Primary Biliary Cholangitis: Pathogenesis, Treatment, and Management

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Student name
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Unit
Title: Primary Biliary Cholangitis

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Introduction
Liver cirrhosis is the final stage of liver fibrosis and is a result of different forms of liver
infections and conditions including chronic alcoholism, autoimmune liver disease, toxic metals,
viruses, chemicals and hepatitis. These diseases causing liver cirrhosis damage and kill the liver
cells whereby the inflammation and repair of the dead liver cells lead to formation of scar tissue.
The remaining live liver cells try to multiply so as to replace the dead cells, leading to
regenerative nodules on the scar tissue. Primary Biliary Cholangitis, also known as primary
biliary cirrhosis is a chronic liver disease that affects the intrahepatic bile ducts. Bile is used in
digestion and also important getting rid of toxins and cholesterol and also destroying worn out
red blood cells. Damaged bile ducts cause back up of bile in the liver leading to hepatic cirrhosis
and hence liver failure and death (Zhao et al. 2015). This disease is said to be autoimmune,
whereby the patient’s immune system mistakenly fights health tissues and cells. According to
Czul, Peyton and Levy (2013) primary biliary cholangitis is caused by a combination of
environmental and genetic factors. This study focuses on primary biliary cholangitis, its
pathogenesis, treatment and clinical management. I would also focus on the risk factors of this
disease.
Patient background
Pamela, a 42 year old lady is suffering from liver cirrhosis as a result of primary biliary
cholangitis. Pamela had been prescribed on Ulsodeoxychloric acid medication, which over time
did not show any improvement in her condition. Her bilirubin level remained high and her ALP
was 315. Silveira, Brunt, Healthcote, Gores and Mayo (2010) claim that Ulsodeoxychloric acid
(UDCA) is the only accepted treatment for primary biliary cholangitis though a research carried
out by Rudic, Porapat, Krstic, Bjelakovic and Gluud (2012) showed little success in the use of

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UDCA in the treatment of primary biliary cholangitis. Pamela frequently complained of being
tired, itching which caused her sleepless nights and experienced dry mouth despite drinking
amounts of water.
Pathophysiology
According to Gulamhusein and Hirschfield (2018) claim that the cause of biliary
cholangitis is not clear but most researchers claim it is just an autoimmune disease whereby the
body turns against its cells. The liver inflammation due to primary biliary cholangitis (PBC)
commences with the accumulation of the T cells in the liver. The T cells detect and fight against
disease causing microorganisms but in the case of primary biliary cholangitis the mistakenly
damage the healthy cells lining the bile ducts. According to Purohit and Cappell (2015) PCB is
common in women than in men, though this difference is not yet known but some researchers
related this to the increased number of X chromosomes monosomy in women suffering from
primary biliary cholangitis. Griffiths, Dyson and Jones (2014) claim that X chromosome
immunodeficiencies cause elevated levels of IgM and granuloma formation with both occurring
in PBC. Griffiths et al (2014) further think that this diseases mostly affects people aged around
50 years and it is more prevalent in the United States. Juran and Lazaridis (2014) relate the
difference in prevalence of PBC with detection bias, disease awareness and ease to access health
care, though Corpechot, Chretien, Chazouilleres and Poupon (2010) environmental factors and
genetic factors result to the difference in the disease prevalence. The environmental factors
include exposure to viruses, toxins, chemicals, bacteria and sunlight. Corpechot et al. (2010)
further claim that for unknown reasons PBC is more prevalent among people of socioeconomic
status.

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Smyk, Rigopoulou, Bogdanos (2013) srongly relate PBC with urinary tract infections.
In their study they claim that 48 percent of PBC patients had suffered from recurrent UTIs
before. They therefore studies the relationship between E. coli (leading cause of UTIs in women)
and PBC. They realized that E. coli triggers autoimmune response which in turn triggers cross
reactive response in T cells and B cells which occur in patients suffering from PBC. Studies by
Prince, Ducker and James (2010) showed a correlation between PBC and tobacco smoking. PBC
has a negative correlation with alcohol consumption. Prince et al. (2010) claim that people who
use hair dyes have a higher risk of contracting PBC though this contradicts with other
researchers.
According to Imam and Lindor (2014) claim that PBC has 4 stages: portal inflammation
(sometimes the inflammation has or lacks florid bile duct lesions), periportal lesions increase in
size with interface hepatitis, hepatic architecture become distorted with a lot of fibrous septa and
finally cirrhosis. Gulamhusein and Hirschfield (2018) anti-mitochondrial antibodies (AMAs) and
the breakdown of apical bicarbonate are some leads to the pathogenesis of this infection. The
AMAs attacks the pyruvate dehydrogenase complex on the inner BECs’ mitochondrial
membrane. Carey and Levy (2018) claim that this disease is characterized by circulation of
AMA and intrahepatic cholangiocytes autoimmune destruction to ductopenia and destruction of
the duct, portal fibrosis and then biliary cirrhosis. Just like other researchers they largely
associate PCB with environmental and genetic factors which cause breakdown of the patient’s
immune breakdown.
Carey and Levy (2018) claim that the male to female gender variation of this disease is
1:10 hence considered as a sexual dimorphism in autoimmunity. PBC has different risk factors.
Epidemiologic studies show that PBC is hereditary as proven by twin and familial aggregation