primary biliary cirrhosis

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Primary Biliary Cirrhosis

 

Definition

Primary biliary cirrhosis is the gradual destruction of the biliary system for unknown reasons.

Description

Although the cause of this serious condition is not known, it has many features to suggest that it is an autoimmune disease. Autoimmunity describes the process whereby the body's defense mechanisms are turned against itself. The immune system is supposed to recognize and attack only dangerous foreign invaders like germs, but many times it attacks, for no apparent reason, the cells of the body itself. Autoimmune reactions occur in many different tissues of the body, creating a great variety of diseases.
Primary biliary cirrhosis progressively destroys the system that drains bile from the liver into the intestines. Bile is a collection of waste products excreted by the liver. As the disease progresses it also scars the liver, leading to cirrhosis. In some patients, the disease destroys the liver in as little as five years. In others, it may lie dormant for a decade or more.

Causes and symptoms

Ninety percent of patients with this disease are women between the ages of 35 and 60. The first sign of it may be an abnormal blood test on routine examination. Itching is a common early symptom, caused by a buildup of bile in the skin. Fatigue is also common in the early stages of the disease. Later symptoms include jaundice from the accumulation of bile and specific nutritional deficiencies-bruising from vitamin K deficiency, bone pain from vitamin D deficiency, night blindness from vitamin A deficiency, and skin rashes, possibly from vitamin E or essential fatty acid deficiency. All these vitamin problems are related to the absence of bile to assist in the absorption of nutrients from the intestines.

Diagnosis

Blood tests strongly suggest the correct diagnosis, but a liver biopsy is needed for confirmation. It is also usually necessary to x ray the biliary system to look for other causes of obstruction.

Treatment

Of the many medicines tried to relieve the symptoms and slow the progress of this disease, only one has had consistently positive results. Ursodeoxycholic acid, a chemical that dissolves gall stones, provides substantial symptomatic relief. It is still unclear if it slows liver damage.
Primary biliary cirrhosis is a major reason for liver transplantation. Patients do so well that this is becoming the treatment of choice. As experience, technique, and immunosuppression progressively improve, patients with this disease will come to transplant surgery earlier and earlier in their disease course.

Prognosis

So far, this disease has not returned in a transplanted liver.

Resources

Organizations

American Liver Foundation. 1425 Pompton Ave., Cedar Grove, NJ 07009. (800) 223-0179. http://www.liverfoundation.org.

Key terms

Biopsy — Surgical removal of tissue for examination.
Cirrhosis — Scarring, usually referring to the liver.
Immunosuppression — Techniques to prevent transplant graft rejection by the body's immune system.

cirrhosis

 [sĭ-ro´sis]
a liver disease (actually a group of chronic diseases) characterized by loss of the normal microscopic lobular architecture and regenerative replacement of necrotic parenchymal tissue with fibrous bands of connective tissue that eventually constrict and partition the organ into irregular nodules. It has a lengthy latent period, usually followed by the sudden appearance of abdominal pain and swelling, hematemesis, dependent edema, or jaundice. In advanced stages, ascites, pronounced jaundice, portal hypertension, and central nervous system disorders, which may end in hepatic coma, become prominent. adj., adj cirrhot´ic.
Clinical Manifestations. The signs and symptoms are manifestations of interference with the major functions of the liver: (1) the storage and release of blood to maintain adequate circulating volume, (2) the metabolism of nutrients and the detoxification of poisons absorbed from the intestines, (3) the regulation of fluid and electrolyte balance, and (4) production of clotting factors.

The patient with alcoholic cirrhosis (Laënnec's cirrhosis) may be admitted to the hospital with acute alcoholic hepatitis, marked by fever and dehydration. 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. Data from liver function tests usually show elevated transaminase levels, elevated bilirubin levels, and decreased values for albumin and clotting factors. (See also alcoholism.)

Continued fluid and electrolyte imbalances and inefficient metabolism of nutrients produce ascites, hypoglycemia, and hypoproteinemia. Obstruction to the return of blood from the portal system causes increased pressure within the veins of the esophagus and stomach. These engorged vessels are subject to rupture with subsequent hemorrhage that is abetted by clotting disorders. jaundice develops as a result of biliary obstruction.

Neurological symptoms begin with subtle changes in mental acuity, mild memory loss, poor reasoning ability, and irritability. Tremor of the outstretched hands (asterixis) is common. These symptoms become more severe and may eventually progress to delirium, suicidal tendencies, and coma.
Treatment. The major goals of treatment for the patient with cirrhosis are: (1) to maintain liver function at its current level and prevent further deterioration of the organ, (2) to maintain electrolytes within normal limits, (3) to maintain sufficient respiratory function, (4) to prevent or resolve gastrointestinal bleeding, and (5) to provide adequate nutritional intake and a positive nitrogen balance.

Since there is no cure for cirrhosis, supportive measures are instituted to help the liver rebuild and repair its damaged cells. Prevention of further deterioration of the cells is accomplished by removing the primary cause; for example, restriction of the intake of alcohol or other toxic agent, treatment of infection, and providing an adequate nutritional intake; supplemental vitamins are often prescribed.

Severe blood loss is compensated for by transfusions of whole blood. Excessive bleeding from esophageal varices may necessitate insertion of a Sengstaken-Blakemore tube. This device has three channels: one for inflation of the esophageal balloon, one for inflation of the gastric balloon, and a third for aspiration of stomach contents.

Relief of portal hypertension sometimes is accomplished by a surgical procedure called a portacaval shunt. The portal vein is surgically connected to the inferior vena cava to allow drainage of excessive amounts of blood from the portal system to the general circulation. A similar procedure called the splenorenal shunt involves connecting the splenic vein to the renal vein.

Removal of fluid from the abdominal cavity (abdominal paracentesis) may be necessary to relieve respiratory embarrassment or pressure on the abdominal organs caused by ascites. A more permanent resolution of the problem of chronic ascites is surgical creation of a peritoneovenous shunt.

Hepatic encephalopathy and coma can be precipitated by any of a number of factors, including gastrointestinal bleeding, fluid and electrolyte and acid-base imbalances, intercurrent infection and fever, administration of analgesics and sedatives that are central nervous system depressants, and increased dietary protein intake.

Patients with chronic hepatic encephalopathy are placed on a protein-restricted diet. An antibiotic may be prescribed to reduce bacterial flora in the intestine. Surgical removal of all or part of the bacteria-laden colon is an alternative treatment, but the surgical risk is high in these chronically ill patients.
Patient Care. In view of the many functions of the liver, it is essential that a thorough assessment of the patient be done to identify specific problems before a plan of care is developed. Among the problems likely to be associated with cirrhosis are self-care deficit related to low energy level due to inadequate metabolism of carbohydrates; fluid volume excess, especially ascites; potential for impairment of skin integrity related to edema, jaundice, and itching; electrolyte imbalance related to poor storage of minerals; alteration in comfort; tendency to bleed excessively related to deficits of vitamin K and prothrombin and the presence of esophageal varices; potential for infection related to decreased levels of gamma globulins and dysfunction of phagocytic Kupffer cells; impaired gas exchange related to pressure against the lungs by ascitic fluid; potential for injury related to altered levels of consciousness; alteration in nutrition related to indigestion, nausea, inability of liver cells to metabolize food elements, or confusion and depression; and diarrhea related to diminished bile production and decreased tolerance to fatty acids.

Patients in the advanced stages of cirrhosis require periodic and thorough monitoring to detect blood loss in the form of hematemesis, tarry stools, bleeding gums, frequent and heavy nosebleeds, and bruising. In order to evaluate fluid status the fluid intake and output and daily weight are measured and recorded.

Dietary restrictions and prohibition of alcohol may result in noncompliance in some patients. Education must include the purpose of these restrictions, the expected effect and dosage of medications that have been prescribed, and the importance of adequate nutrition, rest, and preservation of independence within the patient's capabilities. Compliance may be improved by enhancing the patient's self-esteem, emphasizing personal strengths, encouraging the use of available community resources such as alcoholics anonymous if alcoholism is a problem, and providing for active participation of the patient and family in planning and implementing some aspects of care.
acholangic cirrhosis a liver disorder affecting children up to 12 years of age, due to complete or partial agenesis of the intrahepatic, intralobular bile ducts, with manifestations similar to those seen in obstructive biliary cirrhosis.
alcoholic cirrhosis Laënnec's cirrhosis.
atrophic cirrhosis cirrhosis in which the liver is decreased in size; it may be seen in the alcoholic, but is more common in posthepatic or postnecrotic cirrhosis.
biliary cirrhosis cirrhosis caused by obstruction or infection of the major extra- or intrahepatic bile ducts (except in primary biliary cirrhosis). It is marked by jaundice, abdominal pain, steatorrhea, and enlargement of the liver and spleen.
cardiac cirrhosis cirrhosis complicating heart disease, with recurrent intractable congestive heart failure. Called also cardiocirrhosis.
congestive cirrhosis cirrhosis resulting from increased hepatic venous pressure or thrombosis; commonly due to congestive heart failure (see cardiac cirrhosis) or to obstruction of the hepatic vein.
fatty cirrhosis cirrhosis in which liver cells are infiltrated with fat (triglyceride), the infiltration usually being due to alcohol ingestion; see laënnec's cirrhosis.
Laënnec's cirrhosis cirrhosis associated with chronic alcoholism. In the early stages, liver enlargement may reflect fatty infiltration of liver cells (fatty cirrhosis) with necrosis and inflammation due to acute alcohol injury; progressive fibrosis extending from portal areas separates uniform small regeneration nodules. Some attribute the condition to a nutritional deficiency associated with alcoholism and others to chronic exposure to alcohol as a hepatotoxin. Called also alcoholic cirrhosis.
macronodular cirrhosis morphological changes that cause the liver to become small and shrunken.
metabolic cirrhosis cirrhosis associated with metabolic diseases such as hemochromatosis, wilson's disease, glycogen storage disease, galactosemia, and disorders of amino acid metabolism.
micronodular cirrhosis morphological changes in the liver resulting in an enlarged liver.
mixed cirrhosis morphological changes in the diseased liver that represent both the micronodular and macronodular patterns.
portal cirrhosis Laënnec's cirrhosis.
posthepatic cirrhosis cirrhosis (usually macronodular) resulting as a sequel to acute hepatitis.
postnecrotic cirrhosis cirrhosis following submassive necrosis of the liver (subacute yellow atrophy) due to toxic or viral hepatitis.
primary biliary cirrhosis a rare form of biliary cirrhosis of unknown etiology, occurring without obstruction or infection of the major bile ducts, sometimes developing after the administration of such drugs as chlorpromazine and arsenicals. Affecting chiefly middle-aged women, it is characterized by chronic cholestasis with pruritus, jaundice, and hypercholesterolemia with xanthomas, and malabsorption.
secondary biliary cirrhosis cirrhosis resulting from chronic bile obstruction due to congenital atresia or stricture.

pri·mar·y bil·i·ar·y cir·rho·sis

[MIM*109720]
a condition occurring mainly in middle-aged women, characterized by cholestasis with hyperlipemia, pruritus, and hyperpigmentation of the skin; no obstruction of large bile ducts or proliferation of small bile ducts is found; the liver shows cirrhosis with marked portal infiltration by lymphocytes and plasma cells, and frequently by epithelioid cell granulomas; serum antimitochondrial antibodies are present in 85-90% of patients.
Synonym(s): Hanot cirrhosis

primary biliary cirrhosis

n. Abbr. PBC
A chronic inflammatory disease of the liver in which the bile ducts are gradually destroyed and cirrhosis develops, thought to be an autoimmune disorder.

primary biliary cirrhosis

a chronic inflammatory condition of the liver. It is characterized by generalized pruritus; enlargement and hardening of the liver; weight loss; and diarrhea with pale, bulky stools. 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. Xanthomas commonly develop when the serum cholesterol level exceeds 450 mg/dL. The cause of primary biliary cirrhosis is unknown, although it is associated with autoimmune disorders. The condition most often affects women 40 to 60 years of age. The diagnosis is confirmed by liver biopsy and cholangiography. Antibody is nearly always present. Jaundice, dark urine, pale stools, and cutaneous xanthosis may occur in the later stages of this disease. Treatment commonly includes the administration of fat-soluble vitamins A, D, E, and K to prevent and correct deficiencies caused by malabsorption. Life expectancy is about 5 years for symptomatic patients after the onset of jaundice. Compare secondary biliary cirrhosis.

primary biliary cirrhosis

A disease of ♀ age 30 to 65 Clinical Fatigue, pruritis, steatorrhea, hepatic osteodystrophy, renal tubular acidosis, 4-fold ↑ in liver and breast CAs; 80% of PBC have autoimmune or connective tissue disease–eg, autoimmune thyroiditis, scleroderma, rheumatoid arthritis, Sjögren–sicca syndrome Lab 20-50-fold ↑ alk phos, IgM antimitochondrial antibodies; anti-SS-A/Ro antibodies in 20% Management Liver transplant, colchicine–antifibrotic and anti-inflammatory, ursodiol–a non-toxic bile acid that may slow disease progression. See Ursodiol. Cf Cirrhosis.

pri·mar·y bil·i·ar·y cir·rho·sis

(prīmār-ē bilē-ar-ē sir-ōsis)
Disease in middle-aged women, characterized by cholestasis with hyperlipemia, pruritus, and hyperpigmentation of the skin; serum antimitochondrial antibodies are present in 85-90% of patients.

Hanot,

Victor C., French physician, 1844-1896.
Hanot cirrhosis - a condition characterized by obstructive jaundice with hyperlipemia, pruritus, and hyperpigmentation of the skin. Synonym(s): primary biliary cirrhosis
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