Primary Biliary Cirrhosis: Symptoms, Stages, Life Expectancy
What is Primary Biliary Cirrhosis?
Primary biliary cirrhosis is a progressive condition that involves the liver. The involvement of the bile ducts in the liver explains the disease. The fluid produced by our liver is destroyed as the bile ducts are slowly damaged. Bile is an important product of our body for digestion of fats. This condition is frequently found in women, accounting 75% of patients with primary biliary cirrhosis, and to the population aging 40-50 years. There are cases where people as young as 22 years old have been reported to have this disease. This condition commonly affects the northern Europeans and is uncommon to those with African descent.
Symptoms may be observed right after the patient is diagnosed. Sometimes from routine blood chemistry, diagnosis can be accomplished without knowing its existence. But this condition’s manifestations can be grouped into two stages:
In the early stage of PBC, some people would remain asymptomatic for years, but others would experience mild symptoms earlier. Fatigue is one of the most common symptoms associated with primary biliary cirrhosis. The relationship between fatigue and severity of illness has not been found. No known evidence would say that the more severe the disease is the more exhausted they are. Doctors would say that there is equal fatigability among patients, in any way that it’s mild or severe. Another symptom is itching. This is usually experienced over the legs, arms and back. This can become worse inflicting your sleeping pattern. The root for this has not been pointed out by doctors/researchers in their study. In the early stage, the patient may experience dry eyes and mouth. This is referred as the sicca syndrome. Sicca syndrome usually occurs to those with underlying autoimmune disorders.
In later stages, there is progressive destruction of the bile duct and liver cells causing severe presentations. The patient will experience jaundice. This indicates that the condition has advanced turning your skin yellow. This can be explained by the high blood levels of bilirubin in the blood. Since the function of bile is out of our body’s hands, its task to excrete bilirubin from our body is not attained. Since the bilirubin has piled up in the system, the jaundice becomes prominent and visible as time goes and when the disease is not treated effectively. Another change in the patient’ color of the skin emerges too. This is a symptom called hyperpigmentation. Since there is inadequate blood flow, the production of melanin increases. Resulting for our skin to become darker and sometimes skin color uniformity is not attained. Edema and ascites would indicate fluid retention. This can start from the swelling of the feet and ankles but in time will affect the abdomen. Elimination of salts has become impossible as the disease progresses. The result would be cholesterol deposits accumulation. This can prompt to fatty deposits in the skin around the eyes, eyelids, or in the palms, soles, elbows or knees. Other presenting symptoms in the stage of PBC, digestive problems may occur. Since bile is a vital component of our body for digestion, the absorption of fats can be a problem. This would result to diarrhea and fatty stools (steatorrhea).
Once this condition is not properly treated and monitored. Some complications may arise such as bleeding, damage to the brain, kidney failure and malnutrition. Osteoporosis can also be a form of complication. It is a must that you contact your doctor once clear manifestations are observed.
The etiology of this condition is idiopathic. According to research, the role of an immune system disorder may have paved the way for primary biliary cirrhosis development. Genetics and environmental factors have also been linked to this disease.
This condition started from a simple inflammation of the tiniest ducts of the liver. As the inflammation worsens it can spread to nearby cells, thereby damaging them too. This would leave out a scar tissue. The condition is worsened in time and can lead to cirrhosis. This disease can result to permanent scarring of liver tissue.
Triggering factors such as the genetic composition of a person can be a cause for this disease. A person who has a known medical history of primary biliary cirrhosis will make one susceptible to the disease. An acquired infection has been suspected as a cause for this condition. This can be a result of any bacterial, fungal or parasitic infection. Women who are constantly acquiring urinary tract infections are prone to PBC.
Underlying conditions can predispose you to acquire this disorder such as hepatitis, celiac disease, hypothyroidism, Raynaud’s phenomenon, and Sicca syndrome.
There are a total of four stages for Primary biliary cirrhosis:
Stage I (Portal Stage) –> There is portal inflammation with bile duct damage. Inflammatory cells are found in this stage.
Stage II (Periportal Stage) –> Fibrosis and inflammation has occurred. There is proliferation of small bile ducts.
Stage III (Septal Stage) –> There is active and passive fibrous septae (from root word septum).
Stage IV (Biliary cirrhosis) –> Nodules are already present
In order to treat the condition of the client, the treatment plan’s goal should be to slow the progression of the disease. One of the medications given to patients with PBC is ursodeoxycholic acid. This medication is known to give delay in the need for transplantation or even death. Reports have been given that the effects of this drug to patients are very desirable. Giving patients immunosuppressant is also included in the therapy. This can also slow down the progression of the disease. Corticosteroids and methotrexate are alternative treatments that give great results such as relieving the symptoms of the client. But a side effect of corticosteroid should be evaluated regularly, which is osteoporosis. Colchicine is an anti-inflammatory drug that can help reduce the manifestations of the patient. Antihistamines are given to patient who experience itching as a symptom. This is a first-line treatment for the manifestation.
Medical prescription followed at home is a vital assistance in relieving the patients’ discomfort. In order to relieve the patient’s itching of skin, colestyramine should be taken orally as ordered by the physician. As this itchiness can cause dryness of the skin, it is highly advised to apply moisturizer regularly. To relieve dry eyes and dry mouth we can use artificial tears and saliva, lubricating gels and oestrogen creams. The use of lozenges can help reduce the dryness of the mouth.
There are cases that a liver transplant is necessary. If other treatments are no longer effective to the patient, he/she is needs to undergo the surgical intervention. This has been a good intervention to persons with PBC but is also possible that you might get it with the new liver.
It is included in the home remedy that the person affected should follow a strict diet and a modification of lifestyle. To start, we need to avoid the things that can induce or worsen the condition, primary biliary cirrhosis. Having a reduced-sodium diet is recommended for sodium can increase your risk for tissue swelling and progression of ascites (fluid accumulation in the abdominal cavity). It is highly suggested that one should do regular exercise along with calcium and vitamin D supplements. This can help in avoiding the patient to acquire osteoporosis. Avoid food groups that can trigger allergic reactions, so not to worsen the presenting symptoms of the disease.
Primary biliary cirrhosis is diagnosed through the following tests:
Liver function tests - the doctor orders this to determine level of serum alkaline phosphatase – a liver enzyme.
Serum albumin – a test done to identify the levels of albumin/protein in the blood. High levels of albumin in the blood can be harmful to the body.
Prothrombin time (PT) – testing blood’s clotting time is essential in identifying risk for bleeding and to indicate of there is bleeding
Serum cholesterol and lipoproteins – this test will identify the levels of cholesterol and lipoproteins in the blood, but high levels would not mean grave harm to the patient. This test would be assistive in the differential diagnosis of the condition.
Anti-mitochondrial antibody blood test – the presence of AMA is found in 90-95% of patients with primary biliary cirrhosis.
Liver biopsy – this test is a very good confirmatory exam. The extent of the damage can be identified through this test.
Abdominal ultrasound – can whether the liver and bile ducts are inflamed.
As the patient is treated accordingly and checked time to time, then we can easily identify the prognosis of the client. Indicators such as the serum bilirubin level can be a guide for the prognosis of this condition. Once the bilirubin level is 2-6 mg/dL, the mean survival time of the patient is 4.1 years. Patients with 6-10 mg/dL can present 2.1 years of life. In worst cases of 10 mg/dL would mean a survival time of 1.4 years.
When patient underwent liver transplant, the possibility of recurrence is not left out. Eighteen percent of cases after 5 years have been noted as well as a 30% recurrence rate at 10 years.
Complications may arise and make the prognosis bad. These patients have increased risk for developing hepatocellular carcinoma.
Life expectancy truly signifies the patient’s wellness and perseverance to survive. It has been noted that patients who had the disease for 10 years will possibly experience liver failure thus resulting to an unwanted outcome. Upon diagnosis of the client prompt treatment can facilitate to a probability of 5 years of life. This was according to Kaplan-Meier analysis.