Lupus Disease

Lupus is a disease that occurs when your body's immune system attacks your own tissues and organs (autoimmune disease). Inflammation caused by lupus can affect many different body systems — including your joints, skin, kidneys, blood cells, brain, heart and lungs.

Lupus can be difficult to diagnose because its signs and symptoms often mimic those of other ailments. The most distinctive sign of lupus — a facial rash that resembles the wings of a butterfly unfolding across both cheeks — occurs in many but not all cases of lupus.

Some people are born with a tendency toward developing lupus, which may be triggered by infections, certain drugs or even sunlight. While there's no cure for lupus, treatments can help control symptoms.

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. Most people with lupus have mild disease characterized by episodes — called flares — when signs and symptoms get worse for a while, then improve or even disappear completely for a time.

The signs and symptoms of lupus that you experience will depend on which body systems are affected by the disease. The most common signs and symptoms include:

• Fatigue

• Fever

• Joint pain, stiffness and swelling

• Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body

• Skin lesions that appear or worsen with sun exposure

• Fingers and toes that turn white or blue when exposed to cold or during stressful periods

• Shortness of breath

• Chest pain

• Dry eyes

• Headaches, confusion and memory loss

Lupus facial rash

A typical sign of lupus is a red, butterfly-shaped rash over your cheeks and nose, often following exposure to sunlight.

Causes

As an autoimmune disease, lupus occurs when your immune system attacks healthy tissue in your body. It's likely that lupus results from a combination of your genetics and your environment.

It appears that people with an inherited predisposition for lupus may develop the disease when they come into contact with something in the environment that can trigger lupus. The cause of lupus in most cases, however, is unknown. Some potential triggers include:

• Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people.

• Infections. Having an infection can initiate lupus or cause a relapse in some people.

• Medications. Lupus can be triggered by certain types of blood pressure medications, anti-seizure medications and antibiotics. People who have drug-induced lupus usually get better when they stop taking the medication. Rarely, symptoms may persist even after the drug is stopped.

Risk factors

Factors that may increase your risk of lupus include:

• Your sex. Lupus is more common in women.

• Age. Although lupus affects people of all ages, it's most often diagnosed between the ages of 15 and 45.

• Race. Lupus is more common in African Americans, Hispanics and Asian Americans.

Complications

Inflammation caused by lupus can affect many areas of your body, including your:

• Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus.

• Brain and central nervous system. If your brain is affected by lupus, you may experience headaches, dizziness, behavior changes, vision problems, and even strokes or seizures. Many people with lupus experience memory problems and may have difficulty expressing their thoughts.

• Blood and blood vessels. Lupus may lead to blood problems, including a reduced number of healthy red blood cells (anemia) and an increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels.

• Lungs. Having lupus increases your chances of developing an inflammation of the chest cavity lining, which can make breathing painful. Bleeding into lungs and pneumonia also are possible.

• Heart. Lupus can cause inflammation of your heart muscle, your arteries or heart membrane. The risk of cardiovascular disease and heart attacks increases greatly as well.

Other types of complications

Having lupus also increases your risk of:

• Infection. People with lupus are more vulnerable to infection because both the disease and its treatments can weaken the immune system.

• Cancer. Having lupus appears to increase your risk of cancer; however, the risk is small.

• Bone tissue death. This occurs when the blood supply to a bone declines, often leading to tiny breaks in the bone and eventually to the bone's collapse.

• Pregnancy complications. Women with lupus have an increased risk of miscarriage. Lupus increases the risk of high blood pressure during pregnancy and preterm birth. To reduce the risk of these complications, doctors often recommend delaying pregnancy until your disease has been under control for at least six months.

Diagnosis

Diagnosing lupus is difficult because signs and symptoms vary considerably from person to person. Signs and symptoms of lupus may change over time and overlap with those of many other disorders.

No one test can diagnose lupus. The combination of blood and urine tests, signs and symptoms, and physical examination findings leads to the diagnosis.

Laboratory tests

Blood and urine tests 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. A low white blood cell or platelet count may occur in lupus 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 isn't specific for any one disease. It may be elevated if you have lupus, an infection, another inflammatory condition or cancer.

• Kidney and liver assessment. Blood tests can assess how well your kidneys and liver are functioning. 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. While most people with lupus have a positive antinuclear antibody (ANA) test, most people with a positive ANA do not have lupus. If you test positive for ANA , your doctor may advise more-specific antibody testing.

Imaging tests

If your doctor suspects that lupus is affecting your lungs or heart, he or she may suggest:

• Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs.

• Echocardiogram. This test uses sound waves to produce real-time images of your beating heart. It can check for problems with your valves and other portions of your heart.

Biopsy

Lupus can harm your kidneys in many different ways, and treatments can vary, depending on the type of damage that occurs. In some cases, it's necessary to test a small sample of kidney tissue to determine what the best treatment might be. The sample can be obtained with a needle or through a small incision.

Skin biopsy is sometimes performed to confirm a diagnosis of lupus affecting the skin.

Treatment

Treatment for lupus depends on your signs and symptoms. Determining whether you should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor.

As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages. The medications most commonly used to control lupus include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs), such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin IB, others), may be used to treat pain, swelling and fever associated with lupus. Stronger NSAIDs are available by prescription. Side effects of NSAIDs may include stomach bleeding, kidney problems and an increased risk of heart problems.

• Antimalarial drugs. Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil), affect the immune system and can help decrease the risk of lupus flares. Side effects can include stomach upset and, very rarely, damage to the retina of the eye. Regular eye exams are recommended when taking these medications.

• Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (Medrol) are often used to control serious disease that involves the kidneys and brain. Side effects include weight gain, easy bruising, thinning bones, high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy.

• Immunosuppressants. Drugs that suppress the immune system may be helpful in serious cases of lupus. Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept), methotrexate (Trexall, Xatmep, others), cyclosporine (Sandimmune, Neoral, Gengraf) and leflunomide (Arava). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.

• Biologics. A different type of medication, belimumab (Benlysta) administered intravenously, also reduces lupus symptoms in some people. Side effects include nausea, diarrhea and infections. Rarely, worsening of depression can occur.
  Rituximab (Rituxan, Truxima) may be beneficial for some people in whom other medications haven't helped. Side effects include allergic reaction to the intravenous infusion and infections.

In clinical trials, voclosporin has been shown to be effective in treating lupus.

Other potential drugs to treat lupus are currently being studied, including abatacept (Orencia), anifrolumab and others.