Primary Biliary Cholangitis
What Is PBC?
PBC is a global autoimmune liver disease primarily afflicting women of all races and ethnicities.
Primary Biliary Cholangitis (PBC), formerly known as Primary Biliary Cirrhosis, is a chronic liver disease that slowly destroys the intrahepatic bile ducts within the liver. Over a period of years, liver inflammation may cause scarring, which leads to cirrhosis. PBC is not alcohol or drug related, and it is not contagious.
With early diagnosis and proper medications, most people with PBC will never reach the cirrhosis stage.
The cause of PBC is still unknown. But we do know:
- PBC is not alcohol- or drug-induced.
- Women are affected ten times more than men.
- PBC is usually diagnosed in patients between the ages of 35 to 60 years.
Current studies suggest that PBC may involve autoimmunity, infection, or genetic predisposition, and the disease does seem to appear more often in certain families.
People with PBC usually look extremely healthy. The slight bronze pigmentation of the skin is often present in the later stages of the disease, making the individual look tanned. But outward appearances don’t tell the story of what is going on inside their bodies. Even at the transplant list stage, many with PBC look healthy. A person with PBC commonly hears comments such as, “You look so healthy” or ”You don’t look sick.”
Symptoms
Most people with PBC remain symptom-free for many years, and some may never notice any symptoms.
Initial symptoms vary among people with PBC, which can sometimes make it difficult for doctors to actually diagnose PBC. The variation in symptoms may be due to individual differences, autoimmune response, or other diseases associated with PBC.
These are the first symptoms noticed by most people with PBC, often prior to diagnosis. Symptoms can be present in any combination, and may include:
- Dry eyes that can feel like sand in your eyes.
- Dry mouth, sometimes referred to as cottonmouth.
- Fatigue, which may be the first symptom that causes the individual to visit the doctor. The fatigue associated with PBC appears to be totally different from any other sort of fatigue. In early stages, many people with PBC have commented they could sleep for hours, while some in the later stages say sleeping becomes more difficult. At this time, there is little research into the cause and treatment of liver disease fatigue. It is usually not due to depression, and some researchers believe it is an abnormality of the axis between the pituitary and the adrenal glands. Support and understanding from family members, friends and the doctor is very important, making this symptom somewhat bearable.
- Itching of the skin that can be intense and unrelenting. In some, it is debilitating. Many people with PBC say the itching feels like tiny bugs crawling under the skin. In some cases, the application of heat has helped, while others heat aggravates it.
People with PBC may start noticing other symptoms:
- Abdominal pain or pressure in the liver area. (Yes, it’s real.)
- Arthritic aches and pains in bones, muscles and joints. In some, the pains can be severe and debilitating. Some even report severe pain just touching their leg, feet and hip bones, but this is not common.
- Dental problems.
- Diarrhea that can be greasy (steatorrhea).
- Difficulty sleeping and changes in sleeping habits. Some people with PBC have noted that itching intensifies when they lie down to sleep, and those who have liver pain say it is more severe in a sleeping position.
- Elevated cholesterol
- Gradual darkening (hyperpigmentation) or changes in skin texture, and various skin rashes.
- Reflux and stomach ulcers.
- Small yellow or white bumps (xanthomas) under the skin, or around the eyes.
- Thyroid problems.
Over the years, as the PBC progresses, other symptoms may appear. These symptoms can include any of these:
- Enlarged abdomen from fluid accumulation.
- Easy bruising or bleeding.
- Fever
- Hepatic encephalopathy that causes personality changes – dulling of mental functions, neglect of personal appearance, forgetfulness and trouble concentrating, changes in sleeping habits, confusion, breath odor and muscle stiffness. Encephalopathy occurs in the final stage of PBC.
- Hepatorenal syndrome, progressive deterioration of kidney function leading to kidney failure in a person with liver failure.
- Hepatopulmonary syndrome, associated with difficulty with breathing.
- Hypersplenism, enlarged spleen
- Increased bilirubin.
- Internal bleeding in the upper stomach and esophagus, that may be caused by varices.
- Jaundice (yellowing of the skin and eyes).
- Liver cancer (hepatocellular carcinoma).
- Nausea & vomiting.
- Osteoporosis or other metabolic bone disease.
- Sexual problems (impotence in men, absence of periods in women, dryness, lack of desire.)
- Swelling of the hands, legs and ankles. See edema and ascites.
- Trembling hands.& legs.
- Weight increase or decrease
Diagnosis
There are four disease stages of PBC.
The number of patients being diagnosed at the asymptomatic stage has risen dramatically over the past few years due to widespread laboratory screening. Typically, the blood lab pattern reveals an elevated alkaline phosphatase level with a normal bilirubin. Bilirubin does not increase until the final disease stage.
PBC Disease Stages
Although four typical stages of evolution have been defined, PBC initially is focal, with considerable overlap between stages in any one case.
Normal-sized triads; portal inflammation, subtle bile duct damage. Granulomas – nodules filled with a variety of inflammatory cells – are often detected in this stage.
Enlarged triads; periportal fibrosis and/or inflammation. Typically characterized by the finding of a proliferation of small bile ducts. Progressive scarring continues with less bile duct proliferation and less inflammation.
Active and/or passive fibrous septae. Fibrous bands link the portal tracts, and zone 1 cholestasis and Mallory hyaline can become evident.
Nodules present; garland or jigsaw pattern. The end product is a firm, regular, intensely bile-stained cirrhosis, difficult to distinguish from other cirrhotic processes in the absence of granulomas and the pathognomonic bile duct lesions
The course of PBC varies greatly. It may not diminish the quality or the duration of life. Of patients who present without symptoms, 50% show evidence of liver disease over the ensuing 15 years. Slow progression suggests prolonged survival. A rising serum bilirubin, associated with autoimmune disorders, and advanced histologic changes – especially a high level of ALP – indicate a poor prognosis.
Related Conditions
PBC is considered an Autoimmune Disease
Some people who are diagnosed with PBC may also be diagnosed with one or more other autoimmune diseases.Other Autoimmune Diseases
A number of autoimmune diseases are often associated with PBC.Most often
Occasionally
Rarely
- Arthritis – an inflammation of joints due to infectious, metabolic, or constitutional causes.
- Osteoporosis – a condition characterized by a decrease in bone mass with decreased density and enlargement of bone spaces, producing porosity and brittleness.
- Dental Problems – Liver disorders can show up during dental care, affecting both the dentist and patient. Potential effects include a tendency to bleed more than expected, intolerance to drugs such as general anesthetics and benzodiazepines, gum disease, bone loss, and even underlying infective causes for the liver dysfunction.
- Sicca Complex – Secondary Sjögren’s syndrome can occur, along with other autoimmune disorders like systemic lupus erythematosus, rheumatoid arthritis, and primary biliary cholangitis as a symptom of the primary disease. When the disorder is limited to the eyes and mouth, with no other organ or tissue involvement evident, it is called Sicca Complex. (Don’t confuse the disease Sjogrens Syndrome with the PBC symptoms, “Dry Eyes & Dry Mouth”.)
- Autoimmune Hepatitis – is a chronic disease in which the body’s own immune system attacks the liver and causes it to become inflamed.
- Celiac Disease – a chronic hereditary intestinal disorder in which an inability to absorb the gliadin portion of gluten results in the gliadin triggering an immune response that damages the intestinal mucosa—called also celiac sprue, gluten-sensitive enteropathy, nontropical sprue, sprue.
- Colitis – an inflammation of the colon.
- Costochondritis – a condition of unknown origin that is characterized by inflammation of costochondral cartilage.
- Crohns Disease – chronic ileitis that typically involves the distal portion of the ileum, often spreads to the colon, and is characterized by diarrhea, cramping, and loss of appetite and weight with local abscesses and scarring.
- Diabetes – a disease characterized by an inability to process sugars in the diet, due to a decrease in or total absence of insulin production.
- Fibromyalgia – a neurosensory disorder characterized by widespread muscle pain, joint stiffness, and fatigue. The condition is chronic (ongoing), but pain comes and goes and moves about the body.
- Gallstones – a solid crystal deposit that forms in the gallbladder, which is a pear-shaped organ that stores bile salts until they are needed to help digest fatty foods. Gallstones can migrate to other parts of the digestive tract and cause severe pain with life-threatening complications.
- Hyperthyroidism – is the overproduction of thyroid hormones by an overactive thyroid.
- Hypothyroidism – or underactive thyroid, develops when the thyroid gland fails to produce or secrete as much thyroxine (T4) as the body needs. Because T4 regulates such essential functions as heart rate, digestion, physical growth, and mental development, an insufficient supply of this hormone can slow life-sustaining processes, damage organs and tissues in every part of the body, and lead to life-threatening complications.
- Pulmonary Disorders – pertaining to the lungs or pulmonary artery. Includes respiratory problems such as asthma, cystic fibrosis, chronic obstructive pulmonary disease (COPD), lung cancer, occupational respiratory diseases, mycobacterium avium complex (MAC) lung disease, pulmonary fibrosis and sleep disorders.
- Raynaud’s Phenomenon – intermittent attacks of ischemia of the extremities of the body, especially the fingers, toes, ears, and nose, caused by exposure to cold or by emotional stimuli. The attacks are characterized by severe blanching of the extremities, followed by cyanosis, then redness; they are usually accompanied by numbness, tingling, burning, and often pain.
- Renal Tubular Acidosis – (RTA) is a condition characterized by too much acid in the body due to a defect in kidney function.
- Scleroderma – a progressive disease that affects the skin and connective tissue (including cartilage, bone, fat, and the tissue that supports the nerves and blood vessels throughout the body). There are two major forms of the disorder. The type known as localized scleroderma mainly affects the skin. Systemic scleroderma, which is also called systemic sclerosis, affects the smaller blood vessels and internal organs of the body.
- Sjogrens Syndrome – a disorder in which the mouth and eyes become extremely dry. Sjögren’s syndrome is often associated with other autoimmune disorders. There are three types of Sjögren’s syndrome. Primary Sjögren’s syndrome occurs by itself, with no other associated disorders. Secondary Sjögren’s syndrome occurs along with other autoimmune disorders, like systemic lupus erythematosus, rheumatoid arthritis, and primary biliary cholangitis. When the disorder is limited to involvement of the eyes, with no other organ or tissue involvement evident, it is called sicca complex. (Don’t confuse the disease Sjogrens Syndrome with the PBC symptoms “Dry Eyes & Dry Mouth”.)
- Multiple Sclerosis – a chronic autoimmune disorder affecting movement, sensation, and bodily functions. It is caused by destruction of the myelin insulation covering nerve fibers (neurons) in the central nervous system (brain and spinal cord).
- Lupus – a chronic inflammatory autoimmune disorder that may affect many organ systems including the skin, joints, and internal organs.
As with any other chronic illness, support and understanding is very important in helping the PBC patient cope with day to day living.
