Association Between Sclerosing Cholangitis and Paget Disease: Diagnostic Difficulties - Association Between Sclerosing Cholangitis and Paget Disease: Diagnostic Difficulties - Open Access Pub

A rare case of association between primary sclerosing cholangitis and Paget's disease emphasizing the diagnostic difficulties in front of increased alkaline phosphatase is reported. The association between sclerosing cholangitis and Paget's disease wasn’t yet described and could thus be coincidental. However, our observation underlines the benefit of dosing ALP isoenzyme to characterize the bone or hepatic origin of ALP and therefore, help to guide the diagnosis. DOI 10.14302/issn.2578-2371.jslr-18-2174 Paget's disease (PD) is characterized by an acceleration of bone remodeling responsible for an isolated increased alkaline phosphatase (ALP) 1. It is a frequent component of multisystem proteinopathy and may therefore lead to other medical conditions. Thus, arthritis may be caused by bowing of long bones in the leg, distorting alignment and increasing pressure on nearby joints. Moreover, cardiovascular disease can result from severe PD such as calcification of the aortic valve, aortic stenosis, left ventricular hypertrophy and eventually high-output congestive failure. Kidney stones are also more common in patients with PD. Finally, the teeth may become loose, nervous system problems may occur and angioid streaks may develop, possibly as a result of calcification of collagen or other pathological deposition 2. However, no association with slerosing cholangitis (SC) (primary or secondary), which is due to inflammation and fibrosis of biliary tract that causes biological cholestasis 3, 4, has already been described in the literature. We report a rare case of association between sclerosing cholangitis and Paget's disease emphasizing the diagnostic difficulties in front of increased ALP. We report the case of an asymptomatic 49 years old male patient, in which a routine check objectified a biological cholestasis (gammagmutamytransferase = 2-3N and ALP = 5-6 N without hyperbilirubinemia or cytolysis). Nos past medical facts were noted. Abdominal ultrasound, viral markers and antibodies measurement (Ac Anti-nuclear, anti-Mitochondrial, anti-LKM1, Anti-cytoplasmic) were normal. Magnetic resonance choalngiopancreatography objectified multiple biliary strictures and parietal irregularities evocative of SC (Figure 1). Colonoscopy showed no associated inflammatory bowel disease. Patient received high doses of ursodeoxycholic acid (20mg/kg) for the SC with partial improvement of liver function but persistence of a marked rise in ALP level. In order to better characterize the nature of ALP, a dosage of ALP isoenzymes was performed and objectified a predominant bone