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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Primary biliary cirrhosis is the inflammatory destruction of peripheral bile ducts leading to cirrhosis. The majority of patients are female. It is associated with autoimmune disorders including rheumatoid arthritis, Sjögren syndrome, scleroderma and Hashimoto thyroiditis. The disease is limited to the intrahepatic bile ducts.
Pathology of the Intrahepatic Biliary Tree after Liver Transplantation
Published in Gianfranco Alpini, Domenico Alvaro, Marco Marzioni, Gene LeSage, Nicholas LaRusso, The Pathophysiology of Biliary Epithelia, 2020
James Neuberger, Rebecca Harrison
The diagnosis of recurrent primary biliary cirrhosis is controversial. Criteria for the diagnosis of PBC in the native liver may not be applicable to the allograft: there are many potential causes for the symptoms of PBC lethargy. Anti-mitochondrial antibodies persist after liver transplantation and associated extra-hepatic features may develop, but none of these features prove the existence of recurrent disease. The diagnosis must be made on histological grounds. As in the native liver, portal tracts with noncaseating granulomas around inflamed bile ducts are pathognomic of the condition (Fig. 4). Such features are seen relatively infrequently but most studies now report recurrence in up to 50% at 10 years.19 Unlike chronic rejection, bile ductular transformation is also seen.
Gastrointestinal
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
Primary biliary cirrhosis is the inflammatory destruction of peripheral bile ducts leading to cirrhosis. In total, 90% of patients are female. It is associated with autoimmune disorders including rheumatoid arthritis, Sjogren syndrome, scleroderma and Hashimoto thyroiditis. The disease is limited to the intrahepatic bile ducts.
Cancer risk in systemic sclerosis: identifying risk and managing high-risk patients
Published in Expert Review of Clinical Immunology, 2020
George E. Fragoulis, Dimitris Daoussis, Eleni Pagkopoulou, Alexandros Garyfallos, George D. Kitas, Theodoros Dimitroulas
For hematological malignancies, higher age at SSc onset and absence of SSc-associated antibodies have been suggested as risk factors [46]. Furthermore, the use of calcium channel blockers and higher age at SSc diagnosis has been associated with a higher risk for melanoma and early melanoma, respectively [46]. Finally, a higher frequency of primary biliary cholangitis – previously called primary biliary cirrhosis (PBC) in SSc patients compared to the general population, might account for the observed higher prevalence of liver cancer in these patients [47]. Said that SSc can overlap with other autoimmune rheumatic diseases (most commonly with Sjogren’s syndrome) in about 20% of the cases [86,87]. Although this has been suggested to associate with the milder phenotype of SSc, it has not been examined whether the risk for malignancy is lower in these patients. Whether some higher risk for hematological malignancies observed in SSc can be attributed to the coexistence of other autoimmune diseases like Sjogren’s syndrome, is unclear. Besides, Colaci et al., reviewing the literature found that in 7.8% of the cases with hematological malignancies complicating SSc, Sjogren’s syndrome, or another autoimmune disease co-existed [88].
Emerging drugs for the treatment of chronic pruritic diseases
Published in Expert Opinion on Emerging Drugs, 2020
Kayla Fourzali, Rachel Shireen Golpanian, Gil Yosipovitch
Bile acids, in addition to other endogenous compounds, are increased in serum of patients with cholestatic pruritus [40]. Bile acid re-uptake takes place in the terminal ileum by ileal bile acid transporters (IBAT) and an inhibition of IBAT is a potential therapeutic target [101]. The IBAT inhibitor GSK2330672 was evaluated in a phase II crossover trial for primary biliary cirrhosis and showed significant reduction of itch scores in both sequences of treatment [102]. Another phase II trial for dose-response in patients with this condition has recently completed (NCT02966834). IBAT inhibitor maralixibat did not prove antipruritic efficacy in phase II trials for use in familial intrahepatic cholestasis but phase III study is currently underway (NCT 03905330) [103].
Current and promising therapy for primary biliary cholangitis
Published in Expert Opinion on Pharmacotherapy, 2019
Andrea A Gossard, Keith D. Lindor
Primary biliary cholangitis (PBC) is a chronic, cholestatic liver disease characterized by nonsuppurative destruction of the interlobular and septal bile ducts. It can progress to cirrhosis with complications of end-stage liver disease oftentimes after decades of disease. The etiology of PBC, formerly known as primary biliary cirrhosis, is believed to be due to a combination of genetic predisposition and environmental triggers [1,2]. The prevalence of PBC is estimated to be between 19 and 402 cases per million [3]. There is a female predominance and it is most commonly diagnosed in the fourth and fifth decade of life.