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Primary biliary cirrhosis/Primary biliary cholangitis | Medical Science Optional Notes for UPSC PDF Download
Introduction
It primarily affects middle-aged women and Is frequently associated with other autoimmune conditions
Histopathological stages
- Stage I: Involves the infiltration of lymphocytes in portal areas and the formation of periductal granulomas.
- Stage II: Characterized by bile duct ductopenia and the advancement of progressive fibrosis.
- Stage III: In this phase, bridging fibrosis occurs, indicating a more severe progression of liver damage.
- Stage IV: Represents the final stage, marked by the development of liver cirrhosis.
Question for Primary biliary cirrhosis/Primary biliary cholangitisTry yourself: What is stage II of the disease?View Solution
Primary biliary cirrhosis
Clinical features
- 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 and xanthelasma
Question for Primary biliary cirrhosis/Primary biliary cholangitisTry yourself: Which of the following clinical features is typically seen in primary biliary cirrhosis?View Solution
Diagnosis
- Cholestasis Parameters: Alkaline phosphatase (ALP), gamma-glutamyl transferase (γ-GT), and conjugated bilirubin levels are indicative of cholestasis.
- Transaminases (AST/ALT): These enzymes are either within normal limits or show slight elevation.
- Autoantibodies:
- Highly prevalent antimitochondrial antibodies (AMA) are detected in over 95% of cases.
- Antinuclear antibodies (ANA) are present in up to 70% of cases.
- Elevated immunoglobulin M (IgM) levels are observed.
- Liver Biopsy: Utilized for confirming the diagnosis and staging of the disease.
Question for Primary biliary cirrhosis/Primary biliary cholangitisTry yourself: What parameters are indicative of cholestasis?View Solution
Primary biliary cirrhosis-Management
There is no cure for PBC. Treatment consists of slowing disease progression and alleviating symptoms. Liver transplantation is the only definitive treatment.
First-line medical therapy
Ursodeoxycholic Acid (UDCA):
- Slows down the progression of the disease.
- Improves clinical symptoms.
- Delays the necessity for transplantation.
- Also used for treating cholestatic pruritus.
Liver transplantation necessary if liver cirrhosis is advanced.
Question for Primary biliary cirrhosis/Primary biliary cholangitisTry yourself: Which is the first-line medical therapy for primary biliary cirrhosis?View Solution
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FAQs on Primary biliary cirrhosis/Primary biliary cholangitis - Medical Science Optional Notes for UPSC
| 1. What is primary biliary cirrhosis? |  |
Ans. Primary biliary cirrhosis, also known as primary biliary cholangitis (PBC), is a chronic liver disease characterized by the progressive destruction of small bile ducts in the liver. This leads to the accumulation of bile in the liver, causing inflammation, scarring, and ultimately liver cirrhosis.
| 2. What are the symptoms of primary biliary cirrhosis? | |
Ans. The symptoms of primary biliary cirrhosis may vary from person to person but commonly include fatigue, itching (pruritus), dry eyes and mouth, jaundice (yellowing of the skin and eyes), abdominal pain or discomfort, and swollen legs or ankles. Some individuals may also experience complications such as osteoporosis, vitamin deficiencies, or liver failure.
| 3. How is primary biliary cirrhosis diagnosed? | |
Ans. Primary biliary cirrhosis is usually diagnosed through a combination of medical history, physical examination, blood tests, and imaging studies. Blood tests may be used to detect specific antibodies associated with PBC, such as antimitochondrial antibodies (AMA). Liver biopsy may also be performed to assess the severity of liver damage and confirm the diagnosis.
| 4. What is the management approach for primary biliary cirrhosis? | |
Ans. The management of primary biliary cirrhosis aims to slow down the progression of the disease, alleviate symptoms, and prevent complications. This is typically achieved through the use of medications such as ursodeoxycholic acid (UDCA) to improve liver function, relieve itching, and delay the need for liver transplantation. Regular monitoring of liver function, vitamin supplementation, and lifestyle modifications such as avoiding alcohol and maintaining a healthy diet are also important components of management.
| 5. Can primary biliary cirrhosis be cured? | |
Ans. Currently, there is no known cure for primary biliary cirrhosis. However, early diagnosis and appropriate management can significantly slow down the disease progression and improve the quality of life for individuals with PBC. In severe cases, liver transplantation may be considered as a treatment option. It is important for individuals with PBC to work closely with their healthcare providers to effectively manage the condition and minimize complications.
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Dec 22, 2024 Last updated |
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