endoscopic ultrasound because of persistent pain. Results of imaging were suggestive of a hypoechoic mass in the pancreatic neck. Fine-needle aspiration of this lesion revealed no malignancy. Two months ago the patient was referred to our hospital. MRI of the pancreas (Figures 11 and 12, T1, post-gadolinium; transverse -- Figure 11; coronal -- Figure 12) showed a diffusely enlarged "sausage-like" pancreas with a surrounding halo of inflammation. No definite mass was seen.

Salivary gland disease: One year ago the patient noted bilateral, painful swelling of both submandibular salivary glands; this was associated with the sicca syndrome (dry mouth and eyes). A Schirmer test showed absent tearing. Biopsy of the submandibular gland showed chronic sialadenitis.

A complete physical examination showed weight of 170 pounds and height of 68 inches. Vital signs were normal. There was no jaundice or stigmata of chronic liver disease. There were only 2 abnormal physical findings: bilateral, firm swelling of both submandibular glands, and mild epigastric tenderness.

Routine laboratory tests were ordered; complete blood count, urinalysis, and electrolytes were normal. Results of serum chemistries were as follows: total protein, 8.7 g/dL; albumin, 4.2 gm/dL; total bilirubin, 1.6 mg/dL; direct bilirubin, 0.9 mg/dL; alkaline phosphatase, 142 U/L; alanine aminotransferase, 187 U/L; aspartate aminotransferase, 265 U/L; and amylase, 100 mg/dL.

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