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.
Ultrasound imaging of the liver is recommended for patients whose blood tests
show cholestasis. Cholestatic blood tests feature a disproportionately elevated
alkaline phosphatase and ggt, compared to the ALT and AST. The purpose of the
ultrasound exam is to visualize the bile ducts to exclude mechanical blockage
(obstruction) of larger bile ducts as the cause of the cholestasis. Gallstones
or tumors, for example, may cause mechanical obstruction of bile ducts. The
blockage can cause increased pressure in the bile ducts that leads to dilation
(widening) of the upstream bile ducts.
Dilated bile ducts caused by mechanical obstruction can usually be visualized
on the ultrasonogram. The dilated bile ducts can also be seen using other
imaging techniques such as computerized tomographic (CT) scanning, Magnetic
Resonance Imaging (MRI), or an endoscopic procedure called ERCP. On the other
hand, in PBC, the ducts that are being destroyed are so small that any dilation
of upstream ducts cannot be seen with any of the imaging techniques. For the
diagnosis of PBC in patients with cholestatic liver tests, a positive AMA and a
normal ultrasound examination usually is sufficient. In this situation, other
imaging studies of the bile ducts are usually not required.
The benefits of doing a liver biopsy (taking a tissue sample) include:
Confirmation of the diagnosis
Determination of the stage of disease
Identification of any other concurrent liver disease
Pathologists (physicians who analyze tissue samples) have divided the
evolution of PBC into four stages recognizable by the microscopic appearance of
the liver biopsy.
Progressive inflammation of the portal tracts and
their small bile ducts
The inflammation causes destruction of the small bile
ducts and spreads to also involve the nearby liver cells (hepatocytes)
Extensive scars (fibrosis) protrude from the inflamed
portal tracts into the region of liver cells
Cirrhosis
From a practical perspective, physicians most often divide the disease into
prefibrotic (before scarring) and fibrotic (scarring or cirrhosis) stages, still
usually using the biopsy findings.
Patients often ask if a liver biopsy is mandatory. The answer usually depends
on the level of confidence in establishing the diagnosis of PBC using the liver
tests, autoantibodies, and ultrasound. In the presence of cholestatic liver
tests, high levels of AMA, and an ultrasound showing no bile duct obstruction in
a middle-aged woman, the diagnosis of PBC can be made rather confidently without
a biopsy. Treatment then can often be started, for example, with ursodeoxycholic
acid (UDCA, a naturally occurring bile acid that is produced in small quantities
by normal liver cells).
Without a biopsy, however, the stage (extent) of the disease would remain
undefined. A biopsy helps the patient know where they are in the natural history
of the disease. Furthermore, knowing the stage of PBC can help physicians decide
about prescribing certain medications (for example corticosteroids) that may be
effective in the early stages and less valuable in later stages.
On the other hand, PBC patients who already have the complications of
cirrhosis (for example, ascites, varices, or hepatic encephalopathy) are
presumed to have advanced liver disease. In these PBC patients the imaging
studies alone are usually sufficient to exclude dilated ducts and a biopsy is
not needed for staging the disease. Otherwise, the presence or absence of other
symptoms (apart from the presence of those clearly due to the complications of
cirrhosis) is not an accurate guide to the stage of PBC on a liver biopsy. For
example, in one large series of patients, approximately 40% of those without
symptoms had cirrhosis on liver biopsy.
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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