Edit concept Question Editor Create issue ticket

Biliary Cirrhosis

Liver Cirrhoses Biliary

Biliary cirrhosis is an autoimmune condition characterized by degeneration of the small and medium bile ducts and subsequent scarring of the liver parenchyma. It occurs due to an obstruction of the intrahepatic or extrahepatic bile flow and can be primary or secondary, depending on the etiology. Primary biliary cirrhosis is mainly viewed as an autoimmune disease, whereas secondary biliary cirrhosis is a result of underlying pathologies.


Presentation

Primary biliary cirrhosis (PBC)

Primary biliary cirrhosis is an autoimmune disease [1]. Approximately 1/4th of the patients affected by it tend to be asymptomatic when the diagnosis is made; the latter is established after abnormal laboratory values are detected during a routine examination or one conducted for different reasons [2]. The most common symptom experienced by patients is fatigue. The vast majority of the patients initially present with fatigue as the sole symptom and its existence increases the already elevated mortality rate of primary biliary cirrhosis [3] [4] [5] [6].

Pruritus is the second most common symptom experienced by up to half of the patients with an undiagnosed PBC and is believed to be caused by bile pigments which accumulate on the skin or by central opioid neurotransmission. Pain is reported, located in the right upper abdominal quadrant, in cases of advanced disease, jaundice also occurs.

With a reference to the signs of PBC, the most prominent include hepatomegaly, hyperpigmentation, splenomegaly, xanthelasma and signs related to cirrhosis and end-stage liver diseases, such as ascites and spider naevus.

Secondary biliary cirrhosis (SBC)

Similarly to PBC, secondary biliary cirrhosis tends to produce no symptoms in the initial stages and it is diagnosed when examinations are carried out for other medical reasons. When it does produce symptoms, those mainly include fatigue, jaundice, and pruritus. Pain in the right upper abdominal quadrant, hyperpigmentation, and osteoporotic symptoms can also complete the clinical picture and the patient may sometimes present in a febrile status.

Sjogren's syndrome is often associated with SBC and tends to present with a dry mouth and dry eyes. Night blindness and xanthelasma are common manifestations observed in a patient with secondary biliary cirrhosis.

Pain
  • Pain is reported, located in the right upper abdominal quadrant, in cases of advanced disease, jaundice also occurs.[symptoma.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 28, 2019[patientslikeme.com]
Italian
  • Invernizzi P, Battezzati PM, Crosignani A et al. (2003) Peculiar HLA polymorphisms in Italian patients with primary biliary cirrhosis. Journal of Hepatology 38(4): 401–406.[els.net]
  • Cordell HJ 1 , Han Y 2 , Mells GF 3 , Li Y 2 , Hirschfield GM 4 , Greene CS 5 , Xie G 6 , Juran BD 7 , Zhu D 2 , Qian DC 2 , Floyd JA 8, 9 , Morley KI 8, 10 , Prati D 11 , Lleo A 12 , Cusi D 13, 14 ; Canadian-US PBC Consortium ; Italian PBC Genetics Study[ncbi.nlm.nih.gov]
Camping
  • Although activation of A2bAR induced cAMP and intracellular Ca2 signals, only the Ca2 signals were linked to IL-6 upregulation.[ncbi.nlm.nih.gov]
Multiple Congenital Anomalies
  • Affected patients present a variable phenotype with pre- and post-natal macrosomia, distinctive facial dysmorphism, organomegaly, and multiple congenital anomalies. Intellectual disability is not constant.[ncbi.nlm.nih.gov]
Developmental Delay
  • Together with the associated risk of cancer and developmental delay, liver transplantation was discussed and then successfully performed at the age of 19 months. A hypothesis on the role of GPC3 in the patient's liver disease is also proposed.[ncbi.nlm.nih.gov]
Jaundice
  • When it does produce symptoms, those mainly include fatigue, jaundice, and pruritus.[symptoma.com]
  • Life expectancy is about 5 years for symptomatic patients after the onset of jaundice.[medical-dictionary.thefreedictionary.com]
  • Affecting chiefly middle-aged women, it is characterized by chronic cholestasis (accumulation of bile in the liver) with pruritis, jaundice, and hypercholesterolemia with xanthomas, and malabsorption.[healthcentral.com]
  • Other symptoms may eventually develop, including: jaundice, which leads to a yellowing of the eyes and skin fatty deposits under the skin fluid retention dry eyes and mouth In the later stages of the disease, some people develop osteoporosis, arthritis[oregonclinic.com]
Hepatomegaly
  • With a reference to the signs of PBC, the most prominent include hepatomegaly, hyperpigmentation, splenomegaly, xanthelasma and signs related to cirrhosis and end-stage liver diseases, such as ascites and spider naevus.[symptoma.com]
  • […] and D-penicil . age at registration in years. sex a factor with levels male and female . year number of years between enrollment and this visit date, remaining values on the line of data refer to this visit. ascites a factor with levels No and Yes . hepatomegaly[rdrr.io]
  • Itching of the skin Jaundice Dry Eye Dry mouth Portal hypertension Ascites Splenomegaly (enlarge spleen) Esophageal varices ( engorge vein in lower end of esophagus) Cholesterol deposits in skin- Xanthoma over arms and upper body Xanthoma around eyes Hepatomegaly[epainassist.com]
  • […] insidious/asymptomatic 20/22/6 41.7/45.8/12.5 Cirrhosis at presentation 17 35.4 Fatigue 29 60.4 Jaundice 27 56.3 Anorexia 24 50.0 Abdominal fullness 21 43.8 Splenomegaly 16 33.3 Abdominal pain 16 33.3 Ascites 12 25.0 Arthralgia 10 20.8 Edema 8 16.7 Hepatomegaly[slideshare.net]
Grieving
  • Jones S , Crossey M , Stansfield G , MacNicol C , Wilkins J , Wilhelmsen E , Raymode P , Lee HJ , Durant E , Bishop R , Ncube N , Tripoli S , Casey R , Cowley C , Miller R , Houghton K , Ducker S , Wright F , Bird B , Baxter G , Keggans J , Hughes M , Grieve[ncbi.nlm.nih.gov]
Excitement
  • Meanwhile, research on nuclear receptor hormones has led to the development of exciting new potential treatments.[ncbi.nlm.nih.gov]

Workup

Primary biliary cirrhosis

In the initial stages of the disease, liver functions tests are expected to reveal pathologically elevated levels of γ-glutamyl transpeptidase (γ-GT) and alkaline phosphatase (ALP) [7]. These two abnormal findings are usually the ones to be discovered during a routine examination and alongside a corresponding clinical picture with jaundice, fatigue and right upper abdominal quadrant pain, they raise suspicion towards PBC.

Aminotransferase levels are usually slightly elevated and bilirubin measured in the blood is also primarily normal. If the end-stage liver disease is present, serum bilirubin will be increased. The great majority of SBC patients are also found to have antimitochondrial autoantibodies (AMA) and those who test negative with the standard laboratory methods, tend to re-test positive when more sensitive methods are employed [8]. Serum immunoglobulin M (IgM) is also elevated in such patients. In order for a definitive diagnosis to be established, a liver ultrasonography and a magnetic resonance cholangiopancreatography (MRCP) or an endoscopic retrograde cholangiopancreatography (ERCP) are required. A liver biopsy will reveal findings compatible with PBC, such as inflammation of the bile ducts with depositions of intraepithelial lymphocytes and periductal epithelioid granuloma [9]. Through a liver biopsy, PBC can be classified into four distinct stages, depending on the particular histopathologic characteristics.

Secondary biliary cirrhosis

The diagnostic approach is similar to that of primary biliary cirrhosis. A thorough medical history is required, including a potential history of gallstones, surgical procedures, alcohol ingestion, and medications. Laboratory tests with include liver function tests in order to detect pathologic concentrations of ALP and γ-GT, serum bilirubin and aminotransferases. Plain radiographs and a liver biopsy will help to reach a definitive diagnosis of secondary biliary cirrhosis.

Treatment

  • This review provides a current perspective on potential new approaches to treatment in PBC, and highlights some of the challenges we face in evaluating and effectively implementing those treatments. Copyright 2016 Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • The elevated rectal lesion resolved with rituximab treatment.[ncbi.nlm.nih.gov]
  • Treatment of Esophageal Varices- Surgical treatment Treatment Of Liver Failure- Liver Transplant- The next and the only definitive treatment as of now is liver transplant.[epainassist.com]
  • Treatment for PBC There is no cure for PBC, but treatment can help slow the progression of disease and associated symptoms.[betterhealth.vic.gov.au]
  • Liver transplantation is the only treatment that will cure primary biliary cirrhosis. A liver transplant is considered when complications cannot be controlled by treatment.[british-liver-trust.org.uk]

Prognosis

  • The serum bilirubin level is a marker of prognosis Common Primary Biliary Cirrhosis (PBC) exam questions for medical students, finals, OSCEs and MRCP PACES Perfect revision for medical students, finals, OSCEs and MRCP PACES[oxfordmedicaleducation.com]
  • Early PBC prognosis advocated Biochemical response to ursodeoxycholic acid at 6 months is a better predictor for primary biliary cirrhosis prognosis than the current standard of 1 year, study results suggest.[news-medical.net]
  • […] hyperintensity (cuffing) segmental hypertrophy, notably of the caudate lobe hepatic surface irregularity due to regenerative nodules regional lymphadenopathy: tends to dominate in the gastrohepatic ligament and porta hepatis 4 splenomegaly Treatment and prognosis[radiopaedia.org]
  • Prince MI, Chetwynd A, Craig WL, et al ; Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort. Gut. 2004 Jun53(6):865-70.[patient.info]

Etiology

  • It occurs in patients who underwent liver transplantation for a different etiology. We present the case of a 56-year-old woman who was diagnosed with primary biliary cirrhosis and had liver transplantation for refractory pruritis.[ncbi.nlm.nih.gov]
  • However, the etiology of PBC is still unclear. Recently, the epithelial-mesenchymal transition (EMT) and interleukin-17A (IL-17A), a pro-inflammatory cytokine, were proposed to be involved in the pathogenesis of PBC.[ncbi.nlm.nih.gov]
  • It occurs due to an obstruction of the intrahepatic or extrahepatic bile flow and can be primary or secondary, depending on the etiology.[symptoma.com]
  • The etiology of PBC is poorly understood; however, several lines of evidence suggest an environmental factor that triggers a series of immune-mediated inflammatory reactions in the bile ducts in a genetically susceptible individual.[dovepress.com]
  • Its etiology is still not entirely understood, but clinical, laboratory and histopathologic findings strongly suggest an autoimmune process.[scielo.br]

Epidemiology

  • This Seminar will review the current understanding of the epidemiology, pathogenesis, and natural history of primary biliary cirrhosis, discuss management of the disease and its sequelae, and introduce research on new therapeutic options.[ncbi.nlm.nih.gov]
  • Prince MI, James OF ; The epidemiology of primary biliary cirrhosis. Clin Liver Dis. 2003 Nov7(4):795-819.[patient.info]
  • Epidemiology of primary biliary cirrhosis Prevalence 4 in 100,000 Males:Females 1:9 Peak incidence around 50 years Commonest in northern Europeans, least common in Africans.[oxfordmedicaleducation.com]
  • General Epidemiology: Female male ( 9:1). [1] Usually middle age. Associated with other autoimmune conditions ( Sjögren syndrome , progressive systemic sclerosis, celiac disease ). Etiology: Autoimmune.[librepathology.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology: Septal bile duct attacked. Treatment: Ursodeoxycholic acid. May be indication for transplant. Microscopic Features: "Florid duct lesion": [3] Intraepithelial lymphocytes - in bile duct - key feature .[librepathology.org]
  • Pathophysiology and recent findings of primary biliary cirrhosis complicated by systemic sclerosis. Ohira H1, Watanabe H. Hepatol Res. 2013 Dec 6. 2. Questionnaire based assessment of risk factors for primary biliary cirrhosis.[epainassist.com]
  • Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune[clevelandclinicmeded.com]
  • Reksten TR, Jonsson MV: Sjögren's syndrome: an update on epidemiology and current insights on pathophysiology. Oral Maxillofac Surg Clin North Am 2014;26:1-12.[karger.com]
  • Pathophysiology 9 Kaplan MM, Gershwin ME. N Engl J Med 2005;353:1261-73 10.[slideshare.net]

Prevention

  • A genetic disease that prevents the body from removing extra copper. Having high copper levels can cause life-threatening damage to the body’s organs.[bshr.com]
  • Can primary biliary cirrhosis be prevented? Since the cause of the disease is not known, it is not possible to prevent PBC. However, medications may be able to slow the progression of the disease and control the symptoms.[medicinenet.com]
  • UDCA is thought to help prevent liver damage by changing the composition of the bile and reducing inflammation (swelling) of the liver.[hse.ie]

References

Article

  1. Jones DE. Pathogenesis of primary biliary cirrhosis. Gut. 2007;56:1615-1624.
  2. Prince MI, Chetwynd A, Craig WL, et al. Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort. Gut. 2004 Jun; 53(6):865-70.
  3. Prince M, Chetwynd A, Newman W, et al. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology. 2002; 123:1044-1051.
  4. Jones EA. Fatigue complicating chronic liver disease. Metab Brain Dis. 2004 Dec; 19(3-4):421-9.
  5. Forton DM, Patel N, Oatridge A, et al. Fatigue in primary biliary cirrhosis. Gut. 2005 Mar; 54(3):438.
  6. Jones DE, Bhala N, Burt J, et al. Four year follow up of fatigue in a geographically defined primary biliary cirrhosis patient cohort. Gut. 2006 Apr; 55(4):536-41.
  7. Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. Primary biliary cirrhosis. July 2009. Hepatology. 50 (1): 291–308.
  8. Vierling, JM. Primary biliary cirrhosis and autoimmune cholangiopathy. Clinics in liver disease. Feb 2004. 8 (1): 177–94.
  9. Nakanuma Y, Tsuneyama K, Sasaki M, Harada K. Destruction of bile ducts in primary biliary cirrhosis. Aug 2000. Baillière's Best Practice & Research. Clinical Gastroenterology. 14 (4): 549–70.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-06-28 11:54