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Explain Primary Biliary Cirrhosis?

• Primary Biliary Cirrhosis is abbreviated as PBC.
• It is an autoimmune disorder of the liver.
• It is recognized by the liver destruction that is progressive in stage.
• The small bile ducts are destroyed.
• The liver’s intralobular ducts are affected early in the disease.
• When these channels are damaged, the liver bile becomes more in amount due to accumulation and damage is caused of the tissue over the time.
• This can lead to scarring, fibrosis and cirrhosis.
• It was previously believed to be an uncommon disease.
• More recent studies have indicated that it may affect up to 1 in 3–4,000 people; the gender ratio is at least 9:1.

Signs and Symptoms of Primary Biliary Cirrhosis

Individuals with PBC may present with the following:
• Fatigue
• Pruritus (itchy skin)
• Jaundice (yellowing of the eyes and skin)
• Increased bilirubin in the blood.
• Xanthoma
• Pain in the upper right portion of the abdomen
• Dry eyes can develop
• Dry mouth
• Darkening of the skin that’s not related to sun exposure
• Swollen ankles and feet
• Accumulation of fluid in the abdominal cavity (ascites)
• Diarrhea that can be greasy

Fatty deposits on the skin around your:
– Eyes
– Eyelids
– In the creases in palms, soles, elbows or knees

Complications of cirrhosis and portal hypertension:
– Fluid retention in the abdominal cavity (ascites)
– Hypersplenism
– Esophageal varices
– Hepatic encephalopathy
– Extra hepatic autoimmune disorder such as rheumatoid arthritis or Sjögren’s syndrome

Diagnosis of Primary Biliary Cirrhosis

• Intermediate magnification micrograph of PBC showing bile duct inflammation and periodontal granulomas.
• Liver biopsy
• Immuno fluorescence staining pattern of sp100 antibodies and AMA.
• Diagnose PBC for autoimmune hepatitis or primary sclerosing cholangitis (PSC).

Diagnostic blood tests include:
• Deranged liver function tests
• Presence of certain antibodies: antimitochondrial antibody (AMA), antinuclear antibody (ANA)
• Abdominal ultrasound or a CT scan to rule out blockage to the bile ducts.
• Endoscopic retrograde cholangiopancreatography
• Anti-nuclear antibodies are seen as the prognostic agents in PBC.
• Anti-glycoprotein-210 antibodies are seen.
• To a lesser degree anti-p62 antibodies are seen.
• These are linked to marking the final stage of failure of liver.
• Anti-centromere antibodies correlate with developing portal hypertension.
• Anti-np62 and anti-sp100 are also found in association with PBC.

Summary of Stages
Stage 1 — Portal Stage
• Normal sized triads
• Portal inflammation
• Subtle bile duct damage
• Granulomas

Stage 2 — Periportal Stage
• Enlarged triads
• Periportal fibrosis
• Inflammation
• Proliferation of small bile ducts.

Stage 3 — Septal Stage
• Active or passive fibrous septa.

Stage 4 — Biliary Cirrhosis
Nodules present

Treatment for Primary Biliary Cirrhosis

Ursodeoxycholic acid (Ursodiol)
• It is the most frequently used treatment.
• This helps reduce the cholestasis and improves blood test results.
• It has little effect on the symptoms.
• It improves the symptoms or not is controversial.

Cholestyramine (a bile acid sequestrant)
• When bile acids move within the body an itching is developed which is relieved with the use of Cholestyramine.
• This also helps in absorbing bile acids in the gut and then to be removed.
• This is for the acids to avoid entering into the blood stream.
• Alternative agents include naltrexone and rifampicin.

Provigil (modafinil)
To relieve fatigue associated with primary biliary cirrhosis.

Others
• Patients who suffer from PBC have reduced lipid-dependent absorption of Vitamins A, D, E, and K.
• Appropriate supplementation is recommended when bilirubin is elevated.
• Patients with PBC are at a high risk of osteoporosis.
• They may possible develop even esophageal varices.
• As in all liver diseases, excessive consumption of alcohol is contraindicated.

The Official Patient’s Sourcebook on Primary Biliary Cirrhosis Primary Biliary Cirrhosis Primary Biliary Cirrhosis, An Issue of Clinics in Liver Disease

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