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 key blood test abnormality in PBC and all liver diseases associated with
cholestasis is an elevated alkaline phosphatase enzyme level in the blood. The
finding of a concurrent elevation of the gamma glutamyl transpeptidase (ggt)
blood level proves that the elevated alkaline phosphatase is from the liver,
rather than from bone (another source of alkaline phosphatase). Other liver
enzymes, such as aspartate aminotransferase (AST) and alanine aminotransferase
(ALT), may be either normal or only slightly elevated at the time of diagnosis.
As the duration of disease increases, both of these liver enzymes (the
aminotransferases) usually become elevated to a mild to moderate degree, while
the alkaline phosphatase can become very high. For more information about liver
blood tests, please read the Liver Blood Tests article.
Other blood tests may also be helpful in the diagnosis of PBC. For example,
serum immunoglobulin M (IgM) is frequently elevated. Also, just about all
patients with cholestasis develop increased cholesterol levels (as noted
previously), and some also develop elevated triglycerides. Moreover, testing the
levels of these fats (lipids) can identify patients who might form cholesterol
deposits in the skin or nerves. (See the section on xanthomas above.)
AMA are detectable in the serum in 95 to 98% of patients with PBC, as noted
earlier. The most economical test for AMA applies diluted samples of a patient's
serum onto tissue sections from rat stomach or kidney in the laboratory.
(Remember that the mitochondria are present in all cells, not just the cells of
the liver and bile ducts.) Serum antibodies that attach (bind) to mitochondrial
membranes within the tissue cells can then be observed with a microscope. The
most dilute sample of serum showing this binding reaction is reported, using the
term titer. The titer indicates the most dilute serum sample that reacts with
the tissue mitochondria. A higher titer means there is a greater amount of AMA
in the serum.
The AMA titers in PBC are almost universally greater than or equal to 1 to
40. This means that a serum sample diluted with 40 times its original volume
still contains enough antimitochondrial antibodies to be detected in the binding
reaction. A positive AMA with a titer of at least 1:40 in an adult with an
elevated alkaline phosphatase is highly specific for a diagnosis of PBC. The
antigen recognized by AMA in patients with PBC is now known to be PDC-E2 and is
also often referred to as the M2 antigen, as discussed earlier. So, newly
developed tests for antibodies that bind to PDC-E2 are more specific and are now
available to confirm the diagnosis of PBC.
It is noteworthy that approximately 20% of patients with AMA also have
antinuclear (ANA) and/or anti-smooth muscle (SMA) autoantibodies in their blood.
The ANA and SMA are more characteristically found in a disease called chronic
autoimmune hepatitis. It turns out that patients who have persistently
undetectable AMA but otherwise have clinical, laboratory, and liver biopsy
evidence of PBC, all have either ANA or SMA. These patients have been referred
to as having AMA-negative PBC, autoimmune cholangiopathy, or autoimmune
cholangitis. The natural history, associated diseases, laboratory test
abnormalities, and liver pathology are indistinguishable between the
AMA-positive and AMA-negative patients. Thus, it seems inappropriate, for now at
least, to classify this AMA-negative disease as different from PBC. Accordingly,
this situation should be referred to as AMA-negative PBC. Rarely, some other
patients appear to concurrently have features of both PBC and chronic autoimmune
hepatitis. Such patients are said to have an overlap syndrome.
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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