Primary Biliary Cirrhosis

Suspected autoimmune hepatitis and primary biliary cirrhosis unmasked by interferon-beta in a multiple sclerosis patient

“…The exact mechanism behind the induction of autoimmunity by IFN-b in MS patients is unknown (Durelli et al, 1998;Pulicken et al, 2006). PBC may have remained subclinical, although fatigue was present in this patient, which, in hindsight could have also been related to PBC (Bhandari et al, 2011). The long-term use of IFN-b may have aggravated the presumed pre-existing PBC, triggering the elevations in ALT and AST, appearing as autoimmune hepatitis.…”

“…Similar to the development of MS, PBC and autoimmune hepatitis are thought to begin once a genetically susceptible individual encounters an unknown environmental trigger (Bhandari et al, 2011;Mieli-Vergani and Vergani, 2011); for this patient, the trigger may have been long-term exposure to IFN-b. Although no HLA typing was performed on this patient, therefore conclusions on the patients' genetic predisposition to PBC cannot be made (Invernizzi et al, 2012).…”

Tired of Being Tired

“…3 PBC is now most commonly diagnosed on the basis of abnormal laboratory values, often before the onset of symptoms or significant fibrosis. 4 Liver biopsy is not necessary for diagnosis in the setting of an elevated AP and positive AMA, but can be considered if diagnostic uncertainty exists. If the AP is the only abnormality in the hepatic function panel, fractionation is appropriate to determine whether the elevation stems from the liver or another source.…”

“…4 When symptoms of PBC do occur, the two most common are fatigue and pruritus. Both may result in significantly impaired quality of life and can occur at any point in the disease.…”

Incidence of Primary Biliary Cholangitis in a Rural Midwestern Population

“…[1][2][3] The disease progresses over decades and may eventually lead to cirrhosis and liver failure. [4][5][6] PBC was formerly known as primary biliary cirrhosis, but the name was changed from 'cirrhosis' to 'cholangitis' to remove the stigma associated with cirrhosis, as many patients are free of cirrhosis at the time of diagnosis. 7 The etiology of PBC is thought to be a function of both genetic and environmental factors.…”