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.
Primary biliary cirrhosis (PBC) is a chronic (long duration) disease
characterized by progressive inflammation and destruction of the small bile
ducts within the liver. What are the bile ducts and what do they do? Lined with
cells named biliary epithelial cells, the bile ducts are tubules that make up a
plumbing system for the liver. The bile ducts along with the gallbladder are
part of what is called the biliary tract.
The plumbing system begins in the liver with very small caliber ducts that
connect to increasingly larger caliber ducts, like a tree in which twigs connect
to small branches that connect to larger branches. In fact, this system is often
referred to as the biliary tree. The large right and left bile ducts, still
within the liver, connect to an even larger common bile duct that runs outside
the liver to the small intestine just beyond the stomach. The common bile duct
connects by the cystic duct to the gallbladder. The gallbladder is a
pear-shaped, expandable, sac-like organ in the biliary system. The branching
bile ducts course through special tissue in the liver, called portal tracts,
which act like conduits for the ducts. In fact, the branching portal tracts
containing the bile ducts also contain the blood vessels that enter and leave
the liver.
The bile ducts carry bile, a fluid that is produced by the liver cells
(hepatocytes) and modified by the biliary lining (epithelial) cells as it flows
through the ducts to the small intestine. Bile contains substances required for
digestion and absorption of fat called bile acids, as well as other compounds
that are waste products, such as the pigment bilirubin. (Bilirubin is a
yellow-orange compound produced by the breakdown of hemoglobin from old red
blood cells.) Bile is stored in the gallbladder between meals and discharged
into the small intestine during digestion of the meals.
The inflammation in PBC starts in the liver's portal tracts and involves
the small bile ducts in these areas. The destruction of the small bile ducts
blocks the normal flow of bile into the gut. The medical term for decreased flow
of bile is cholestasis. (Chole means bile and stasis means failure to flow.)
Cholestasis is a very important aspect of this disease. As the inflammation
continues to destroy more of these bile ducts, it spreads to destroy nearby
liver cells (hepatocytes). As the inflammatory destruction of the hepatocytes
proceeds, scar tissue (fibrosis) forms and spreads through the areas of
destruction.
The combined effects of progressive inflammation, scarring, and toxicity of
bile trapped within hepatocytes (liver cells) culminates in cirrhosis. Cirrhosis
is defined as the stage of disease when there is both widespread scarring of the
liver and clusters (nodules) of hepatocytes reproducing (regenerating)
themselves within the scars. Since cirrhosis occurs only in the later stage of
PBC, the name primary biliary cirrhosis is actually a misnomer for patients in
the earlier stages of the illness. The more technically correct and ponderous
term for PBC, chronic non-suppurative destructive cholangitis, however, has
never been widely used and is unlikely to replace PBC.
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.
Gallstones are "stones" that form in the gallbladder or bile ducts.
The common types of gallstones are cholesterol, black pigment, and brown pigment.
Cholesterol gallstones occur more frequently in several ethnic groups and are associated with female
gender, obesity, pregnancy,
oral hormonal therapy, rapid loss of weight,
elevated blood triglyceride levels, and Crohn's disease.
Black pigment gallstones occur when there is increased destruction of red blood cells, and brown pigment gallstones
occur when there is slow flow and infection of bile.
The majority of gallstones do not cause symptoms.
The most common symptoms of gallstones are biliary colic and cholecystitis.
Gallstones do not cause intolerance to fatty foods,
belching,
abdominal distention, or
gas.
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