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:

• Dry eyes and mouth
• Fatigue [5]
• 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

• 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]

  Fatigue (usually the first symptom) Marked generalized pruritus Hyperpigmentation Hepatomegaly, dull lower margin, RUQ discomfort Splenomegaly Jaundice Maldigestion (may involve manifestations of deficiency of fat-soluble vitamins, e.g., osteoporosis) [amboss.com]

  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]

• 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]

• Fatigue

  This article presents a structured approach to managing fatigue in cholestatic liver disease to improve fatigue severity and quality of life. Copyright © 2016 Elsevier Inc. All rights reserved. [ncbi.nlm.nih.gov]

  Clinically, fatigue has both central and peripheral components. [karger.com]

  Modafinil appears to be a safe, effective treatment for PBC-related fatigue. Keywords Modafinil Primary biliary cirrhosis Fatigue PBC This is a preview of subscription content, log in to check access. References 1. Kaplan MM, Gershwin ME. [doi.org]

• 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]

  Antimitochondrial antibodies may also be detected in patients with diffuse muscle weakness and mitochondrial abnormalities. [scielo.br]

  A calcium supplement or other bone medicines may be added to prevent or treat weak or soft bones. Long-term monitoring and treatment of liver failure is needed. Liver transplant may be successful if it is done before liver failure occurs. [nlm.nih.gov]

• Weight Loss

  […] and Inflammatory bowel disease -75 % have ulcerative colitis -5% have Crohn's -2.5 -7.5% of patients with ulcerative colitis have PSC PSC associated with multiple other autoimmune type diseases PSC - Clinical Presentation -Asymptomatic >10%* -Fatigue, weight [quizlet.com]

  loss Dark-colored urine Get useful, helpful and relevant health + wellness information enews Cleveland Clinic is a non-profit academic medical center. [my.clevelandclinic.org]

  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 [nlm.nih.gov]

  Its typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain[ 1 ]. [doi.org]

  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]

• Anemia

  Autoimmune hemolytic anemia as a complication of primary biliary cirrhosis [Turk J Hematol] Turk J Hematol. 2004; 21(3): 153-155 Autoimmune hemolytic anemia as a complication of primary biliary cirrhosisCarlos R. De Medeiros, Daniela C. [tjh.com.tr]

  Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. [ncbi.nlm.nih.gov]

  Autoimmune hemolytic anemia as a complication of primary biliary cirrhosisCarlos R. De Medeiros, Daniela C. [journalagent.com]

  Its typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain[ 1 ]. [doi.org]

  Other diseases, such as hypothyroidism and anemia, can also develop. Progressive cirrhosis can lead to liver failure. [nlm.nih.gov]

• Fever

  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]

  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]

  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]

  Definition Drug-induced diarrhea is loose, watery stools that occurs when you take certain medicines. Alternative Names Diarrhea associated with medicines Causes Nearly all medicines may cause diarrhea as a side effect. [mclaren.org]

  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]

• Right Upper Quadrant Pain

  Right upper quadrant pain or discomfort: occurs in 17%[3]. At a later stage the patient may present with jaundice of cholestatic origin with dark urine and pale stool. Sjögren's syndrome is commonly present, with dry eyes and dry mouth. [patient.info]

  Right upper quadrant pain e. Bone pain 4.  Which of the following medications have not been used in trials of prognostic benefit in PBC? a. Cyclosporin A b. Ursodeoxycholic acid c. Colchicine d. Methotrexate e. Rifampicin 5. [pmj.bmj.com]

  Approximately 10% of patients complain of vague intermittent right upper quadrant pain or discomfort, which usually resolves spontaneously [ 28 ]. Physical examination Physical examination may be normal in those with early-stage disease. [pubmedcentral.nih.gov]

• Clay-Colored Stool

  […] the digestive tract Confusion or problems thinking Pale or clay-colored stools The health care provider will do a physical exam. [nlm.nih.gov]

  […] 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]

• 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

  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]

  In advanced disease, increased fibrotic changes lead to typical signs of cholestasis (e.g., jaundice) and portal hypertension (e.g., ascites, gastrointestinal bleeding). [amboss.com]

  […] 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 abdomen (ascites) Yellow color in the skin, mucous membranes, or eyes (jaundice [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]

• Hepatomegaly

  Fatigue (usually the first symptom) Marked generalized pruritus Hyperpigmentation Hepatomegaly, dull lower margin, RUQ discomfort Splenomegaly Jaundice Maldigestion (may involve manifestations of deficiency of fat-soluble vitamins, e.g., osteoporosis) [amboss.com]

  The multivariate analysis identified two independent predictive factors: high bilirubin level and hepatomegaly ( Table 3 ). [nejm.org]

  […] 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]

  […] 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]

  In later phases of the disease, patients may develop hepatomegaly, xanthomas/xanthalasmas, melanotic patches, or evidence of portal hypertension (splenomegaly, ascites, edema, encephalopathy). [cancertherapyadvisor.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]

  […] 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]

  Physical examination at initial presentation may be normal, although jaundice and hepatosplenomegaly are present in up to 50% of patients. [clevelandclinicmeded.com]

  With progressive liver failure, ascites and hepatosplenomegaly may occur, as well as blood derangements such as hypoalbuminemia and prolonged coagulation times. [consultant360.com]

• 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.org]

• Fetor Hepaticus

  This may be visible as an increase in abdominal girth.[13] Fetor hepaticus is a musty breath odor resulting from increased dimethyl sulfide.[14] Jaundice, or icterus is yellow discoloration of the skin and mucous membranes, (with the white of the eye [en.wikipedia.org]

• Arthralgia

  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]

  A trial of corticosteroids in patients with idiopathic granulomatous hepatitis who are symptomatic, with fever, myalgias, and arthralgias, may be helpful. Empirical antituberculous therapy should be considered before instituting corticosteroids. [clevelandclinicmeded.com]

  As well as sicca symptoms, cold hands, muscle weakness and arthralgia were all associated with more traditional ‘symptoms’ of PBC. [doi.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.com]

• Pruritus

  Pharmacological inhibition of IBAT with GSK2330672 may reduce BA levels in the systemic circulation and improve pruritus. [ncbi.nlm.nih.gov]

  Chronic pruritus. N Engl J Med 2013;368:1625-1634. Paus R, Schmelz M, Bíró T, Steinhoff M: Frontiers in pruritus research: scratching the brain for more effective itch therapy. J Clin Invest 2006;116:1174-1186. [karger.com]

• Xanthoma

  Xanthoma formation, however, is not a very common sign in this disorder. [doi.org]

  अंग्रेज़ी शब्द जिसकी XANTHOMA के साथ तुकबंदी है अंग्रेज़ी में xanthoma के पर्यायवाची और विलोम «xanthoma» शब्द का 25 भाषाओं में अनुवाद का अनुवाद XANTHOMA हमारे अंग्रेज़ी बहुभाषी अनुवादक के साथ xanthoma का 25 भाषाओं में अनुवाद ढूंढ़ें।. [educalingo.com]

  […] 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]

  Fatigue (usually the first symptom) Marked generalized pruritus Hyperpigmentation Hepatomegaly, dull lower margin, RUQ discomfort Splenomegaly Jaundice Maldigestion (may involve manifestations of deficiency of fat-soluble vitamins, e.g., osteoporosis) Xanthomas [amboss.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]

• Hyperpigmentation

  Clinical features include 12: pruritus (often the first symptom) jaundice skin hyperpigmentation, especially of arms and trunk xanthomata and xanthelasma due to hypercholesterolemia interestingly hypercholesterolemia does not confer an increased risk [radiopaedia.org]

  Fatigue (usually the first symptom) Marked generalized pruritus Hyperpigmentation Hepatomegaly, dull lower margin, RUQ discomfort Splenomegaly Jaundice Maldigestion (may involve manifestations of deficiency of fat-soluble vitamins, e.g., osteoporosis) [amboss.com]

  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]

  The most common symptom of PBC is fatigue, affecting 80 percent of people with the disease. ( 4 ) Other signs include these symptoms: Severe itching (pruritus) Dry skin Patches of darkened skin (hyperpigmentation) Jaundice (yellowing of the skin and eyes [everydayhealth.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]

  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, and peripheral edema American Liver Foundation. [emedicine.medscape.com]

  erythema Ascites Temporal and proximal muscle wasting Peripheral edema See Clinical Presentation for more detail. [emedicine.com]

• Yellow Discoloration of the Skin

  When PBC is very severe, it can lead to yellow discoloration of the skin (jaundice). If PBC is untreated, it can lead to cirrhosis, or scarring of the entire liver, which can lead to liver failure. [rarediseases.org]

  This may be visible as an increase in abdominal girth.[13] Fetor hepaticus is a musty breath odor resulting from increased dimethyl sulfide.[14] Jaundice, or icterus is yellow discoloration of the skin and mucous membranes, (with the white of the eye [en.wikipedia.org]

• 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]

• Peripheral Neuropathy

  Peripheral neuropathy and other autoimmune disorders associated with PBC may also develop. [merckmanuals.com]

  Peripheral neuropathy and other autoimmune disorders associated with primary biliary cholangitis may also develop. [msdmanuals.com]

  Xanthomatous peripheral neuropathy may occur. American Liver Foundation. Primary biliary cholangitis (PBC, primary biliary cirrhosis). March 29, 2016. Available at http://www.liverfoundation.org/abouttheliver/info/pbc/. Accessed: June 3, 2016. [emedicine.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 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]

  […] disease Involves most 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 [pathologyoutlines.com]

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

  Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(+) patients (26% vs. 5 %, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. [ncbi.nlm.nih.gov]

  Among patients with early disease, bilirubin increased to >2 mg/dL in the anti‐NPC(+) patients (26% vs. 5%, P = .019). Anti‐NPC antibodies remained stable or slightly increased over the period of observation. [dx.doi.org]

• Hypertriglyceridemia

  Further supporting this idea is that hypercholesterolemia on its own, i.e., without hypertriglyceridemia, is a very rare cause of significant hyponatremia; all reported cases thus far have involved the presence of LpX ( 9 ). [clinchem.org]

  «MataWanita.com, मई 15» Severe hypertriglyceridemia 환자에서 fenofibrate 처방 증례 신체 검사상 무릎 주위에도 비슷한 병변이 다수 관찰되었고, 피부과에서 조직검사를 시행하여 발진형 황색종(eruptive xanthoma)으로 진단되었다. 진단 후 환자는 ... [educalingo.com]

  Fibrates are licensed for the treatment of hypertriglyceridemia. They also exert potent anticholestatic effects. [en.wikipedia.org]

  NAFLD is closely associated with features of the metabolic syndrome such as insulin resistance, obesity, type II diabetes and hypertriglyceridemia ( 51 ). [doi.org]

• Antinuclear Autoantibodies

  Seldin, Xiangdong Liu, Liangdan Sun and Xiong Ma, Genome‐wide Association Studies of Specific Antinuclear Autoantibody Subphenotypes in Primary Biliary Cholangitis, Hepatology,, (2019). Gary L. [dx.doi.org]

  Yannick Chantran, Eric Ballot and Catherine Johanet, Autoantibodies in primary biliary cirrhosis: Antinuclear envelope autoantibodies, Clinics and Research in Hepatology and Gastroenterology, 38, 3, (256), (2014). Christopher L. Bowlus and M. [doi.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.org]

• Globulins Increased

  Findings include elevations in serum globulins, especially gamma globulins.[13] Hereditary hemochromatosis. [en.wikipedia.org]

• 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]

• 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 can result in the following: Development of varices or enlarged blood vessels - the enlarged blood vessels can rupture and lead to internal bleeding within the gastrointestinal tract The spleen can become enlarged, if too many red blood cells and [dovemed.com]

  Other liver findings suggestive of cirrhosis in imaging are an enlarged caudate lobe, widening of the fissures and enlargement of the spleen. [en.wikipedia.org]

• Liver Biopsy

  KEYWORDS: Anti-mitochondrial M2 antibody; Chronic inflammatory demyelinating polyneuropathy; Liver biopsy; Primary biliary cirrhosis [ncbi.nlm.nih.gov]

  […] evidence on liver biopsy At least 2 of the following: ALP >2 x ULN or GGT >5 x ULN AMA titer >1:40 Florid bile duct lesions on liver biopsy And at least 2 of the following: ALT >5 x ULN IgG >2 x ULN or SMA positivity Liver biopsy with moderate or severe [arupconsult.com]

  Primary biliary cirrhosis is diagnosed through laboratory tests, x rays, and in some cases, a liver biopsy to help to determine the extent of disease progression. A liver biopsy uses a thin needle to remove a small sample of liver tissue. [oregonclinic.com]

• Hepatocellular Carcinoma

  Ryuta Ide, Akihiko Oshita, Takashi Nishisaka, Hideki Nakahara, Shiomi Aimitsu and Toshiyuki Itamoto, Primary biliary cholangitis metachronously complicated with combined hepatocellular carcinoma-cholangiocellular carcinoma and hepatocellular carcinoma [doi.org]

  About 10% of patients presented with complications such as ascites (10.3%), variceal bleeding (5.8%) and/or hepatocellular carcinoma (HCC) (1.3%). Liver transplantation was undertaken in 71 patients (2.5%) for 5 years. [ncbi.nlm.nih.gov]

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 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:

• Malabsorption due to improper absorption of nutrients
• Malnutrition
• Kidney failure
• Bleeding 
• Electrolyte imbalance
• Fluid imbalance
• Encephalopathy 
• Osteomalacia or osteoporosis

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.

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].

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].

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.

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.

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. 

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.
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