primary biliary cirrhosis

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.

---

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. Pathologic 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.