Lupus

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Lupus


Lupus is a chronic inflammatory disease that can target your joints, skin, kidneys, blood cells, heart and lungs. The great majority of people affected are women. For reasons that aren't clear, lupus develops when the immune system attacks your body's own tissues and organs.

Three main types of lupus exist — systemic lupus erythematosus (SLE), discoid lupus erythematosus and drug-induced lupus. Of these, SLE is the most common and serious form of the disease, frequently causing swollen, painful joints, skin rash, extreme fatigue and kidney damage. In rare cases, mothers can pass antibodies to their babies during childbirth (neonatal lupus erythematosus), though the mothers themselves usually show no signs of lupus.

The outlook for people with lupus was once grim, but diagnosis and treatment of lupus has improved considerably. With proper care, most people with lupus can lead normal, active lives.


Signs and symptoms

No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Even the distinctive rash that gives the disease its name — "lupus" is the Latin word for "wolf" because doctors once thought the rash resembled a wolf bite — doesn't occur in every case.

Most people with lupus have one thing in common, however, and that's the tendency of the disease to get decidedly worse in episodes called flares and then to improve or even disappear completely for a time.

Common signs and symptoms
The most common signs and symptoms of lupus, which usually develop at intervals, rather than all at once, include the following:

Rash. Although it only affects about one in three people with lupus, the skin problem most often associated with the disease is a butterfly-shaped rash (malar rash) that develops across the cheeks and bridge of the nose. A malar rash may be flat or raised and may be blotchy or completely red in the affected areas.

Some people with lupus develop skin lesions (subacute cutaneous lesions) that initially resemble small pimples but that eventually turn scaly and itchy, whereas others have large, flat, itchy lesions with clear centers. Both types of lesions usually appear after exposure to sunlight.

 Still other people develop a crusty, red, raised rash (discoid rash) on the face, neck, chest or scalp. A discoid rash is usually thick and scaly, may last for days or years, and often leaves hypopigmented or hyperpigmented scars. This rash is the only symptom in people with the discoid form of lupus. In a small percentage of cases, people with discoid lupus may go on to develop a mild version of SLE.

Arthritis.
Most people with lupus eventually develop joint pain, stiffness and swelling, mainly in their fingers, hands, wrists and knees. The pain may shift from one joint to another or affect the same joint on both sides of the body. Lupus-associated arthritis can come and go quickly and usually isn't deforming, although the pain can be severe during a flare.

Kidney problems.
People with lupus are especially vulnerable to kidney damage — lupus can cause numerous types of glomerulonephritis, a condition that affects the kidneys' ability to filter toxins, leading to kidney failure. Although some people with kidney problems may develop frothy or tea-colored urine, swelling in their ankles or lower legs, or high blood pressure, kidney damage often occurs without any warning signs or symptoms.

Sensitivity to sunlight (photosensitivity).
People with lupus are often highly sensitive to sunlight, frequently developing severe rashes on sun-exposed skin. Sometimes even indoor lighting, especially fluorescent lighting, can cause the same response.
 
Brain or central nervous system problems.
Lupus can cause a range of serious problems related to the brain or central nervous system, including headaches, seizures, dizziness, vision problems, behavior changes or stroke.

Heart problems.
Lupus can affect all parts of the heart, including the sac surrounding the heart (pericardium), the heart muscle (myocardium) and the lining of the inside of the heart (endocardium).

Pericarditis, which occurs when the pericardium becomes inflamed, is particularly common in people with lupus, causing shortness of breath and sharp chest pain that may feel like a heart attack.

Lung problems.
Lupus can cause a number of lung problems, the most common of which is pleurisy, an inflammation of the chest cavity lining that can produce sharp, stabbing chest pain. Less common lung conditions include pleural effusion, which is the accumulation of excess fluid in the pleural space between the lung and the chest wall, and lupus pneumonitis, a lung inflammation.

Occasionally, people with lupus develop high blood pressure in the blood vessels in the lungs (pulmonary hypertension).
 
Mucosal ulcers.
These generally painless sores develop in the mouth, especially the roof of the mouth or, less often, in the nose. You're not likely to notice mucosal ulcers unless you eat spicy food or experience recurring nosebleeds.
 
Blood vessel disorders.
Inflamed blood vessels can affect blood circulation. The inflammation may be mild and not require treatment or it can be quite severe.
 

Other signs and symptoms also may occur that aren't specific to lupus. These include:

Fatigue.
The fatigue that accompanies lupus is not the same tiredness you experience after a sleepless night or hard physical work. Instead, it's an ongoing, extreme exhaustion that's usually not relieved by rest.
 
Fever.
An unexplained fever, especially a chronic fever higher than 100 F may be an early sign of lupus.

Raynaud's phenomenon.
In this condition your fingers, toes, nose and ears turn pale and numb when exposed to cold temperatures.

Swelling.
You may have swollen glands or swelling in your legs or around your eyes.
 
Digestive problems.
These problems may include abdominal pain, weight loss, nausea and vomiting.
 
Hair loss.
Because lupus can affect the skin of your scalp, you may experience some patchy hair loss.
 
Depression.
People with lupus may also experience depression or difficulty concentrating, either because of the disease itself or because of the challenges of living with a chronic condition.



Causes

Lupus is an autoimmune disease, which means that instead of just attacking foreign substances, such as bacteria and viruses, the immune system also turns against healthy tissue. This leads to inflammation and damage to various parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels and brain.

Why autoimmune diseases occur still isn't well understood. But doctors believe that like many diseases, lupus results from a combination of factors, which may include heredity, environment and hormones. Although lupus isn't directly inherited, it's likely that inheriting certain genes makes you more susceptible to the disease, which then may be triggered by certain factors, such as:

Infection.
It appears that a viral or bacterial infection may trigger SLE in vulnerable people. In particular, lupus may be linked with recurrent infections with the Epstein-Barr virus, the same virus that causes mononucleosis.
 
Certain prescription medications. One of the three main forms of lupus — drug-induced lupus — results from the long-term use of certain prescription drugs. Although many drugs can potentially trigger lupus, those most clearly linked with the disease include the antipsychotic chlorpromazine, high blood pressure medications such as hydralazine, the tuberculosis drug isoniazid and the heart medication procainamide.

Beta blockers have also been associated with lupus, as have some drugs used to treat arthritis and ulcers, and certain antibiotics such as minocycline. It usually takes several months or years of therapy with these drugs before symptoms appear, and even then, only a small percentage of people will ever develop lupus.

Unlike SLE, drug-induced lupus affects more men than women, primarily because men are more likely to develop chronic conditions that require long-term treatment. And unlike other types of lupus, symptoms such as joint pain and swelling, fever, and fatigue usually disappear after stopping the medication, although you may require short-term treatment with NSAIDs or corticosteroids.
 
Sunlight.
Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people. Exactly why ultraviolet radiation has this effect isn't well understood, but scientists suspect that sunlight may cause skin cells to express certain proteins on their surface.

Antibodies that are normally present in the body then latch onto these proteins, initiating an inflammatory response. Damaged skin cells also seem to die more frequently in people with lupus, leading to even more inflammation.
 
Hormones.
Because so many more women than men have SLE, researchers think that female hormones, particularly estrogen, may play a role in the disease. The exact mechanism isn't known, but some women with lupus report that their symptoms become worse during menstruation and pregnancy and with the use of birth control pills or hormone therapy.

On the other hand, although the Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA) trial found a small risk of increased mild to moderate flares of the disease in menopausal women treated with hormone therapy, severe flares were rare. In addition, studies suggest that oral contraceptives are well tolerated by most but not all women with lupus.

Many other factors have been suggested as possible causes of lupus, including stress, certain foods, the artificial sweetener aspartame, silicone breast implants, mercury dental fillings, hair dye, and pesticides and other toxic chemicals. To date, no clear link has been found between these factors and lupus.



Risk factors

Although anyone can develop lupus at any age, common risk factors include:

Your sex.
Women account for the vast majority of lupus cases and are approximately nine times as likely to develop the disease as men are.
 
Age.
Although lupus affects people of all ages, including infants and children, it's most often diagnosed between the ages of 15 and 45.

Race.
Black Americans are at increased risk of developing lupus. They also tend to develop lupus at a younger age and have more serious complications. Researchers don't know the reason why, but in studies, blacks were found to have many more recurrent Epstein-Barr virus infections.

Hispanics, Asians and Native Americans also are at higher risk of developing lupus.
 
Family history. Having a relative who has lupus increases your odds of developing the disease.

Infection with the Epstein-Barr virus. Almost everyone has been infected with the Epstein-Barr virus, a member of the herpes family and one of the most common human viruses. Once the initial infection, which is usually marked by nonspecific symptoms such as fever and sore throat, subsides, the virus remains dormant in the cells of your immune system unless something reactivates it. For reasons that aren't clear, recurrent Epstein-Barr infections seem to greatly increase the chances of developing lupus.

Pregnancy.
Lupus sometimes shows up for the first time during pregnancy or shortly after giving birth. The disease may also flare after a woman who already has lupus gives birth.

When to seek medical advice

If you develop an unexplained rash, ongoing fever, persistent aching or fatigue, see your doctor. If you've already been diagnosed with lupus, meet with your doctor on a regular basis so that your condition and treatment can be monitored. And because people who have lupus can experience different symptoms at different times, see your doctor immediately if new symptoms arise, especially if they include any of the following:

Blood in your stool
Severe abdominal pain
Chest pain from pleurisy or pericarditis
Seizures
New fever or a fever much higher than it usually is
Unusual bruising or bleeding anywhere on your body
A severe headache with neck pain and fever

Screening and diagnosis

Lupus has been called a disease with a thousand faces because the signs and symptoms vary considerably from person to person. What's more, problems associated with the disease change over time and overlap with those of many other disorders.

 For these reasons, doctors may not initially consider lupus until the signs and symptoms become more obvious. Even then, lupus can be challenging to diagnose because nearly all people with lupus experience fluctuations in disease activity. At times the disease may become severe and at other times subside completely.

The American College of Rheumatology (ACR) has developed clinical and laboratory criteria to help physicians diagnose and classify lupus. If you have four of the 11 criteria at one time or individually over time, you probably have lupus. Your doctor may also consider the diagnosis of lupus even if you have fewer than four of these signs and symptoms. The criteria identified by the ACR include:

Malar rash — a butterfly-shaped rash that covers the bridge of the nose and spreads across the cheeks
Discoid rash — raised, scaly patches that may cause scarring
Marked sensitivity to sunlight
Oral ulcers
Arthritis that involves, but doesn't destroy, two or more peripheral joints
Inflammation of the lining of the heart or lung (serositis)
Kidney (renal) disease
A neurological disorder, such as seizures or psychosis
A blood (hematologic) disorder, such as anemia, low platelets (thrombocytopenia), or a low white cell count (leukopenia)
Anti-nuclear antibody, an indication that you may have an autoimmune disease
Immunological disorder — a positive double-stranded anti-DNA test, positive anti-Sm test, positive antiphospholipid antibody or false-positive syphilis test — any one of which may indicate an autoimmune disease
 
Laboratory tests
In addition to using your clinical history and a physical examination to check for the classification criteria, your doctor will use laboratory tests. These may include:

Complete blood count.
This test measures the number of red blood cells, white blood cells and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may indicate you have anemia, which commonly occurs in lupus. Low white blood cell or platelet counts may occur as well.
 
Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in an hour. A faster-than-normal rate may indicate a systemic disease such as lupus. The sedimentation rate is not specific for any one disease but may be elevated if you have lupus, another inflammatory condition or an infection.

Kidney and liver assessment.
Blood tests can assess how well your kidneys and liver are functioning because lupus can affect these organs.
 
Urinalysis.
An examination of a sample of your urine may show an increased protein level or red blood cells in the urine, which may occur if lupus has affected your kidneys.
 
Antinuclear antibody (ANA) test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system, which is common in lupus and other autoimmune diseases. A positive ANA doesn't always mean that you have lupus, however. ANA levels can be elevated if you have an infection or if you're taking certain medications.
If you test positive for ANA, your doctor may advise more specific antibody testing and refer you to a rheumatologist, a doctor who specializes in musculoskeletal and autoimmune disorders such as arthritis or lupus.

Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs. It may also show an enlarged heart as a result of a buildup of fluid within the pericardium (pericardial effusion).

Electrocardiogram (ECG). This test measures the pattern of electrical impulses generated in your heart. It can help identify irregular rhythms or damage.

Syphilis test. Strange as it may seem, a false-positive result on a syphilis test can indicate anti-phospholipid antibodies in your blood, another indication of lupus. The presence of anti-phospholipid antibodies has been associated with an increased risk of blood clots, strokes and recurrent miscarriages.



Complications

With treatment, most people with lupus can live active, healthy lives. Without treatment, complications from lupus can be life-threatening. These complications include:

Kidneys.
Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. Kidney problems are often symptomless, but can be detected through routine urine and blood tests, and if needed, a kidney biopsy. A blood test called serum creatinine level is used to check kidney function.

Central nervous system.
If your central nervous system is affected by lupus, you may experience headaches, dizziness, memory problems, behavior changes, even seizures.
 
Blood and blood vessels. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis).
 
Lungs.
Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy) that can make breathing painful. You may also be more susceptible to a noninfectious form of pneumonia.
 
Heart.
Lupus can cause inflammation of your heart muscle (myocarditis and endocarditis), your arteries (coronary vasculitis) or heart membrane (pericarditis).
Having lupus also greatly increases your risk of cardiovascular disease and heart attacks. Controlling high blood pressure and high blood cholesterol, not smoking, and getting regular exercise are essential to help reduce the risk of heart disease.
 
Infection.
People with lupus are vulnerable to infection because both the disease and its treatments — corticosteroid and cytotoxic drugs, in particular — affect the immune system. And in a vicious cycle, infection can bring on a lupus flare, increasing the risk of infection even more.
 
Cancer.
Having lupus appears to increase your risk of cancer, especially non-Hodgkin's lymphoma, which affects the lymph system, lung cancer, and liver and bile duct cancers. Immunosuppressant drugs that are sometimes used to treat lupus can also up the risk of cancer.
 
Bone tissue death (avascular necrosis).
This occurs when the blood supply to a bone diminishes, often leading to tiny breaks in the bone and eventually to the bone's collapse. The hip joint is commonly affected, although avascular necrosis can occur in other bones as well.

Avascular necrosis can be caused by lupus itself or by high doses of corticosteroids used to treat the disease.

Risks of complications for younger women
Lupus poses special health risks for women during their childbearing years.

 Complications may include:
Increased risk of miscarriage. The risk is usually highest early or late in pregnancy, but careful planning and treatment can minimize the chance of a miscarriage in women with lupus.
Increased risk of complications during pregnancy. Women with lupus are more likely to experience a flare during pregnancy. They're also at greater risk of high blood pressure, diabetes, kidney problems and preterm birth.
Limited birth control options. Women with lupus may not tolerate birth control pills well, and intrauterine devices (IUDs) pose an increased risk of infection.



Treatment

The treatments for lupus can be as varied and individual as the disease itself and may change over time. But common-sense measures, such as rest, protection from sunlight, exercise, not smoking and a healthy diet are important for everyone with lupus.

Beyond this, medications can ease symptoms and reduce complications of lupus, but these drugs carry their own risks. In recent years, more judicious use of drugs has helped reduce some of these risks. The type of medication and length of treatment depend on which parts of the body are affected and the severity of symptoms. Yet because the course of lupus is so unpredictable, doctors may need to try several different drugs or change doses before finding an effective treatment.

Some medications used to treat lupus include:

Nonsteroidal anti-inflammatory drugs.

Aspirin or other nonsteroidal anti-inflammatory medications (NSAIDs) such as naproxen sodium and ibuprofen may reduce joint pain and inflammation. Check with your doctor before taking NSAIDs because some have been associated with serious side effects in people with lupus. What's more, a medication you've taken may have caused drug-induced lupus in the first place and taking more drugs may make the problem even worse.
 
Antimalarial drugs.
Although there's no known relationship between lupus and malaria, these medications may be useful for treating skin and joint problems and the ulcers that some people develop in the mouth or nose. Antimalarials may also prevent flares of the disease. Hydroxychloroquine (Plaquenil), the most commonly prescribed antimalarial, is sometimes used to control lupus in pregnant women because it appears to have fewer risks to the fetus than other medications used to treat lupus do.
 
Corticosteroids.
These drugs counter the inflammation of lupus, but can have serious long-term side effects, including weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection, already a major concern for people with lupus. The risk of side effects increases with higher doses and longer-term therapy.

 To help reduce these risks, your doctor will try to find the lowest dose that controls your symptoms and prescribe corticosteroids for the shortest possible time. Taking the drug every other day can also help reduce side effects. Corticosteroids are sometimes combined with another medication to help reduce the dose, and therefore the toxicity, of both drugs. Taking calcium and vitamin D supplements while using corticosteroids can reduce the risk of osteoporosis.
 
Immunosuppressive medications.
Drugs such as azathioprine (Imuran) and cyclophosphamide (Cytoxan) work by suppressing the immune system. Other medications that may be used to treat lupus include methotrexate (Rheumatrex), chlorambucil (Leukeran), cyclosporine (Neoral, Sandimmune) and mycophenolate mofetil (CellCept).

Because immunosuppressive medications can have serious side effects, they're usually reserved for people with severe systemic disease or organ failure. Immunosuppressive drugs can cause anemia and a low white blood cell count, and increase your risk of infection and cancer.
Sometimes, even with the use of corticosteroids and immunosuppressive drugs, your kidneys may fail. In that case, you may need kidney dialysis or, if kidney failure is permanent, a kidney transplant.

New treatments
Researchers continue to look for more effective lupus treatments. Some studies have focused on blocking the expression of genes that may cause some of the symptoms of lupus, but any drugs that might result from this research are still years away.

Scientists are also investigating the use of existing drugs to treat lupus. One medication that has received attention is the cancer drug rituximab, which works by lowering the number of white blood cells in the body.

 Although people with lupus usually have lower than normal B cell counts, the B cells that do exist are involved in the autoimmune process. In studies, people with lupus who were treated with rituximab had a reduction in symptoms and few serious side effects. More studies are needed, however, to fully understand the risks and benefits of using rituximab to treat lupus.




Self-care

Staying healthy requires extra effort for people with lupus, yet recognizing when symptoms are getting worse and knowing how to treat them can reduce your chance of permanent tissue or organ damage. Early treatment can also reduce the time you spend taking higher doses of medications, which can cause serious side effects.

In addition to learning to recognize the warning signs of a flare, such as pain, rash, fever, increased fatigue, abdominal discomfort, headache and dizziness, you also can take steps to help prevent flares in the first place:

Get adequate rest. People with lupus often experience persistent fatigue that's different from normal tiredness and that isn't necessarily relieved by rest. For that reason, it can be hard to judge when you need to slow down. Many experts recommend eight to 10 hours of sleep a night and naps or breaks during the day as needed. Friends and family members need to understand and respect your need for rest.
 
Be sun smart. Because ultraviolet light can trigger a flare, wear protective clothing such as a hat, long-sleeved shirts and long pants, and use sunscreens with a sun protection factor (SPF) of at least 15 every time you go outside, even if it's just a quick trip to the mailbox.
Be sure that your ears, scalp and the backs of your hands are protected.

Avoid tanning beds and stay out of the sun entirely when it's strongest, from 10 a.m. to 4 p.m. Because fluorescent and halogen lights also can emit UV rays and thus aggravate lupus, you may need to wear sunscreen and protective clothing indoors or use plastic devices that block UV emissions from indoor lights.

Learn to deal with stress. Many people with lupus find that stress can bring on symptoms of their disease. It's not possible to avoid stress, especially when living with a chronic illness, but you often can learn to cope with it constructively so that it doesn't take such a severe physical toll.

Try various relaxation techniques such as yoga, meditation or deep breathing or consider working with a counselor who can help you learn to deal with stress.

Get regular exercise. It might seem counterintuitive to suggest exercise for people who are frequently exhausted, but in fact, physical activity in general and regular exercise in particular is extremely important for people with lupus.
Exercise can help you recover from a flare, reduce your risk of heart attack, help fight depression and promote general wellbeing. Time outdoor activities so that you avoid the sun when it's most intense, and if you're having a flare, stay out of the sun entirely.
 
Don't smoke.
Smoking increases your risk of cardiovascular disease and can worsen the effects of lupus on your heart and blood vessels.
 
Limit alcohol.
Alcohol can affect your liver, kidneys, heart and muscles, and may interact with your medications.
 
Eat a healthy, balanced diet.
A well-balanced, nutritious diet is important for everyone, including people with lupus. Sometimes you may have dietary restrictions, especially if you have high blood pressure, kidney damage or gastrointestinal problems. And although no specific foods have been shown to cause or exacerbate lupus, it's best to avoid any food that seems to make your symptoms worse.
 
Take care with vaccines.
Immunizations using killed vaccines don't seem to make lupus worse, but you should avoid live vaccines such as the intranasal flu vaccine. It's best to check with your doctor regarding all immunizations.
 
If you're a woman with lupus and are considering becoming pregnant, seek medical counseling to determine what steps you can take to ensure the safest possible pregnancy. And keep in mind that some lupus medications are contraindicated during pregnancy.
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Systemic Lupus Erythematosus

Lupus is a disease that affects thousands of Canadians. One in 2,000 Canadians have this autoimmune disease, totaling over 15,000 people. Women are up to ten times more likely to develop the disease than men, usually between the ages of 15 and 45 years.

“Autoimmune” diseases are as a result of an over-active immune system in which the cells of immunity are attacking the affected person’s own healthy tissues. Other examples of autoimmune diseases include certain types of thyroid problems, type I diabetes, vitiligo, and inflammatory disease. With lupus, the immune system attacks the affected person’s joints, skin, and muscles, but can become “systemic.” This means that many parts of the body are involved. This is the most common and most serious type of lupus, and is referred to as ‘systemic lupus erythematosus,’ or SLE. The heart, kidneys, blood vessels, lungs, and nervous system are the next most frequently affected parts of the body in SLE.

With the attack of the body’s healthy tissues lupus causes swollen, tender, and red areas of inflammation. The disease will flare up and then back down again, with periods of remission where the person feels quite good. Other types of lupus that are more restricted to the skin and less serious than SLE are: discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). About 10% of people who develop these other forms of lupus will go on to the systemic form unfortunately.

Warning signs of lupus include pain in the joints and muscles of the hands, arms, shoulders, jaw, hips, or knees. The skin may look red and feel hot to the touch and the pain may come and go. With lupus, the person may lose their appetite, have a fever, and lose weight. There is a classic butterfly-shaped rash that appears on the face and spreads over the cheeks. Mucosal ulcers or small and painless sores can appear in the mouth or nose. People with lupus become very sensitive to the sunlight and may have color changes in their fingers and toes with cold temperatures (Raynaud’s Phenomenon). Hair loss and headaches are common, and chest pain is a possible symptom as well.

Diagnosis of lupus is done with blood tests and if there are skin symptoms, a biopsy may be needed where a sample is analysed in a laboratory. Treatment does not cure the disease at this point, but is aimed at decreasing the number of flare-ups and improving symptoms. Anti-inflammatories are usually part of treatment when there is a flare-up. Of all things, medicines that are for treating malaria work to help with the tiredness, rashes, and joint pain in people with lupus. Stronger medications like chemotherapy drugs may be needed. Diet and exercise are very important to prevent flare-ups, and too much alcohol is usually a problem. People with lupus need to protect their joints and this means to try and avoid anything that puts extra stress on them.

In all, people with lupus usually lead normal lives if they maintain balanced, healthy diets and stay active. There are support groups as it can be quite discouraging to be diagnosed with lupus. Ask your doctor for help in finding a group in your area if you suffer from lupus.

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Lupus and the symptoms of nervous system involvement   


Studies revealed that is possible for systemic lupus erythematosus to affect the nervous system. Patients with lupus may experience confusion, difficulty with concentrating, headaches, fatigue, strokes or other signs that show nervous system involvement.

Studies suggested that the nerve tissue may be damaged when antibodies attack nerve cells or blood vessels. It is known that the nervous system requires an uninterrupted flow of blood, that is needed to supply with oxygen and nutrients its tissues. When this flow of blood is slowed or interrupted, the nervous cells are unable to function normally, and there appear symptoms. The symptoms vary, depending where the tissue injury is situated.It is good to know that the nervous system contains three parts. The central nervous system comprises the brain and spinal cord, the peripheral nervous system nerve fibers that have the role to provide the skin and muscles the power needed for sensation and movement, and the third part is the autonomic nervous system that has the role to regulate spinal, peripheral nerves and to innervate the internal organs.
 
An inflammation of the blood vessels of the brain that appears to 10% of all lupus patients is called the central nervous system vasculitis.This disease usually requires hospitalization and high doses of corticosteroids. Some of the symptoms that appear are high fevers, seizures, psychosis and meningitis-like stiffness of the neck. If it is not aggressively managed, the central nervous system vasculitis rapidly progresses to stupor and coma.

People with mild to moderated systemic lupus erythematosus can experience the cognitive dysfunction. That is a group of symptoms that appear at about 50% of these patients, and we can mention here fatigue, memory impairment, feelings of confusion, and difficulty to express the thoughts. By taking a neuropsychological test or a test called the positron emission tomography, these symptoms can be clearly documented.It is known that cognitive dysfunction may come and go on its own, but no optimal therapy is available. Also, it is not known which is the reason for the symptoms that appear. Dealing with a cognitive dysfunction is frustrating, and often counseling a person in developing coping skills may be helpful.
About 20% of the patients having systemic lupus erythematosus experience the lupus headache. This manifests by severe headaches, is similar to migraine and can be often seen in persons who have also Raynaud's phenomenon. As a treatment, it is useful the same one used in tension headaches or migraine, and sometimes corticosteroids.
 
It is known that about a third of the patients having lupus can have a false positive syphilis test, a positive anticardiolipin antibody, or a prolonged clotting time test.This is known under the name of the lupus anticoagulant or the antiphospholipid antibody. About 1/9 of the patients having lupus will develop blood clots in various parts of the body, which is called the antiphospholipid syndrome.If blood clots appear in the nervous system, they can cause a stroke, and symptoms of a stroke include the painless onset of neurologic deficits without any signs of active lupus.If a stroke appears, there must be taken blood-thinning medications. We can mention here low-dose aspirin, coumadin or heparin.

Among patients having systemic lupus erythematosus there is a percentage of 20% that have fibromyalgia syndrome as well. These patients experience increased pain in the soft tissues, tender points, and, in addition cognitive dysfunction, decreased ability to concentrate, lack of stamina and difficulty sleeping. As a treatment, we can mention anti-depressants, counseling, and physical therapy if needed.

It was discovered that medications used to treat systemic lupus erythematosus can develop symptoms like those of the central nervous system lupus.Psychosis can appear due to anti-malarials in very high doses; headache, dizziness, and in rare situations meningitis-like symptoms can be provoked by nonsteroidal anti-inflammatory drugs. Also if a patient uses corticosteroids, there can appear moods swings, psychosis, depression, agitation, confusion, if there are taken high doses can appear seizures, and anti-hypertensive medications may be associated with depression or loss of libido.

A study discovered that people that have both lupus and Sjogren's syndrome may be predisposed to develop vasculitis or cognitive dysfunction. Sometimes, circulating proteins in the blood can lead to cryoglobulinemia or hyperviscosity syndrome.Plasmapheresis or filtering the blood can ease these complications.Sometimes, pronounced decreases in platelet counts may be associated with bleeding.People with thrombotic thrombocytopenic purpura or who lack Protein S or Protein C may clot, and those with lupus, idiopathic thrombocytopenic purpura and kidney failure may bleed.

In peripheral nervous system lupus, involvement of the cranial nerves can cause visual disturbances, drooping of the eyelid(s), ringing in the ear(s), facial pain and dizziness.Symptoms of numbness or tingling in the arms or legs can appear if there is an inflammation of the blood vessels supplying the peripheral nerves. There can also appear symptoms due to other conditions than lupus and electromyogram and nerve conduction tests are usually helpful to determine if symptoms are due to some other cause. Corticosteroids are used to treat inflammation of the peripheral nerves.

It is important for your doctor to know if you experience nervous system symptoms. It is possible that these symptoms to appear due to lupus, due to a medication or a particular aspect of your life. The doctor will ask you about the symptoms you experience, he will perform a physical examination and a laboratory evaluation including a blood chemistry panel, complete blood count and urinalysis. Also, diagnostic tests like sedimentation rate, ANA, anti-DNA, anti-ribosomal P antibodies and complement may be useful in order to determine nervous system involvement.There are neurodiagnostic tests, that include CT and MRI brain scans, brain waves or electroencephalogram and spinal taps.In a few hospitals, there can also be performed PET scans.The spinal fluid may be examined for cells, protein components and antineuronal antibodies. In patients with cognitive dysfunction, neuropsychologic tests may be helpful.

The treatment for nervous system lupus depends upon its source, and can include immunosuppressants, blood thinners, antibiotics, steroids, anti-depressants, counseling or surgery. If there are evident diagnostic difficulties, a rheumatologist and/or neurologist should be involved in your care. It was seen that for many people with lupus, nervous system involvement is completely reversible.






 
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