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Primary Biliary Cirrhosis

PBC

Primary biliary cirrhosis (PBC) is a chronic and progressive disease of the liver which involves destruction of the interlobular bile ducts.

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Presentation

In many cases, primary biliary cirrhosis causes no symptoms. However, in certain percentage of individuals this disease is known to cause the following signs and symptoms:

Easy Bruising
  • Patients may have a low platelet count because of an enlarged spleen causing easy bruising. Hepatic encephalopathy : The liver also helps remove waste products from the bloodstream.[medicinenet.com]
  • Cirrhosis can lead to easy bruising, nosebleeds, gallstones, intense itching, kidney failure and liver cancer. It is most commonly caused by alcoholism and hepatitis. Too much iron in the body causes hemochromatosis.[bshr.com]
  • bruising and abnormal bleeding, most often from swollen veins in the digestive tract Confusion or problems thinking Pale or clay-colored stools The health care provider will do a physical exam.[medlineplus.gov]
  • bruising and abnormal bleeding, most often from swollen veins in the digestive tract Confusion or problems thinking Pale or clay-colored stools Exams and Tests The health care provider will do a physical exam.[mountsinai.org]
Splenomegaly
  • MRI include: periportal 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[radiopaedia.org]
  • The multivariate analysis identified two independent predictive factors: low albumin level and splenomegaly ( Table 3 ).[nejm.org]
  • This may backup blood flow from other organs, causing splenomegaly , an enlarged spleen , and varices, swollen veins, especially in the esophagus and stomach that can predispose to bleeding.[medicinenet.com]
Fatigue
  • Fatigue is a significant problem in approximately 50% of primary biliary cirrhosis (PBC) patients, with 20% of all patients experiencing significant or life-altering fatigue.[ncbi.nlm.nih.gov]
  • This article presents a structured approach to managing fatigue in cholestatic liver disease to improve fatigue severity and quality of life.[ncbi.nlm.nih.gov]
  • The protocol will be valuable to research groups planning clinical trials targeting fatigue in PBC and also transferrable to other conditions associated with fatigue.[ncbi.nlm.nih.gov]
  • A verbal report of fatigue for at least 6 months was required for enrollment.[ncbi.nlm.nih.gov]
  • PBC is a cholestatic autoimmune liver disease in which fatigue is experienced by at least half of patients.[bmj.com]
Weakness
  • About 4 years earlier, she suffered from muscle weakness probably due to PM.[ncbi.nlm.nih.gov]
  • However, primary biliary cirrhosis (PBC) is known to cause muscular weakness. It is therefore critical to differentiate between PBC-associated muscular weakness and GBS in these patients.[academic.oup.com]
  • Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves.[ncbi.nlm.nih.gov]
  • We report the case of a 53-year-old patient who presented with weakness, myalgia, arthralgia, shortness of breath and purpura. Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia.[ncbi.nlm.nih.gov]
  • She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine.[ncbi.nlm.nih.gov]
Weight Loss
  • loss, abdominal pain symptoms secondary to late state cholestasis: -Pruritis (25-60%) -Episodic Jaundice (30 -70 %) -Acute cholangitis - relatively rare (5-25%) symptoms secondary to biliary cirrhosis: -portal HTN -liver failure Advanced liver disease[quizlet.com]
  • As it grows, symptoms may include pain in the upper abdomen on the right side, a lump in the upper abdomen, bloating, loss of appetite, weight loss, weakness, fever and yellow skin and eyes. These symptoms may be caused by other health problems.[bshr.com]
  • Early symptoms may include: Nausea and belly pain Fatigue and loss of energy Fatty deposits under the skin Fatty stools Itching Poor appetite and weight loss As liver function worsens, symptoms may include: Fluid buildup in the legs (edema) and in the[medlineplus.gov]
Anemia
  • Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive.[ncbi.nlm.nih.gov]
  • Secondary conditions such as hypothyroidism and anemia can develop as a result of PBC. It has been estimated that about 25% of patients who have suffered the disease for 10 years will ultimately develop liver failure.[symptoma.com]
  • Laboratorial data revealed anemia (hemoglobin 5 g/dl; reference: 12.0-19.0 g/dl); white blood cell and blood platelet count were normal. Vitamin B12 serum level was normal and antiparietal cell antibodies were absent, excluding pernicious anemia.[karger.com]
Fever
  • In addition to typical clinical findings as fever, hepatosplenomegaly, and cachexia, VL is associated with autoimmune phenomena.[ncbi.nlm.nih.gov]
  • A 47-year-old male was admitted with subacute onset of dry cough and fever. Chest tomography demonstrated multifocal areas of consolidation and ground glass attenuation.[ncbi.nlm.nih.gov]
  • A 40-year-old woman developed diarrhea, fever and arthralgia while receiving treatment for PBC. Colonoscopy revealed ileal erosion, a longitudinal ulcer in the ascending colon but only mild rectal inflammation.[ncbi.nlm.nih.gov]
  • Idiopathic granulomatous hepatitis is a rare disease of unknown cause which is characterized by recurrent fevers, sweats, elevated levels of liver enzyme tests, particularly the serum alkaline phosphatase, and granulomas in the portal and lobular regions[ncbi.nlm.nih.gov]
  • Symptoms include jaundice, dark urine, fever and flulike symptoms. It can be prevented with hepatitis A vaccine. A virus that causes liver disease and inflammation.[bshr.com]
Abdominal Pain
  • At 45 years of age, the patient complained of bloody diarrhea and abdominal pain, and she was diagnosed with ulcerative colitis by colonoscopy and histological examination.[ncbi.nlm.nih.gov]
  • We reported a 69-year-old female patient with abdominal pain, malaise, vertigo, headaches, hands tremor and partial loss of hearing.[ncbi.nlm.nih.gov]
  • A 65-year-old male was admitted to our hospital with jaundice, pruritus, mild abdominal pain and darkening urine. Serum alkaline phosphatase, γ-glutamyltransferase, bilirubin and IgG4 were prominently elevated.[ncbi.nlm.nih.gov]
  • We report a case of 69-year-old male with recurrent lower abdominal pain for 10 months.[ncbi.nlm.nih.gov]
  • However, in some it is known to cause abdominal pain, fatigue, liver enlargement, hepatitis, itching, deposition of fats under the skin and diarrhea. Diagnosis Blood work is done to determine the levels of serum enzymes and antibodies.[symptoma.com]
Diarrhea
  • After the pneumonia improved, she developed severe watery diarrhea. Although vancomycin was administered enterally, the diarrhea persisted. She died of multiple organ failure within 16 days of the onset of diarrhea.[ncbi.nlm.nih.gov]
  • At 45 years of age, the patient complained of bloody diarrhea and abdominal pain, and she was diagnosed with ulcerative colitis by colonoscopy and histological examination.[ncbi.nlm.nih.gov]
  • A 40-year-old woman developed diarrhea, fever and arthralgia while receiving treatment for PBC. Colonoscopy revealed ileal erosion, a longitudinal ulcer in the ascending colon but only mild rectal inflammation.[ncbi.nlm.nih.gov]
  • Modafinil was associated with minimal adverse events (headaches, diarrhea, and rash).[ncbi.nlm.nih.gov]
  • However, in certain percentage of individuals this disease is known to cause the following signs and symptoms: Dry eyes and mouth Fatigue Pruritus Development of jaundice Inflammation of the feet and ankles Diarrhea characterized by greasy stools Ascites[symptoma.com]
Clay-Colored Stool
  • stools The health care provider will do a physical exam.[medlineplus.gov]
  • […] or eyes (jaundice) Redness on the palms of the hands In men, impotence, shrinking of the testicles, and breast swelling Easy bruising and abnormal bleeding, most often from swollen veins in the digestive tract Confusion or problems thinking Pale or clay-colored[mountsinai.org]
Xerostomia
  • She was 38 years old and was admitted to our hospital for generalized pruritus, xerostomia, xerophthalmia, fatigue, and reticulonodular changes on her chest X-ray.[ncbi.nlm.nih.gov]
  • Pinto A: Management of xerostomia and other complications of Sjögren's syndrome. Oral Maxillofac Surg Clin North Am 2014;26:63-73.[karger.com]
  • Other findings may include the following: Hepatomegaly (25%) Hyperpigmentation (25%) Splenomegaly (15%) Jaundice (10%) Xanthelasma (10%) - In late stages of the disease Sicca syndrome (50-75%) - Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth)[emedicine.medscape.com]
  • Other common features include, pruritus, xerophthalmia and xerostomia; the last two are an integral part of Sicca syndrome which coexists in over 50% of cases of PBC.[prognosisapp.com]
  • They include xerostomia but also dental caries, gum infection and tracheobronchitis. Celiac disease is not so frequent but should be recognized because of the beneficial effect of the gluten free diet.[albi-france.org]
Jaundice
  • A 65-year-old male was admitted to our hospital with jaundice, pruritus, mild abdominal pain and darkening urine. Serum alkaline phosphatase, γ-glutamyltransferase, bilirubin and IgG4 were prominently elevated.[ncbi.nlm.nih.gov]
  • Life expectancy is about 5 years for symptomatic patients after the onset of jaundice.[medical-dictionary.thefreedictionary.com]
  • -Patients with rapidly progressive jaundice or recurrent cholangitis in the setting of PSC should undergo cholangiography to identify any of these three features.[quizlet.com]
  • Jaundice can be seen in late stages of the disease.[articles.chicagotribune.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]
Hepatomegaly
  • The multivariate analysis identified two independent predictive factors: high bilirubin level and hepatomegaly ( Table 3 ).[nejm.org]
  • […] mouth Fatigue Pruritus Development of jaundice Inflammation of the feet and ankles Diarrhea characterized by greasy stools Ascites Skin darkening Abdominal pain experienced in the upper right region Deposition of fat in areas around eyes and eyelids Hepatomegaly[symptoma.com]
  • […] proximal portion of biliary tree, the small bile ducts and canals of Hering; larger bile ducts affected only irregularly ( Hum Pathol 2002;33:983 ) 85% women, usually ages 40 - 60 Symptoms: insidious onset of pruritis, malaise, dark urine, light stools, hepatomegaly[pathologyoutlines.com]
  • Her bowel sounds are normal and hepatomegaly is present. A right upper quadrant ultrasound shows no evidence of extrahepatic biliary dilation.[step2.medbullets.com]
  • Physical examination is usually normal but may occasionally reveal hepatomegaly, xanthelasma, xanthoma, and features suggestive of portal hypertension such as splenomegaly and spider naevi.[prognosisapp.com]
Hepatosplenomegaly
  • In addition to typical clinical findings as fever, hepatosplenomegaly, and cachexia, VL is associated with autoimmune phenomena.[ncbi.nlm.nih.gov]
  • […] biliary cirrhosis History Asymptomatic (often diagnosed after incidental finding of abnormal liver function tests) Fatigue and lethargy Pruritus Abnormal bleeding or bruising Pale stool or dark urine Examination Jaundice Xanthelasma and tendon xanthomata Hepatosplenomegaly[oxfordmedicaleducation.com]
  • Physical examination reveals hepatosplenomegaly. Initial labs are notable for increased conjugated bilirubin, increased cholesterol, and increased alkaline phosphatase.[step2.medbullets.com]
  • […] phosphatase or γ-glutamyl transferase levels may be observed Fatigue (excess daytime sleepiness) is one of the earliest symptoms Itching may also be one of the earliest symptoms Anorexia, malaise (general discomfort or unease) Liver and spleen enlargement (hepatosplenomegaly[dovemed.com]
  • Simptomatologia bolii: initial, afectiunea este asimptomatica (doar colestaza este prezenta la analizele de laborator); ulterior, afectiunea se manifesta prin: prurit (mancarimea pielii), urmat de icter sclero-tegumentar; fatigabilitate; hepatosplenomegalie[endocrinopedia.info]
Scleral Icterus
  • Physical exam was remarkable for scleral icterus and mild jaundice; no xanthomas were noted. Laboratory studies were performed ( Table 1 ). Once more, the patient was found to have hyponatremia, along with hypokalemia and hypochloremia.[clinchem.aaccjnls.org]
Kayser-Fleischer Ring
  • Other findings may include the following: Hepatomegaly (25%) Hyperpigmentation (25%) Splenomegaly (15%) Jaundice (10%) Xanthelasma (10%) - In late stages of the disease Sicca syndrome (50-75%) - Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth) Kayser-Fleischer[emedicine.medscape.com]
  • […] following signs may be noted: Hepatomegaly (25%) Hyperpigmentation (25%) Splenomegaly (15%) Jaundice (10%) Xanthomas and Xanthelasmas (10%): In late stages of the disease Sicca syndrome (50-75%): Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth) Kayser-Fleischer[emedicine.medscape.com]
Pruritus
  • Pharmacological inhibition of IBAT with GSK2330672 may reduce BA levels in the systemic circulation and improve pruritus.[ncbi.nlm.nih.gov]
  • Intense pruritus can be itself an indication to liver transplantation, in extreme cases leading to suicide.[ncbi.nlm.nih.gov]
  • To demonstrate the efficacy of plasma exchange in primary biliary cirrhosis resistant to pharmacological treatment of pruritus. Review of medical records and scientific literature of plasma exchange and pruritus in primary biliary cirrhosis.[ncbi.nlm.nih.gov]
  • Patients who are non-responders to UDCA or have highly symptomatic disease (e.g., intractable pruritus) are thus in critical need of novel therapeutic approaches, which are both safe and effective.[ncbi.nlm.nih.gov]
  • The case of a 78-year-old man with PBC who presented with pruritus is discussed. Liver biopsy and antimitochondrial antibody serology were diagnostic of early-stage PBC.[ncbi.nlm.nih.gov]
Xanthoma
  • […] mouth Yellowing of the skin and eyes (jaundice) Darkening of the skin Swollen feet and ankles Accumulation of abdominal fluid (ascites) Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas[columbiasurgery.org]
  • 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]
  • […] of biliary tree, the small bile ducts and canals of Hering; larger bile ducts affected only irregularly ( Hum Pathol 2002;33:983 ) 85% women, usually ages 40 - 60 Symptoms: insidious onset of pruritis, malaise, dark urine, light stools, hepatomegaly, xanthomas[pathologyoutlines.com]
  • However, not all people with PBC develop xanthomas.[liversupport.com]
Hyperpigmentation
  • Clinical features include 12 : pruritus (often the first symptom) jaundice skin hyperpigmentation, especially of arms and trunk xanthomata and xanthelasma due to hypercholesterolaemia interestingly hypercholesterolaemia does not confer an increased risk[radiopaedia.org]
  • Hanot cirrhosis - a condition characterized by obstructive jaundice with hyperlipemia, pruritus, and hyperpigmentation of the skin. Synonym(s): primary biliary cirrhosis[medical-dictionary.thefreedictionary.com]
  • […] due to liver failure (ascites) Fatty deposits (xanthomas) on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees Yellowing of the skin and eyes (jaundice) Darkening of the skin that's not related to sun exposure (hyperpigmentation[mayoclinic.org]
  • Other findings may include the following: Hepatomegaly (25%) Hyperpigmentation (25%) Splenomegaly (15%) Jaundice (10%) Xanthelasma (10%) - In late stages of the disease Sicca syndrome (50-75%) - Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth)[emedicine.medscape.com]
Palmar Erythema
  • […] after incidental finding of abnormal liver function tests) Fatigue and lethargy Pruritus Abnormal bleeding or bruising Pale stool or dark urine Examination Jaundice Xanthelasma and tendon xanthomata Hepatosplenomegaly Features of chronic liver disease Palmar[oxfordmedicaleducation.com]
  • erythema, ascites, temporal and proximal muscle wasting, and peripheral edema American Liver Foundation.[emedicine.medscape.com]
  • Prominent spider angiomas and redness of the palms of the hands (palmar erythema) are usually present. delirium tremens may be a difficult problem during the early phase of hospitalization.[medical-dictionary.thefreedictionary.com]
  • erythema Ascites Temporal and proximal muscle wasting Peripheral edema See Clinical Presentation for more detail.[emedicine.medscape.com]
Epistaxis
  • Petechiae, epistaxis, or hemorrhage resulting from hypoprothrombinemia may also be evident. Pathological fractures and collapsed vertebrae may develop as the result of the associated malabsorption of vitamin D and calcium.[medical-dictionary.thefreedictionary.com]
Dark Urine
  • We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels.[ncbi.nlm.nih.gov]
  • Jaundice, dark urine, and darkening of the skin exposed to the sun occur as the liver disease progresses. The slow progression results eventually in portal hypertension, the accumulation of fluids, and eventually liver failure and death.[healthcentral.com]
  • Symptoms include jaundice, dark urine, fever and flulike symptoms. It can be prevented with hepatitis A vaccine. A virus that causes liver disease and inflammation.[bshr.com]
  • Symptoms of primary biliary cirrhosis (PBC) Primary biliary cirrhosis may not show any symptoms for years, but without treatment symptoms may progress to: Itching Fatigue Jaundice (yellowing of the skin and eyes) Dark urine Pale-colored stools Dry eyes[henryford.com]
Peripheral Neuropathy
  • Xanthomatous peripheral neuropathy may occur. American Liver Foundation. Primary biliary cholangitis (PBC, primary biliary cirrhosis). March 29, 2016. Available at . Accessed: June 3, 2016.[emedicine.medscape.com]

Workup

Blood tests to detect the levels of liver enzymes are done. The enzyme levels would give an indication of liver disease and extent of damage to bile ducts. In addition, blood work would also reveal the presence of anti-mitochondrial antibodies. The presence of antibodies in the blood would confirm PBC. This antibody test is considered to be a very important marker for PBC; as the antibodies do not show up in the blood tests of other liver conditions except this.

Imaging studies such as MRI and CT scan of the liver would have to be carried out to look for abnormalities in the organ. Biopsy of the liver tissue is also required to confirm the presence of PBC and to determine the extent of the disease [6]. The histopathologic findings from the biopsy may enable the pathologist to stage PBC and guide the course of treatment and predict clinical outlook [7].

Bilirubin Increased
  • Initial labs are notable for increased conjugated bilirubin, increased cholesterol, and increased alkaline phosphatase.[step2.medbullets.com]
Enlargement of the Spleen
  • Complications from Primary Biliary Cirrhosis can include hypersplenism (enlargement of the spleen), hepatic encephalopathy (confusion or coma resulting from liver failure), and esophageal varices (varicose veins in the esophagus which can rupture).[disability-benefits-help.org]
  • This may result in signs of portal hypertension such as: Blood in the stool Enlargement of the spleen Esophageal variceal bleeding (bleeding in enlarged veins of the lower esophagus) Development of ascites (fluid accumulation in the peritoneal cavity)[dovemed.com]
Hypertriglyceridemia
  • ., without hypertriglyceridemia, is a very rare cause of significant hyponatremia; all reported cases thus far have involved the presence of LpX ( 9 ).[clinchem.aaccjnls.org]
Transaminases Increased
  • A liver profile test panel showed mild increases in transaminases, increased alkaline phosphatase activity, hypoalbuminemia, significant hyperbilirubinemia, and evidence of cholestasis with increased bile acids in the blood. Table 1.[clinchem.aaccjnls.org]
Alkaline Phosphatase Increased
  • We had also observed in the other three groups of patients that the average of the values of γ-glutamyl transpeptidase and alkaline phosphatase increases with the increase of the value EliA-M2.[ncbi.nlm.nih.gov]
Immunoglobulin A Increased
  • Serum immunoglobulins were increased: IgG 1767 mg/dl, (952-1538); IgA 703 mg/dl (153-359) IgM 617 mg/dl (73-171). Erythrocyte sedimentation rate was 78 mm. Immunofluorescence was positive showing a granular cytoplasmic pattern (1:160).[scielo.br]
Liver Biopsy
  • Some blood biomarkers or histological examination by liver biopsy are used for the diagnosis of liver diseases in clinics. However, conventional blood biomarkers show poor specificity and sensitivity, and liver biopsy is highly invasiveness.[ncbi.nlm.nih.gov]
  • Since liver biopsy is an invasive method, liver biopsy tends to be replaced by noninvasive methods.[ncbi.nlm.nih.gov]
  • PBC patients with liver biopsy, esophagogastroscopy and Second Harmonic Generation (SHG)/Two-photon Excited Fluorescence (TPEF) microscopy images were retrospectively enrolled in this study. qFibrosis parameters in portal, septal and fibrillar areas were[ncbi.nlm.nih.gov]
  • This is a case report of acetylcholine receptor antibody positive generalized myasthenia gravis in a female patient with antimitochondrial antibody positive, liver biopsy-confirmed primary biliary cirrhosis.[ncbi.nlm.nih.gov]
  • Liver biopsy and antimitochondrial antibody serology were diagnostic of early-stage PBC.[ncbi.nlm.nih.gov]
Hepatocellular Carcinoma
  • carcinoma requiring liver transplantation.[ncbi.nlm.nih.gov]
  • The incidence of hepatocellular carcinoma (HCC) complicating primary biliary cirrhosis (PBC) is between 0.7% and 16%.[ncbi.nlm.nih.gov]
  • An adverse clinical outcome was defined as hepatic decompensation, development of hepatocellular carcinoma, liver transplantation, or liver-related death.[ncbi.nlm.nih.gov]
  • Findings with other imaging modalities such as ultrasonography, magnetic resonance imaging, and hepatic angiography were consistent with hepatocellular carcinoma.[ncbi.nlm.nih.gov]
  • Early diagnosis of hepatocellular carcinoma (HCC) is critical in the management of patients with primary biliary cirrhosis (PBC), since the prognosis of PBC has improved.[ncbi.nlm.nih.gov]

Treatment

The primary goal of treatment of PBC is to manage the symptoms and prevent the disease progression. Timely initiation of treatment would prevent the onset of complications. The following are the treatment methods employed to treat PBC:

  • Ursodeoxycholic acid (UDCA) is administered to help move the bile through the liver. It however does not cure PBC but helps primary management of the symptoms [8].
  • Medications to relieve symptoms of fatigue and pruritus are also administered.
  • Liver transplant is considered when the disease has reached its end stage. It is also the treatment of choice when other modes of treatments fail to show any effect. Liver transplant may also help prolong the life of the individuals.
  • Vitamin replacement therapy is also done in order to replenish the stores. Considerable amount of vitamins are lost in stools which may call for a deficiency to set in. A calcium supplement would also be required to strengthen the weakened bones.
  • Immunosuppressants like Rituximab has been used to control autoimmune response in PBC [9]. Chlorambucil have shown promising immunesuppressant action against serum bilirubin and immunoglobulin M [10].

Prognosis

Prognosis of the disease condition would majorly depend on how soon the diagnosis was made and treatment initiated. In many cases, patients die of liver cirrhosis as liver transplant was not done on time. Secondary conditions such as hypothyroidism and anemia can develop as a result of PBC. It has been estimated that about 25% of patients who have suffered the disease for 10 years will ultimately develop liver failure.

Complications

The following are the possible complication of PBC:

Etiology

The exact cause that triggers the development of such a condition is yet to be figured out. However, scientists believe that autoimmune disease condition causes such a disorder to develop. In this, the body regards its own cells as invaders and destroys them. Another factor that is thought to play foul includes infections caused by bacteria, parasites or viral agents. In addition, exposure to toxic compounds and smoking of cigarettes is also known to cause primary biliary cirrhosis.

Epidemiology

Primary biliary cirrhosis was thought to be a rare condition. However, recent statistics point towards the fact that about 1 in every 3000 to 4000 individuals is affected by this chronic liver disease. In addition, women are thought to be more affected by this disease than men with the women to men ratio being 9:1 due to exogenous estrogen secretions [1]. Mortality in primary biliary cirrhosis has been declining since the 1980’s because of advances in its therapeutic management [2].

Sex distribution
Age distribution

Pathophysiology

The process of PBC primarily begins with the accumulation of the T cells of the immune system that gradually destroy the cells of the bile ducts. Such a phenomenon occurs due to faulty response of the immune system wherein the immune cells of the body regards its own cells as invaders and tries to destroy them. Thus, primary biliary cirrhosis is considered to be an autoimmune condition.

It is also postulated that patients with PBC may harbor telomere dysfunction which may lead to chromosomal instability and cellular senescence [3]. In addition, PBC is also known to have a strong association with other autoimmune–mediated conditions such as scleroderma, telangiectasia syndrome, esophageal motility disorder, celiac disease, sclerodactyly and Raynaud phenomenon [4].

Prevention

So far there are no guidelines laid down for prevention of PBC. There is a strong association between alcohol and liver disease but in this case, alcohol consumption is not responsible for the condition.

Summary

Primary biliary cirrhosis (PBC) is a chronic disorder of the liver characterized by inflammation of the bile ducts. This condition gradually destroys the bile ducts of the organ thereby obstructing the flow of bile in the bloodstream. As a result, the cells of the liver get damaged leading to cirrhosis.

Bile juice produced by the bile ducts aids in the process of digestion of food and also helps the body get rid of cholesterol, toxins and worn out blood cells. Damage to these ducts ultimately interferes with food digestion and causes accumulation of toxicogenic compounds in the body leading to various secondary conditions.

Patient Information

Definition

Primary biliary cirrhosis abbreviated as PBC is a chronic liver disease that gradually damages the bile ducts. The bile ducts get inflamed and are destroyed in the long run. Women are more affected by this condition than men. 

Cause

The exact cause of PBC is not known; but autoimmune disorders are known to play foul. In this condition, the body’s immune system is known to attack the healthy liver cells causing PBC. It can also be linked to other autoimmune disorders such as thyroid disease, Sicca syndrome, celiac disease and Raynaud’s phenomenon.

Symptoms

In many cases, individuals with PBC do not suffer any symptoms. However, in some it is known to cause abdominal pain, fatigue, liver enlargement, hepatitis, itching, deposition of fats under the skin and diarrhea.

Diagnosis

Blood work is done to determine the levels of serum enzymes and antibodies. Imaging studies such as CT scan and MRI also need to be done in order to study the liver and the extent of damage. In many instances, biopsy of the liver tissue is also done to confirm diagnosis.

Treatment

Ursodeoxycholic acid is given to facilitate the removal of bile from the blood. In addition, medication such as colestipol is given to reduce the itching. Liver transplant may be necessary if all the above methods to treat the condition were not successful. 

References

Article

  1. McNally RJ, Ducker S, James OF. Are transient environmental agents involved in the cause of primary biliary cirrhosis? Evidence from space-time clustering analysis. Hepatology. Jun 24 2009
  2. Mendes FD, Kim WR, Pedersen R, et al. Mortality attributable to cholestatic liver disease in the United States. Hepatology. Apr 2008; 47(4):1241-7.
  3. Invernizzi P, Bernuzzi F, Lleo A, Pozzoli V, Bignotto M, Zermiani P. Telomere dysfunction in peripheral blood mononuclear cells from patients with primary biliary cirrhosis. Dig Liver Dis. 2014; 46(4):363-8 
  4. Solis Herruzo JA, Solis Munoz P, Munoz Yague T. The pathogenesis of primary biliary cirrhosis. Rev Esp Enferm Dig. Jun 2009; 101(6):413-23.
  5. Niro GA, Poli F, Andriulli A, Bianchi I, Bernuzzi F, Caliari L, et al. TNF-alpha polymorphisms in primary biliary cirrhosis: a northern and southern Italian experience. Ann N Y Acad Sci. Sep 2009; 1173:557-63.
  6. Drebber U, Mueller JJ, Klein E, Kasper HU, Schulze F, Schardt K, et al. Liver biopsy in primary biliary cirrhosis: clinicopathological data and stage. Pathol Int. Aug 2009; 59(8):546-54.
  7. Scheuer P. Primary biliary cirrhosis. Proc R Soc Med. Dec 1967;60(12):1257-60
  8. Rudic JS, Poropat G, Krstic MN, Bjelakovic G, Gluud C. Ursodeoxycholic acid for primary biliary cirrhosis.Cochrane Database Syst Rev. 2012; 12:CD000551
  9. Lazrak F, Abourazzak FE, Berrada K, Kadi N, Manssouri S, Harzy T. A rare association of rheumatoid arthritis and primary biliary cirrhosis treated with rituximab: a case report. J Med Case Rep. 2013; 7:99 
  10. Li WX, Yan X, Shi CR, Zhang AP. Chlorambucil for patients with primary biliary cirrhosis. Cochrane Database Syst Rev. 2012; 9:CD008714 

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Last updated: 2018-06-21 21:57