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Lupus

Definition


Disease: Lupus Lupus
Category: Dermatological diseases
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Disease Definition:

When the body's immune system attacks its own tissues and organs, lupus occurs, which is a chronic inflammatory disease. The skin, joints, blood cells, kidneys, lung, heart and other body systems could be affected by the inflammation caused by lupus.

The four types of lupus are:

 

  • Systemic lupus erythemaosus
  • Discoid lupus erythematosus
  • Drug-induced lupus erythematosus
  • Neonatal lupus


The most common and serious form of lupus is systemic lupus erythematosus. Lupus occurs more frequently in women, the reason of which is still not known.
Thanks to the improvement of diagnosis and treatment of lupus, its outlook isn't as grim as it used to be. Most people with lupus could lead active lives if they receive treatment.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

The signs and symptoms of lupus could come on suddenly, develop slowly, be mild or severe.
The signs and symptoms will be based on which body systems are affected by this disease. Some of the common signs and symptoms of lupus may be:

 

  • Weight loss or gain
  • Mouth sores
  • Fatigue
  • Malar rash, which is a butterfly-shaped rash on the face that covers the cheeks and bridge of the nose.
  • Shortness of breath
  • Fever
  • Dry eyes
  • Joint pain, stiffness and swelling
  • Chest pain
  • Memory loss
  • Anxiety
  • Alopecia, which is hair loss
  • Depression
  • Easy bruising
  • Fingers and toes that turn white or blue when they are exposed to cold or during stressful periods, a condition called Raynaud's phenomenon.
  • Skin rashes that appear or worsen with sun exposure.


Most people with lupus have the mild form of the disease characterized by episodes (flares), which is when signs and symptoms get worse for a while, then improve or even disappear completely for a period of time.

A person should see a doctor in case they develop an unexplained rash, ongoing fever, fatigue or persistent aching.

In case someone has been diagnosed with lupus, the doctor should monitor the patient’s condition and treatment. In case new symptoms arise, a doctor should be consulted.

Causes:

Lupus is an autoimmune disease, which means that the person’s own immune system will turn against healthy tissue. Inflammation and damage to various parts of the body will be caused by this including the brain, heart, kidneys, blood vessels, lungs, skin and joints.

What exactly causes an autoimmune disease is still not known. It is thought that a person may inherit a predisposition to lupus, but not lupus itself. Instead, doctors believe that lupus could be the result of a combination of genetics and environment. People with an inherited predisposition only develop lupus when they come in contact with a virus or a medication that could trigger the disease.

TYPES OF LUPUS:
Each of the four types of lupus has a different treatment and prognosis.

Systemic lupus eryhemaosus:
Usually, when people talk about lupus, they will be talking about this type. This form of lupus usually affects the joints, skin, blood, kidneys and lungs, but it could affect almost any part of the body.

Discoid lupus erythematosus:
A small number of people with this type may also develop systemic lupus erythematosus, but it's not possible to know who will develop it. This type affects only the skin, in the form of a circular rash on the face, scalp and neck. This type of lupus is also called cutaneous lupus.

Drug-induced lupus eryhematosus:
A wide variety of body systems could be affected by this type of lupus. This form usually occurs when a person takes certain prescription medications, but not everyone who takes these medications will develop lupus. Once the person stops taking the medications that caused the lupus, the signs and symptoms usually go away.

Neonatal lupus:
This is a rare form of lupus that only affects newborn babies. A baby with this type of lupus may experience a rash in the weeks following birth. Before disappearing, neonatal lupus could last several months. However, a problem with the electrical system of the heart (congenital heart block) could be caused by more-serious cases of lupus. This type of lupus develops when a mother with certain antibodies that are linked to autoimmune diseases passes them to her developing fetus. Even if the mother doesn't have any signs or symptoms of an autoimmune disease, this could occur.

The exact cause of lupus is still not clear, however, doctors have found factors that could increase people’s risk of developing lupus, such as:

Sex:
Women are more likely to develop lupus.

Age:
Lupus is most commonly diagnosed in people between the ages of 15 and 40, but it could also occur in people of all ages, including older adults, infants and children.

Race:
Blacks, Hispanics and Asians are more likely to develop lupus.

Sunlight:
Being exposed to the sun may cause the development of lupus skin lesions or an internal response may be triggered in susceptible people. Although it is believed that sunlight could cause skin cells to express certain proteins on their surface, but it is still not understood exactly why does ultraviolet radiation have this effect. Then, antibodies, which are normally present in the body, latch onto these proteins and thus initiate an inflammatory response. In people with lupus, damaged skin cells seem to die more frequent, which ends up causing even more inflammation.

Infection with Epstein-Barr virus:
Epstein-Barr virus is such a common virus that almost everyone has been infected with it. Some nonspecific signs and symptoms are caused by this virus, such as sore throat and fever. After the initial infection subsides, the virus will stay dormant in the cells of the immune system until something reactivates it. Recurrent Epstein-Barr infections seem to increase the risk of developing lupus, the cause of which is still not known.

Exposure to chemicals:
Some studies have shown that cigarette smokers, in addition to people who work in jobs involving exposure to mercury and silica might have an increased risk of developing lupus. However, proving that certain chemicals could cause or increase the risk of a disease is quite difficult.

Certain prescriptions medication:
Procainamide, isoniazid, hydralazine,are examples of drugs that could be linked to the symptoms of lupus . It could take months or years of therapy with these drugs before symptoms appear.
 

Complications

Complications:

Various areas of the body could be affected by the inflammation caused by lupus, such as:

Kidneys:
Serious kidney damage could be caused by lupus; and among people with lupus, kidney failure is one of the leading causes of death. Usually, during the first two years after diagnosis, about three-quarters of people with lupus develop kidney damage. However, medications could effectively treat lupus-related kidney damage. To monitor kidney function, a blood test called serum creatinine level will be done, in addition to urinalysis. Weight gain, generalized itching, leg swelling (edema), nausea and vomiting are some of the signs and symptoms of kidney problems.

Blood and blood vessels:
Lupus could cause inflammation of the blood vessels also called vasculitis, which is a serious complication that is responsible for about 7% of lupus-related deaths. Additionally, anemia and an increased risk of bleeding or blood clotting are some of the other blood problems caused by lupus.

Lungs:
Someone’s chance of developing an inflammation of the chest cavity lining (pleurisy) will be increased if they have lupus. Pleurisy could make breathing painful. Having lupus also makes people more susceptible to developing a noninfectious form of pneumonia. Usually, lupus-related lung complications don't cause any signs or symptoms, are mild, and could be detected by pulmonary tests.

Avascular necrosis:
This condition causes tiny breaks to occur in the bone and eventually lead to the collapse of the bone when the blood supply to a bone diminishes. The most commonly affected joint is the hip joint, but any other bone could also be affected. Usually, one in ten people with lupus develops symptoms of avascular necrosis. Either lupus itself or high doses of corticosteroids that are used to treat lupus could cause avascular necrosis.

Heart:
Pericarditis, which is inflammation of the heart membrane; myocarditis and endocarditis, inflammation of the heart muscle; and coronary vasculitis, inflammation of the coronary arteries could be caused by lupus. Additionally, someone’s risk of cardiovascular disease and heart attacks will be increased greatly if they have lupus. Almost 40% of people who have lupus also develop prematurely hardened arteries (atherosclerosis), compared with the 15% of their peers who don't have lupus. To help reduce the risk of heart disease, people should control their high blood pressure, high blood cholesterol, get regular exercise and stop smoking.

Central nervous system (CNS):
A person may experience dizziness, seizures, headaches, behavior changes and hallucinations in case their central nervous system is affected by lupus. Memory problems, confusion and difficulty expressing thoughts are some of the cognitive problems that almost 80% of people with lupus experience.

Infection:
Because lupus and its treatment, corticosteroids and cytotoxic drugs, affect the immune system, people with lupus will be vulnerable to infection. Infection could bring on a lupus flare, which increases the risk of infection even more, creating a vicious cycle. Common respiratory infections, such as colds; shingles; urinary tract infections; herpes; yeast infections and salmonella are some of the infections that commonly affect people with lupus. About one-third of lupus-related deaths are caused by more serious infections, such as pneumonia.

Cancer:
The risk of cancer, particularly lung cancer and non-Hodgkin's lymphoma that affects the lymphatic system, will be increased if someone has lupus. The risk of cancer could also be increased by the immunosuppressant drugs that are used to treat lupus. Even though people with lupus have a slightly increased risk of death from non-Hodgkin's lymphoma or lung cancer, the general population are more likely to die of cancer than people with lupus.

Pregnancy complications:
Some women with lupus may experience a flare during pregnancy. Women with lupus have an increased risk of miscarriage. The risk of high blood pressure during pregnancy (preeclampsia) and preterm birth is also increased by lupus. About one in four women with lupus give birth to healthy babies prematurely; about two in ten women with lupus develop preeclampsia; and about one in four pregnant women with lupus experience a miscarriage. A woman will be advised to wait in order to get pregnant until her disease has been in remission or under control for at least a year, to reduce her risk of the complications mentioned above.
 

Treatments:

Depending on the signs and symptoms of lupus, treatment will be decided. The patient should discuss the pros and cons of taking medications and treating the signs and symptoms with a doctor. They may need to change the medications or doses in case signs and symptoms flare and subside.

MEDICATIONS USED TO TREAT LUPUS:
In case signs and symptoms of lupus are mild, three types of commonly used medications could treat this condition. However, more aggressive medications may be needed if the lupus is more aggressive. Usually, when someone’s first diagnosed with lupus, the doctor may suggest:

NSAIDs:
These nonsteroidal anti-inflammatory drugs include aspirin, ibuprofen and naproxen sodium. Several signs and symptoms that are associated with lupus could be treated with these medications. There are over-the-counter NSAIDs, in addition to prescription ones. However, because some of the over-the-counter NSAIDs have been linked to serious side effects in people with lupus, patients should check with their doctor before taking them. Kidney problems, stomach bleeding and an increased risk of heart problems are some of the side effects of NSAIDs.

Antimalarial drugs:
These medications have been useful in treating the signs and symptoms of lupus, despite the fact that there's no known relationship between lupus and malaria. Flares of lupus could also be prevented by antimalarials. Muscle weakness and vision problems are some of the side effects of these drugs. The most commonly prescribed antimalarial is hydroxychloroquine.

Corticosteroids:
Even though these drugs could have some serious long-term side effects such as osteoporosis (thinning of the bones), easy bruising, diabetes, high blood pressure, increased risk of infection and weight gain, but they could counter the inflammation caused by lupus. The higher the dose and longer the term of therapy, the more the risk of side effects. Because of this, the patient will be given the lowest dose that controls the symptoms and for the shortest possible time. To help reduce the side effects, the patient could take the drug every other day. To help reduce the dosage, corticosteroids are sometimes given in combination with other medications, which also reduces the toxicity of these drugs. The risk of osteoporosis can be reduced by taking calcium and vitamin D supplements along with corticosteroids.

TREATMENT FOR SPECIFIC SIGNS AND SYMPTOMS:
Treatment will depend on the signs and symptoms. These are some of the treatments for specific signs and symptoms:

Swelling around the lungs and heart:
NSAIDs, corticosteroids or antimalarial drugs could be effective in reducing the swelling around the heart and lungs, which causes chest pain.

Joint pain and swelling:  
NSAIDs could control joint pain initially. However, the patient may be suggested corticosteroids or antimalarial drugs in case the joint pain becomes more significant.

Fatigue:
In order to treat fatigue, the underlying cause should be found and treated. Poorly controlled pain, difficulty sleeping or even depression could cause fatigue. However, someone may consider medications such as corticosteroids and antimalarial drugs in case the underlying cause of fatigue can't be found.

Skin rashes:
By keeping the skin covered, staying out of the sun and wearing sunblock year-round, skin rashes could be avoided. However, skin rashes in people with lupus could be caused by even indoor fluorescent lighting. In some cases, topical corticosteroids could treat these rashes; to reduce the inflammation in the skin cells, these creams are applied to the affected area. Other options include antimalarial drugs or oral corticosteroids.

TREATMENT FOR AGGRESSIVE LUPUS:
As mentioned before, more aggressive treatment may be needed for life-threatening cases of lupus, such as those that involve kidney problems, central nervous system problems and inflammation in the blood vessels. Some of those aggressive treatments include:

Immunosuppressive drugs:
As the name suggests, immunosuppressive drugs suppress the immune system. These medications could be taken intravenously or orally. Liver damage, an increased risk of cancer, an increased risk of infection and infertility are some of the side effects of these medications.  Even though these drugs could cause some serious side effects, but they are helpful in treating serious cases of lupus. Azathioprine and cyclophosphamine are the most commonly used immunosuppressive medications. To control lupus-related kidney problems, the drug mycophenolate could be used. In some cases, to control severe and treatment-resistant symptoms, the immune protein gamma globulin and the disease-modifying anti-rheumatic drug methotrexate could be used.

High dose corticosteroids:
Even though high dose corticosteroids could cause some serious side effects, such as mood swings, osteoporosis, infections and high blood pressure, but they could also control dangerous signs and symptoms more quickly. However, the patient will be given the lowest dose needed to control the signs and symptoms to minimize side effects, and that dosage will be reduced over time. High dose corticosteroids could be either administered through a vein in the arm (intravenously), or they could be taken orally.
To reduce the dosage of each drug, and thus reduce the risk of side effects, immunosuppressive drugs could be taken in combination with high-dose corticosteroids.

In some cases, kidney failure may occur, which could happen even with aggressive treatment. If this happens, the patient may need kidney dialysis or a kidney transplant if the condition is permanent.

Prognosis:

Not Available

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