Primary Sclerosing Cholangitis and Paget\'s Disease of Bone, A Case Report and Review of Imaging Findings

Abstract

Paget's disease of bone is a metabolic bone disorder characterized by localized areas of increased bone production and cycle with excessive and irregular bone formation. Rapid bone resorption and formation lead to irregular bone tissue. Most cases are diagnosed incidentally on radiographs or as an isolated elevation of serum alkaline phosphatase. Primary sclerosing cholangitis is a chronic progressive disorder of unknown etiology characterized by inflammation, fibrosis, and narrowing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree. The association of both diseases with other diseases has been reported, but there are few reports of the association of these two diseases together. This association can present the physician with an important challenge for both diseases because both diseases lead to an increase in alkaline phosphatase. Here, we present a 52-year-old man who was also diagnosed with Paget's disease of the femur on evaluation for abdominal pain and elevated alkaline phosphatase. Zoledronic acid 5 mg was prescribed for the patient. Six months later, the patient did not complain of hip pain. The femur graph remained unchanged and the patient's alkaline phosphatase decreased and reached the normal range. Laboratory findings, imaging, and histology of the patient were explained. It seems that in patients with high alkaline phosphatase and primary biliary cirrhosis, the presence of pain or deformity of bones and joints should be checked with imaging to detect the presence of co-morbidities.

Keywords

• paget disease
• sclerosing cholangitis
• alkaline phosphatase
• zoledronic acid
• bone turnover