Symptoms
Many people with PBC have no symptoms, especially in the early stages. When symptoms do occur, they can include:
Fatigue (most common)
Itching (pruritus)
Dry eyes and mouth
Jaundice (yellowing of the skin and eyes)
Darkening of the skin
Pain in the upper right abdomen
Swelling in the legs and feet (edema)
Ascites (fluid buildup in the abdomen)
Xanthomas (fatty deposits under the skin, usually around the eyes)
Osteoporosis (thinning of the bones)
Causes
The exact cause of PBC is unknown, but it is considered an autoimmune disease. This means the body's immune system mistakenly attacks the bile ducts in the liver. Genetic factors and environmental triggers may play a role in its development. PBC is not caused by alcohol consumption, medications, or viral infections, although these factors can worsen the condition.
Medicine Used
Ursodeoxycholic acid (UDCA): This medication helps improve bile flow and can slow the progression of liver damage. It's often the first-line treatment.
Obeticholic acid (OCA): Used in combination with UDCA for patients who don't respond adequately to UDCA alone.
Fibrates (e.g., bezafibrate): May be used off-label to help with itching or in combination with UDCA.
Cholestyramine: Used to help manage itching by binding bile acids in the intestine.
Calcium and Vitamin D supplements: To help prevent or treat osteoporosis.
Liver transplantation: In advanced cases of liver failure.
Budesonide: May be used in specific cases and in clinical trials
Seladelpar: This medication is a PPAR-delta agonist and has been approved to treat PBC.
Precautions
While you cannot prevent PBC, you can take precautions to manage symptoms and protect your liver:
Regular medical follow-up: See your doctor regularly to monitor your condition and adjust treatment as needed.
Avoid alcohol: Alcohol can further damage the liver.
Maintain a healthy weight: Obesity can worsen liver disease.
Quit smoking: Smoking can worsen liver damage.
Eat a healthy diet: Focus on fruits, vegetables, and whole grains.
Get vaccinated: Get vaccinated against hepatitis A and B, and get a flu shot every year.
Manage other health conditions: Control diabetes, high blood pressure, and high cholesterol.
Protect your bones: Take calcium and vitamin D supplements as recommended by your doctor. Consider bone density testing.
Treat itching: Use lotions, avoid hot showers, and take medications as prescribed to relieve itching.
Dry eyes and mouth: Use artificial tears and saliva substitutes.
Sun Protection: PBC patients can have increased skin pigment and sunburns, use sunblock
How long does an outbreak last?
PBC is not characterized by "outbreaks" in the traditional sense. It is a chronic, progressive disease that lasts for many years. The disease can progress at varying rates, and some individuals may remain relatively stable for a long time, while others may experience a more rapid decline in liver function. Symptoms can fluctuate in intensity over time.
How is it diagnosed?
PBC is typically diagnosed based on the following:
Blood tests: Elevated levels of alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) are common. Antimitochondrial antibodies (AMA) are present in most patients with PBC.
Liver biopsy: This can help confirm the diagnosis and assess the severity of liver damage.
Imaging tests: Ultrasound, CT scan, or MRI may be used to rule out other liver conditions.
Ruling out other conditions: Doctors need to exclude other possible causes of liver disease.
Timeline of Symptoms
The timeline of symptoms in PBC can vary greatly from person to person:
Early stages: Many people are asymptomatic. The disease may be discovered incidentally during routine blood work.
Middle stages: Symptoms such as fatigue, itching, and dry eyes/mouth may develop. Blood tests will show elevated liver enzymes.
Late stages: Jaundice, edema, ascites, and other complications of cirrhosis may occur. Liver function tests will be significantly abnormal. The rate of progression through these stages varies considerably
Important Considerations
Early diagnosis and treatment are crucial: Starting treatment with UDCA or other medications early in the course of the disease can help slow its progression and prevent complications.
PBC is a lifelong condition: There is currently no cure for PBC, but treatment can help manage symptoms and improve quality of life.
Individualized treatment: The best treatment plan for PBC will depend on the individual's symptoms, disease stage, and overall health.
Support groups: Joining a support group can provide valuable emotional support and information.
Clinical Trials: Participation in clinical trials may offer access to new treatments and contribute to research efforts.
Complications Management: Managing complications like osteoporosis, portal hypertension, and ascites are important aspects of care.