Primary Biliary Cirrhosis (cont.)
In this Article
Sicca syndrome
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
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
Scleroderma
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.
Next: Rheumatoid arthritis »
- colchicine, Colchicine - Read about colchicine, a drug prescribed for the pain of gouty arthritis. Side effects, drug interactions, dosage, and preagnancy information is also included in the information.
- Liver Blood Tests - Learn about liver blood tests used to detect liver damage disease such as fatty liver, cirrhosis, hepatitis, Tylenol liver damage, and more. This includes measuring the aminotransferases enzymes (AST and ALT levels)
- Liver Cancer - Get information on liver cancer, its symptoms, causes, diagnosis, staging, prognosis, treatment, and prevention.
Latest Medical News
Digestion RSS