Symptoms Of Primary Biliary Cholangitis

Primary biliary cholangitis is a chronic autoimmune disease that causes bile ducts in the liver to die slowly over time. When bile ducts are damaged, bile can back up in the liver and not excrete properly. This occurs because the immune system is unable to fight the white blood cells called T-lymphocytes (T-cells) that collect in the liver to allow it to function properly. The cells slowly die causing fibrosis, otherwise known as scar tissue, and ultimately cirrhosis of the liver. This impacts the function of the liver in the body. The cause of primary biliary cholangitis is unknown, but it is not the result of substance abuse. There is no cure for primary biliary cholangitis, so it is imperative to be aware of the symptoms so it can be prevented or treated as early as possible.

Fatigue

Fatigue is the most common symptom of primary biliary cholangitis. Fatigue can be understood as extreme tiredness caused by mental effort, physical effort, or an illness. Most patients notice this symptom first, and throughout all stages of the disease. This symptom is not changed by sleep or rest. Some patients are unable to fall or stay asleep, making it hard to accomplish daily tasks both mentally and physically. Primary biliary cholangitis and extreme tiredness can cause significant mental impairments like trouble concentrating and depression. It can also cause decreased function in motor skills. There are no treatments for this symptom.

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Itchy Skin

Itchy skin is another common symptom of primary biliary cholangitis. Itchy skin is a symptom that occurs most often in the early stages of primary biliary cholangitis. This is caused when bile spills over into the bloodstream and is not excreted properly through the liver. The liver normally acts as a filter for bile. When bile hits the bloodstream, it can cause an extreme itching sensation. This symptom can be debilitating because it can be uncontrollable. This symptom can cause rashes, sensitivity, and irritation.

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Musculoskeletal Pain

Musculoskeletal pain is a symptom of primary biliary cholangitis that occurs in the later stages of the disease. Musculoskeletal pain is pain experienced in the joints, bones, or muscles. This is caused by vitamin deficiencies, which can lead to osteoporosis. The lack of bile in the liver affects the body's ability to absorb fats and fat-soluble vitamins important for bone health. Vitamin D specifically aids in bone, joint, and muscle health, and helps maintain bone density throughout the body. The liver is in charge of making vital nutrients, so when the liver isn't working properly, the body is deficient. In the late stages of primary biliary cholangitis, bone loss and bone fractures can occur, which can be debilitating and extremely painful. This symptom can be severe and should receive medical attention immediately.

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Jaundice

Jaundice, the yellowing of the skin and eyes, can be a symptom of primary biliary cholangitis in the end-stage of the disease and is a critical condition. This symptom is a way that the body communicates an organ isn't functioning properly. Jaundice is caused when the body breaks down excess dead red blood cells in the liver, which creates bilirubin, the yellow pigment. This occurs because the liver is not excreting the bile and is backed up in the liver. This symptom is a serious symptom and affected individuals should receive medical attention immediately.

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Hyperpigmentation

Hyperpigmentation is a symptom that occurs in primary biliary cholangitis. Hyperpigmentation is the darkening of the epidermis and dermis (skin) in the form of spots. This often caused by sun exposure and is normally harmless. However, in primary biliary cholangitis, this symptom is the darkening of the skin without exposure to the sun.

Further, discoloration in the skin in primary biliary cholangitis can be widespread, covering parts of the body. Skin can also change in texture and have rashes. The darkening of the skin is caused by the increased amount of melanin in the body when the liver isn't functioning correctly.

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Dry Mouth And Eyes

Primary biliary cholangitis may manifest in the form of dry mouth and eyes. The dryness in an affected individual's mouth is the result of inflammation that occurs in the glands responsible for producing their saliva, while the dryness in the eyes is the result of inflammation in the glands responsible for producing tears. Because no blood vessels are present in the cornea, tear production is the only mechanism that keeps this part of the eye moisturized. Decreased tear production causes the eyes to become excessively dry and irritated as a result. Saliva production is the way the body keeps the mucous membranes in the oral cavity moisturized. Reduced saliva production results in excessive drying out of the mouth. This gland inflammation is caused by the patient's immune system inappropriately attacking such glandular tissues. Primary biliary cholangitis is characterized by inflammation and degradation of the biliary ducts, which is also the result of an inappropriate attack by the affected individual's immune system. Because both issues are the result of the same underlying mechanism action on epithelial elements, they often occur alongside each other.

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Swollen Spleen

Individuals with primary biliary cholangitis may present with a swollen spleen as a symptom of their disease. Portal hypertension, high blood pressure in the vein that blood uses to move from the intestines, gallbladder, pancreas, stomach, and spleen to the liver, can contribute to the spleen enlargement. Portal hypertension is the result of liver scarring caused by a build-up of bile in the liver. The spleen is an organ responsible for recycling old red blood cells, storing white blood cells and platelets, and fighting off certain pathogens. Portal hypertension causes the enlargement of the proximal spleen in a patient's body due to reduced blood flow to the portal blood vessels from the spleen. This reduced blood flow results in the accumulation of white blood cells and platelets in the spleen being overproduced. The overproduction of such blood components is caused by the liver's inability to perform its function of filtering toxins out of the blood. A healthy spleen and immune system can help with filtering toxins from the body to a certain extent, but not enough to stop them from accumulating in the absence of healthy liver function.

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Ascites

Ascites in an individual can indicate they are being affected by primary biliary cholangitis. Ascites is a medical term used to describe the accumulation of ascitic or protein-containing fluid in an individual's abdomen. Ascites occurs as a result of portal hypertension, which is high blood pressure in the vein that moves blood from the intestines, gallbladder, pancreas, stomach, and spleen to the liver. Cirrhosis of the liver is the mechanism that precipitates portal hypertension in primary biliary cholangitis patients. Fluid leaks from the intestine surface, liver surface, and out of the blood vessels as a homeostatic response to mediate high blood pressure. Levels of albumin in the blood fall as it leaks out of the blood vessels, which causes further leakage of fluid into abdominal tissues. The result of this fluid build-up is the visible enlargement of the abdomen and feelings of fullness, shortness of breath, and loss of appetite. These symptoms are common with ascites because the excess fluid causes the organs in the abdominal cavity to become compressed.

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Fatty Deposits Around The Body

Fatty deposits around the body can be a symptom indicative of primary biliary cholangitis. As the bile ducts become damaged due to inflammation, the secretions of bile into the intestines decrease. Because lipids or fats are secreted in bile as bile acids, individuals with less bile secretion all together tend to have high levels of serum cholesterol. Additionally, lipoprotein X or LpX is found in individuals affected by primary biliary cholangitis. Lipoprotein X is an abnormal lipoprotein that causes blood cholesterol levels to stay elevated due to its inability to be synthesized properly. The high cholesterol levels in the blood can result in it depositing in the form of fatty yellow-colored lumps around the body. These deposits commonly occur on the lower and upper eyelids, around both eyes, and near the inner corner of the eyes. The deposits themselves are not particularly harmful but can cause discomfort in affected individuals. Fatty deposits from primary biliary cholangitis can be surgically removed for aesthetic and symptomatic purposes but will grow back if the cholesterol levels remain elevated in the patient's blood.

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Hypothyroidism

Individuals affected by primary biliary cholangitis can present with hypothyroidism. Thyroid hormones are critical for healthy organ development, growth, and function. The liver is responsible for metabolizing such hormones and regulating their endocrine effects systemically. When the liver is unable to perform its function of activating and deactivating thyroid hormones, the affected individual will present with hypothyroidism. An impairment of this hormone metabolism process over time results in the thyroid gland going into an adaptive hypothyroid state. Some primary biliary cholangitis patients have concurrent immune system abnormalities that can result in the immune system mistakenly attacking the cells of the thyroid gland. This immune attack on the gland causes the tissues that produce essential hormones to decay. When the thyroid gland is unable to produce an adequate amount of hormones for the body, the individual has hypothyroidism. Both of these mechanisms can occur independently of one another, or they may both simultaneously affect the thyroid function of an individual with primary biliary cholangitis.