PRIMARY BILIARY CIRRHOSIS(PBC)

Primary biliary cholangitis (PBC) is a liver ailment that impacts the intricate network of bile ducts coursing through your liver. Over time, it gradually erodes these ducts, impeding the smooth flow of bile. This build-up of bile within the liver leads to tissue damage, wherein healthy tissue is replaced by scar tissue, ultimately resulting in cirrhosis. Formerly known as primary biliary cirrhosis, PBC earns its name from the root causes of inflammation in the bile ducts. Unlike other conditions where inflammation might be triggered by infections or obstructions, PBC initiates inflammation directly.

The chronic inflammation characteristic of PBC triggers a cascade of tissue degradation. While short-lived inflammation is part of the body’s natural healing process, persistent inflammation prompts excessive scarring. Scarred bile ducts become narrowed and distorted, hindering the passage of bile. Consequently, bile backs up into the liver, inciting inflammation and, eventually, cirrhosis.

Given its chronic and progressive nature, PBC necessitates ongoing management. The disease evolves slowly, often with subtle symptoms initially. However, left unchecked, it can culminate in liver failure, a life-threatening condition that mandates a liver transplant for survival. Thankfully, medication exists to retard disease progression, offering hope to patients in managing its course.

Primary biliary cholangitis Vs. Primary sclerosing cholangitis (PSC)

Distinguishing between primary biliary cholangitis and primary sclerosing cholangitis (PSC) reveals subtle yet critical differences. While both conditions affect bile ducts, PBC primarily targets intrahepatic ducts within the liver, whereas PSC extends its impact to all bile ducts, including extrahepatic ones. Furthermore, treatment options diverge between the two, with PBC offering medications to mitigate progression, while effective treatments for PSC remain elusive.”

Causes of Primary biliary cholangitis

In primary biliary cholangitis (PBC), the root cause lies within the body’s immune system, which erroneously targets and attacks the cells lining the intrahepatic bile ducts, inducing inflammation. This autoimmune response is akin to mistaking these cells as foreign intruders, leading to chronic inflammation and eventual scarring.

The precise triggers for autoimmune diseases like PBC remain elusive. However, genetic predisposition plays a significant role, as individuals with autoimmune diseases often have a familial history of such conditions, sometimes experiencing multiple types. Additionally, environmental factors are believed to contribute, suggesting that external factors, such as certain chemicals or infections, may act as triggers for the disease onset.

Prevalence

Primary biliary cholangitis predominantly impacts women and individuals assigned female at birth (AFAB) at a striking ratio of 10-to-1 compared to those assigned male at birth (AMAB). In the United States, approximately 60 AFAB individuals per 100,000 are affected, compared to 15 AMAB individuals per 100,000. Diagnosis typically occurs after the age of 40. The prevalence is notably higher in regions such as Scotland, Scandinavia, and Northeast England. Moreover, individuals with a personal or familial history of autoimmune diseases are at increased risk, hinting at a potential genetic component to the disorder.

Symptoms of Primary biliary cholangitis

Primary biliary cholangitis (PBC) often lurks silently in its early stages, with many individuals exhibiting no symptoms. However, as the disease progresses, manifestations of biliary dysfunction gradually surface. The earliest and most prevalent symptoms reported by individuals with PBC include:

Fatigue (65%)
Itchy skin (pruritus) (55%)

These symptoms vary in intensity from person to person and can manifest at different stages of the disease, regardless of its advancement. Nonetheless, some studies suggest that more severe symptoms early on may indicate a swifter disease progression. Some of the symptoms include:

Dry eyes and mouth (sicca syndrome)
Abdominal pain and discomfort
Jaundice (yellowing of the skin and eyes)
Easy bruising and bleeding
Swelling in the legs and ankles (edema)
Weight loss
Darkening of the skin (hyperpigmentation)

Complications of PBC

As biliary disease advances, it paves the way for various complications:

Fat Malabsorption: Insufficient bile in the digestive system impedes the breakdown and absorption of fats, resulting in:
- High blood cholesterol
- Subcutaneous fat deposits
- Fatty stools or diarrhea
- Weight loss
- Reduced levels of fat-soluble vitamins A, D, E, and K
- Osteoporosis due to impaired absorption of fat-soluble vitamin D
Portal Hypertension: Scar tissue formation within the liver obstructs blood vessels, leading to portal hypertension—elevated blood pressure in these veins and their tributaries. This can lead to:
- Enlarged liver and spleen
- Increased susceptibility to infections due to reduced immunity
- Easy bruising and bleeding (thrombocytopenia)
- Spider angiomas—red, spider-like blood vessels beneath the skin
- Varices in the esophagus and abdomen
- Gastrointestinal bleeding
- Ascites—a buildup of fluid in the abdomen
- Edema—swelling in the lower extremities
- Occasional mental confusion resulting from toxin accumulation in the bloodstream

These complications underscore the progressive nature of PBC and highlight the importance of timely diagnosis and management to mitigate their impact.”

Risk Factors

Female gender
Middle-aged
Family history of autoimmune diseases
Presence of other autoimmune diseases, such as rheumatoid arthritis or Hashimoto’s thyroiditis

Diagnosing Primary biliary cholangitis

Diagnosing primary biliary cholangitis (PBC) typically involves a series of steps to gather information and confirm the presence of the disease. Here’s how it’s done:

Medical History and Physical Examination: Your healthcare provider will begin by discussing your medical history and symptoms. They’ll then conduct a physical examination to assess any visible signs of liver or bile duct abnormalities.
Blood Tests: A sample of your blood will be analyzed to detect specific antibodies associated with PBC, notably the antimitochondrial antibody (AMA). Additionally, the liver function tests (LFTs) testing levels of liver enzymes, particularly alkaline phosphatase, will be measured to assess liver function and stress.
Imaging Studies: To further evaluate the liver and biliary system, imaging studies are performed. This typically starts with an abdominal ultrasound, which provides initial insights. In some cases, more detailed images may be necessary, requiring procedures such as magnetic resonance imaging (MRI).
Liver Biopsy (if necessary): Approximately 5% of individuals with PBC may test negative for AMA but exhibit other signs and symptoms. In such cases, a liver biopsy may be recommended to confirm the diagnosis. This involves inserting a needle into the liver to extract a tiny tissue sample for microscopic examination.

Treatments for Primary biliary cholangitis

Medication: While there’s no cure for PBC, medication can effectively slow its progression and alleviate symptoms. Ursodeoxycholic acid (UDCA) is commonly prescribed, as it helps clear bile from the liver and reduce liver damage. For those who don’t respond to UDCA, alternative medications like obeticholic acid may be considered. Additionally, individual symptoms such as itching can be managed with antihistamines, ultraviolet light therapy, or bile acid sequestrants. Vitamin supplements (fat-soluble vitamins A, D, E, and K) may also be prescribed to prevent deficiencies and associated complications like osteoporosis. Stimulants like modafinil may help alleviate fatigue in some individuals.
Surgery: In cases where medication fails to improve liver function and the condition continues to deteriorate, liver transplant surgery may be recommended. Liver transplants have shown excellent outcomes for individuals with PBC. However, it’s important to note that PBC can recur after transplantation, albeit at a slower pace. Nonetheless, life expectancy following a successful liver transplant returns to normal levels.

Home Care Tips

Maintain a healthy diet low in sodium and saturated fats
Avoid alcohol and certain medications that can worsen liver damage
Practice good hygiene to prevent infections
Manage symptoms with proper skin care and hydration

Prevention

Since the exact cause of PBC is unknown, there are no specific prevention methods. However, maintaining a healthy lifestyle, avoiding known liver toxins, and promptly treating other autoimmune conditions may help reduce the risk of developing PBC.

When to See a Doctor

If you experience persistent symptoms like fatigue, itching, or abdominal pain, it’s essential to consult a healthcare professional. Early diagnosis and treatment can significantly improve outcomes and slow the progression of the disease.

Outlook/Prognosis

Living with primary biliary cholangitis (PBC) entails understanding the condition’s progression and adopting strategies to manage symptoms and maintain overall well-being. Here’s what you can expect and how to take care of yourself:

Slow Progression: PBC typically advances slowly, allowing for effective symptom management and disease control with early diagnosis and treatment. While fatigue remains challenging to address, medications can help alleviate symptoms, enabling many individuals to lead relatively normal lives.
Prognosis: The outlook varies depending on factors such as disease severity and response to treatment. While some may experience a more aggressive form of PBC, others may live for years without significant interference in their daily lives. Those who progress to advanced stages may require liver transplants to survive, with favorable outcomes for transplant recipients.
Life Expectancy: On average, it takes 15 to 20 years for PBC to reach the terminal stage. The initial asymptomatic stage can persist for a considerable duration, with symptoms typically manifesting within five to ten years for about half of individuals. Once symptoms develop, the average life expectancy is approximately 10 years. However, successful liver transplants yield a 65% survival rate at the ten-year mark post-transplantation.

Sources

Mayo Clinic. Primary Biliary Cirrhosis.
American Liver Foundation. Primary Biliary Cholangitis (PBC).
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Primary Biliary Cholangitis.
European Association for the Study of the Liver (EASL). Clinical Practice Guidelines for the Diagnosis and Management of Patients with Primary Biliary Cholangitis. Journal of Hepatology, Volume 67, Issue 1, Pages 145-172, July 2017.
MedlinePlus. Primary Biliary Cirrhosis.