John M. Vierling M.D. is Professor of Medicine and Surgery at the Baylor College of Medicine in Houston, Texas, where he also serves as Director of Baylor Liver Health and Chief of Hepatology. In addition, he is the Director of Advanced Liver Therapies, a center devoted to clinical research in hepatobiliary diseases at St. Luke's Episcopal Hospital. Dr. Vierling is board certified in internal medicine and gastroenterology and a Fellow of the American College of Physicians.
Dr. Schoenfield served as associate professor of medicine and consultant in gastroenterology on the faculty of the Mayo Clinic for seven years. He became a professor of medicine in residence at UCLA from 1972 to 1999 (now emeritus). He was the director of gastroenterology at Cedars-Sinai Medical Center in Los Angeles for 25 years, where he received the chief resident's teaching award, the president's award, and the pioneer of medicine award.
The most common symptom of PBC is fatigue, which occurs in up to 70% of
patients. The presence and severity of fatigue, however, does not correspond
(correlate) with the severity of the liver disease. It should be noted that
significant fatigue can be either the cause or the result of difficulty sleeping
or depression.
Fatigue associated with inflammation of the liver is often characterized by
normal energy during the initial half to two thirds of the day followed by a
profound loss of energy that requires rest or a substantial reduction in
activity. Thus, when patients report being exhausted in the morning, it is
likely that sleep deprivation and depression are the cause of the exhaustion
rather than PBC. Most people with PBC report that a nap does not rejuvenate
them. Conversely, many PBC patients inexplicably experience occasional days
without a loss of energy.
In summary, the main characteristics of fatigue due to liver inflammation in
PBC are:
Just about as common as fatigue in PBC, itching (pruritus) of the skin
affects 66% of patients at some time during the disease. The itching tends to
occur early in the course of the disease, when patients still have good liver
function. As a matter of fact, itching can even be the initial symptom of PBC.
It is interesting to note that some women with PBC experienced itching during
the last trimester (three months) of a prior pregnancy, before they knew about
their PBC. In a condition called cholestasis of pregnancy, some otherwise normal
women during the last trimester develop cholestasis and itching that resolve
following delivery. (Remember that cholestasis means decreased bile flow.) Of
course, most women with cholestasis of pregnancy do not go on to develop PBC.
Yet, it turns out that about 5% of women diagnosed with PBC give a history of
having had such itching during a prior pregnancy.
Characteristically, the itching in PBC begins in the palms of the hands and
soles of the feet. Later, it may affect the entire body. The intensity
fluctuates in a circadian rhythm, meaning that the itching can worsen at night
and improve during the day. Nocturnal itching can disrupt sleep and lead to
sleep deprivation, fatigue, and depression. Rarely, the itching is so severe and
unresponsive to therapy that the person may become suicidal. Prolonged itching
and scratching causes scratch marks (excoriations), thickening, and darkening of
the skin.
The cause (etiology and pathogenesis) of itching remains unclear. The bile
acids, as previously mentioned, normally are transported in bile from the liver,
through the bile ducts, to the intestine. Most of the bile acids are then
reabsorbed in the intestine and go back to the liver for reprocessing and
recycling. In cholestasis, therefore, the bile acids back up from the liver,
accumulate in the blood, and, for some years, were presumed to be the cause of
the itching. Modern studies, however, have just about refuted the notion that
the itching in PBC and other cholestatic liver diseases is caused by bile acids.
Recently, the itching was considered (postulated) to be due to accumulation
of an endorphin, a natural substance that attaches (binds) to the natural
receptors (acceptors) for morphine in nerves. You see, nerves in the skin carry
the sensation of itching. Indeed, the finding that itching improved in some
people treated with drugs that block the binding of morphine or endorphins to
nerves supported this consideration. Yet, many patients do not respond to these
blocking drugs, suggesting that other causes or mechanisms are involved in
producing itching.
Gallstones are stones that form when substances in the bile harden. Gallstones (formed in the gallbladder) can be as small as a grain of sand or as large as a golf ball. There can be just one large stone, hundreds of tiny stones, or any combination. The majority of gallstones do not cause symptoms.
Hypothyroidism is any state in which thyroid hormone production is below normal. Normally, the
rate of thyroid hormone production is controlled by the brain at the pituitary.
Hypothyroidism is a very common condition and the symptoms of hypothyroidism are
often subtle.
Liver cancer is the fifth most common cancer in the world and the majority of patients with liver cancer will die within one year as a result. Patients with associated cirrhosis caused by chronic hepatitis B or C infections, alcohol, and hemochromatosis are at the greatest risk of developing liver cancer. Many patients with liver cancer do not develop symptoms until the advanced stages of the tumor which usually makes prognosis poor. The combination of an imaging study (ultrasound, CT, or MRI scans) and an elevated blood level of alpha-fetoprotein will most effectively diagnose liver cancer, while a liver biopsy can make a definitive diagnosis. Medical treatments, including chemotherapy, chemoembolization, ablation, and proton beam therapy, are not very effective. Surgical removal of the tumor or a liver transplant may be most effective in certain cases.
Cirrhosis of the liver refers to a disease in which normal liver cells are replaced by scar tissue caused by alcohol and viral hepatitis B and C. This disease leads to abnormalities in the liver's ability to handle toxins and blood flow, causing internal bleeding, kidney failure, mental confusion, coma, body fluid accumulation, and frequent infections. Symptoms include yellowing of the skin, itching, and fatigue.
Sjögren's syndrome is an autoimmune disease involving the abnormal production of extra antibodies that attack the glands and connective tissue. Sjögren's syndrome with gland inflammation (resulting dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome that is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Though there is no cure for Sjögren's syndrome, the symptoms may be treated by using lubricating eye ointments, drinking plenty of water, humidifying the air, and using glycerin swabs. Medications are also available to treat dry eye and dry mouth.
Edema is the swelling of tissues as a result of excess water accumulations. Peripheral edema occurs in the feet and legs. There are two types of edema, non-pitting edema and pitting edema. Causes of pitting edema is caused by systemic diseases, most commonly involving the heart, liver, and kidneys. Local conditions that cause edema are thrombophlebitis and varicose veins. Edema is generally treated with medication.
Itching can be a common problem. Itches can be localized or generalized. There are many causes of itching to include: infection (jock itch, vaginal itch), disease (hyperthyroidism, liver or kidney), reactions to drugs, and skin infestations (pubic or body lice). Treatment for itching varies depending on the cause of the itch.
Jaundice is a yellowish staining of the skin and whites of the eyes (sclerae) with bilirubin, the pigment found in bile. Jaundice can be an indicator of liver or gallbladder disease, or it may result from the rupture of red blood cells (hemolysis).
Ulcerative colitis is a chronic inflammation of the colon. Symptoms include abdominal pain, diarrhea, and rectal bleeding. Ulcerative colitis is closely related to Crohn's disease, and together they are referred to as inflammatory bowel disease. Treatment depends upon the type of ulcerative colitis diagnosed.
Learn about osteoporosis, a condition characterized by the loss of bone density, which leads to an increased risk of bone fracture. Unless one experiences a fracture, a person may have osteoporosis for decades without knowing it. Treatment for osteoporosis may involve medications that stop bone loss and increase bone strength and bone formation, as well as quitting smoking, regular exercise, cutting back on alcohol intake, and eating a calcium- and vitamin D-rich balanced diet.
Scleroderma is an autoimmune disease of the connective tissue. It is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body, leading to thickness and firmness of involved areas. Scleroderma is also referred to as systemic sclerosis, and the cause is unknown. Treatment of scleroderma is directed toward the individual features that are most troubling to the patient.
The liver is the largest solid organ in the body, and is actually an gland. The liver has a wide variety of critical functions such as manufacturing proteins and metabolizing fats and carbohydrates. The liver also eliminates harmful biochemical waste products from the body (alcohol, drugs, toxins). The liver secretes bile that aids in digestion. Examples of diseases of the liver include cirrhosis, hepatitis, cancer, and fatty liver. Symptoms of liver disease include bleeding, easy bruising, edema, fatigue, and jaundice.
Digestion is the complex process of turning food you eat into the energy you need to survive. The digestive process also involves creating waste to be eliminated, and is made of a series of muscles that coordinate the movement of food.
Thyroiditis is the inflammation of the thyroid gland. The inflamed thyroid gland can release an excess of thyroid hormones into the blood stream, resulting in a temporary hyperthyroid state. Some forms of thyroiditis can be diagnosed based on tenderness and enlargement of the thyroid gland. A thyroid scan sometimes is used in making the diagnosis. Thyroiditis can also be diagnosed with a biopsy of the thyroid gland.
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