Endocrine Abstracts

Introduction: Primary biliary cholangitis (PBC) is a rare chronic cholestatic liver disease, which is most likely of autoimmune origin. It is often associated with other autoimmune diseases, although it is rarely seen with Basedow.

Observation: We report the case of a 38 years old patient, chronic smoker, who presents clinical and biological hyperthyroidism related to Basedow, treated with synthetic antithyroid drugs for years with poor compliance and notion of discontinuation of treatment. He was admitted to our department for rapid preparation for radical treatment. The clinical examination showed a patient with alteration of the general state, tachycardic, with frank icterus of cholestatic appearance evolving since 2 months, presence of exophthalmos, and perception of a homogeneous goiter at cervical palpation. In front of the frank icterus, a hepatic assessment was carried out showing a hepatic cytolysis, a hepatic cholestasis and a hepatocellular insufficiency. Abdominal ultrasound was unremarkable. Viral serologies were negative. A liver biopsy was performed, showing a PBC, an esogastro-duodenal fibroscopy was performed, which did not show the presence of esophageal varices. An autoimmune workup was performed to look for associated autoimmune hepatitis, as part of the overlap syndrome is ongoing. For his hepatic problem, the patient was treated with ursodesoxycholic acid with slight improvement of the hepatic balance. Then received a rapid preparation with lugol, bblocker and corticosteroid therapy, and had a total thyroidectomy. The post-operative course was complicated by severe bleeding and hepato-cellular failure, resulting in the patient’s death.

Discussion and conclusion: Work has been done to determine the prevalence of autoimmune diseases associated with PBC and to assess their impact on the prognosis of this disease. These diseases were dominated by autoimmune hepatitis and Hashimoto’s thyroiditis. The association with Basedow was very rare. PBC is often associated with other autoimmune diseases, which should be routinely screened. Conversely, the search for PBC in patients with other autoimmune diseases is necessary in order to initiate treatment at early stages, thus improving the patient’s prognosis.