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.
Up to 70% of patients with PBC experience a sensation of dry eyes or dry
mouth referred to as sicca syndrome or alternatively, as Sjogren's syndrome.
This syndrome is caused by an autoimmune inflammation of the lining cells of the
ducts that carry tears or saliva. Rarely, patients experience the consequences
of dryness in other areas of the body including the windpipe or larynx (causing
hoarseness) and the vagina. This autoimmune inflammation and drying of
secretions can also occur, although even more rarely, in the ducts of the
pancreas. The resulting poor pancreatic function (pancreatic insufficiency) can
cause impaired absorption of fat and the fat-soluble vitamins.
Raynaud's phenomenon starts with an intense blanching (paling) of the skin
of the fingers or toes when they are exposed to the cold. When the hands or feet
are re-warmed, the blanching characteristically changes to a purplish
discoloration and then to a bright red, often associated with throbbing pain.
This phenomenon is due to the cold causing a constriction (narrowing) of the
arteries that supply blood to the fingers or toes. Then, with re-warming of the
hands or feet, the blood flow is restored and causes the redness and pain.
Raynaud's phenomenon is often associated with scleroderma. For more
information about this phenomenon, please read the
Raynaud's phenomenon. article
Approximately 17% of patients with PBC develop mild scleroderma, a condition
in which the skin around the fingers, toes, and mouth becomes tight. In
addition, scleroderma involves the muscles of the esophagus and small intestine.
The esophagus connects the mouth to the stomach, and its muscles help to propel
swallowed food into the stomach. In addition, a band of muscle (the lower
esophageal sphincter), which is located at the junction of the esophagus and
stomach, has two other functions. One is to open to let food pass into the
stomach. The other is to close to prevent the stomach juices that contain acid
from flowing back into the esophagus.
Scleroderma, therefore, can also cause esophageal and intestinal symptoms.
Thus, involvement of the esophageal muscles that propel food through the
esophagus results in difficulty swallowing. Most often, patients experience this
difficulty as a sensation of solid food sticking in the chest after swallowing.
Involvement of the lower esophageal sphincter muscle prevents the closure of the
lower end of the esophagus and thereby, allows reflux of stomach acid, causing
the symptom of heartburn. The heartburn, which is not caused by a heart problem,
is usually experienced as a sensation of burning in the center of the chest.
Involvement of the muscles of the small intestine in scleroderma can cause a
condition called bacterial overgrowth, which can lead to malabsorption of fat
and diarrhea. For more about this condition, please read the Scleroderma article.
Finally, a minority of PBC patients has a variant of scleroderma referred to
as CREST syndrome. The term CREST refers to Calcium deposits in the skin,
Raynaud's phenomenon, muscle dysfunction of the Esophagus, tightening of the
skin of the fingers called Sclerodactyly, and dilated small blood vessels
beneath the skin called Telangiectasias.
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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