Women and Primary Biliary Cirrhosis

Abstract

Primary biliary cirrhosis occurs more frequently in women, and previous studies indicated that the average age of primary biliary cirrhosis (PBC) onset makes pregnancy in PBC patients uncommon. However, more recently, improved diagnostic testing has enabled detection of PBC in younger women, including those of childbearing age. This has led investigators to become increasingly interested in the relationship between the ontogeny of PBC and pregnancy. Published cases indicate that the typical age for pregnant women to be diagnosed with PBC is in the early 30s, and that during gestation, pruritus and jaundice are the most common symptoms. During gestation, susceptible women may experience onset of PBC resulting from the drastic changes in female hormones; this would include not only the mitochondrial damage due to accumulation of bile acids but also changes in the immune response during the different stages of pregnancy that might play an important role in the breakdown of self-tolerance. The mechanisms underlying the potential relationship between PBC and pregnancy warrant further investigation. For women first diagnosed with PBC during gestation, or those for whom first appearance of a flare up occurs during and postpartum, investigation of the immune response throughout gestation could provide new avenues for immunologic therapeutic intervention and the discovery of new treatment strategies for PBC.

The interplay of type I and type II interferons in murine autoimmune cholangitis as a basis for sex‐biased autoimmunity

“…Female bias is one of the hallmarks of autoimmunity and remains one of the critical enigmatic issues in autoimmunity, including PBC. (25,26) Recently, we have provided evidence that chronic overexpression of IFN-c leads to sex bias autoimmune cholangitis, which mimics the characteristics of PBC. (8) However, IFN-c is promiscuous with a pleiotropic mode of action.…”

Chemokine receptor CXCR3 deficiency exacerbates murine autoimmune cholangitis by promoting pathogenic CD8+ T cell activation

“…Primary biliary cholangitis (PBC) is a progressive autoimmune liver disease characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts [20–24]. The etiology of PBC remains enigmatic, but is linked by a combination of genetic susceptibility and environmental factors [25–28].…”

The Natural History and Prognosis of Primary Biliary Cirrhosis with Clinical Features of Autoimmune Hepatitis

“…There have been extraordinary and extensive studies of the genetic basis, the immunological features, and the molecular characteristics of not only human PBC but also of several mouse models [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21].…”