What is Lupus?
What is Lupus?
Lupus is a chronic (long-term) disease that can cause inflammation and pain in any part of your body. It’s an autoimmune disease, which means that your immune system — the body system that usually fights infections — attacks healthy tissue instead.
Lupus most commonly affects your:
- Internal organs, like your kidneys and heart
Because lupus affects many parts of the body, it can cause a lot of different symptoms.
Because lupus can affect so many different parts of the body, it can cause a lot of different symptoms. And many people with lupus don’t have all the symptoms.
Common signs and symptoms of lupus
The most common lupus symptoms (which are the same for men and women) are:
- Extreme fatigue (feeling tired all the time)
- Pain or swelling in the joints
- Swelling in the hands, feet, or around the eyes
- Low fevers
- Sensitivity to sunlight or fluorescent light
- Chest pain when breathing deeply
Many people with lupus also have problems that affect their skin and hair, like:
- A butterfly-shaped rash on the cheeks and nose
- Hair loss
- Sores in the mouth or nose
- Fingers and toes turning white or blue and feeling numb when a person is cold or stressed (Raynaud’s Disease)
Lupus symptoms may come and go — and they can change over time.
If I have these symptoms, does that mean I have lupus?
Not necessarily. Lupus shares a lot of symptoms with other diseases, like arthritis and diabetes.
So if you have these common lupus symptoms, it’s important to talk to your doctor and find out whether you have lupus or a different health problem. That way, you can get the treatment you need.
Diagnosing lupus can be challenging. There’s no single test that can give doctors a “yes” or “no” answer. Sometimes it can take months—or even years—to gather all the right information.
Making a lupus diagnosis is kind of like putting together a puzzle. Your doctor will look at several different puzzle pieces: your symptoms, medical history, family history, and lab tests. If enough of the pieces fit together, you may be diagnosed with lupus.
What questions will my doctor ask?
If your doctor thinks you might have lupus, they’ll ask you questions about your symptoms, like:
- What symptoms are you having?
- How often do you have these symptoms?
- When did your symptoms start?
- Does anything make your symptoms better or worse?
- Are your symptoms constant or do they come and go?
- Do your symptoms get worse at a certain time of day?
- Do your symptoms get in the way of your daily routine?
Your doctor may also ask if anyone in your family has had lupus or another autoimmune disease (a disease where the immune system attacks healthy tissue). That’s because people who have a family member with an autoimmune disease may be more likely to develop lupus.
What types of tests can help diagnose lupus?
Your doctor might give you different lab tests to figure out if you have lupus. While no single test can diagnose lupus, tests help doctors check for changes in your body — like inflammation — that could be caused by lupus.
What is inflammation?
Inflammation usually happens when your immune system is fighting an infection or an injury. When lupus makes your immune system attack healthy tissue, it can cause inflammation in lots of different body parts. Symptoms can include swelling and pain.
Blood tests can help doctors see things like how your immune system is working, or if there are signs of inflammation in your body. Your doctor may ask you to get these blood tests:
- A complete blood count (CBC) to measure the numbers of red blood cells, white blood cells, and platelets (cells that help blood clot) in your blood
- Antibody tests to find out if your immune system is attacking healthy tissue
- Blood clotting time tests to see if you have clotting problems
- Complement tests to check for signs of inflammation
Urine (pee) tests can help doctors see if there are problems with your kidneys. Your doctor may test your urine once or test it many times to check for changes.
Doctors may remove a small piece of tissue (what your organs are made of) from different parts of your body, like your skin. Then they can check that tissue to see if there are any signs of inflammation and damage.
Lab tests for lupus
Many different laboratory tests are used to detect changes or conditions in your body that can occur with lupus. Each test result adds more information to the picture your doctor is forming of your illness.
The most common types of tests you may be asked to get are blood and urine tests. It is important to understand these laboratory tests so you can feel confident as you work with your doctor to better understand your health.
There are several important things to keep in mind with lab tests:
- Lab work alone usually cannot diagnose lupus. Signs and symptoms of the disease are also important.
- When a positive antinuclear antibody (ANA) test is accompanied by several other clues that doctors look for in diagnosing lupus, it is often a strong indication to consider lupus.
- It’s common for positive lab tests to come and go over time. If this happens, it’s less likely that you will receive a lupus diagnosis, though still possible.
- It’s very common to get somewhat different results at different labs.
- If your doctor rules out lupus, but you continue to have signs and symptoms, talk to your doctor or seek additional medical help. Whether or not it’s lupus, it’s important to address your symptoms.
Common tests used to diagnose lupus
Routine blood tests
Usually, your doctor will first request a complete blood count (CBC). Your blood is made up of red blood cells (RBCs), white blood cells (WBCs), platelets, and serum. The complete blood count measures the levels of each. In cases of lupus, these blood tests may reveal low numbers.
- Red blood cells carry oxygen to all parts of the body.
- White blood cells (lymphocytes and others) help the immune system, protect the body against foreign invaders.
- Platelets form in the bone marrow. They go to the site of a wound to begin the blood-clotting process.
- Blood serum is the fluid portion of whole blood from which certain substances in the clotting of blood have been removed.
Antibody blood tests
The body uses antibodies to attack and neutralize foreign substances like bacteria and viruses. The antibodies your body makes against its own normal cells and tissues play a large role in lupus.
Many of these antibodies are found in a panel—a group of tests that are ordered at the same time. The test you will hear about most is called the antinuclear antibody test (the ANA test).
97% of people with lupus will test positive for ANA.
ANA connects or binds to the nucleus or command center of the cell. This process damages and can destroy the cells. The ANA test is not a specific test for lupus. However, it is sensitive and does detect these antibodies in 97 percent of people with the disease.
The ANA can be positive in people with other illnesses or positive in people with no illness. For this reason, simply having a positive ANA test does not necessarily mean you have lupus. Test results can also vary in the same person. When a positive ANA is accompanied by several other clues that doctors look for in diagnosing lupus, it is often a strong indication to consider lupus.
Doctors trying to diagnose lupus often look for a number of other antibodies as well.
Blood clotting time
The rate at which your blood begins to clot is important. If it clots too easily, a blood clot (thrombus) could break free and travel through the body. Blood clots can cause damage such as a stroke or miscarriage. If your blood does not clot quickly enough, you could be at risk for excessive bleeding if you are injured.
Other blood tests
Some blood tests measure levels of proteins that are not antibodies. The levels of these proteins can alert your doctor that there is inflammation somewhere in your body.
Lupus can attack the kidneys without any warning signs, so urine tests are very important.
The kidneys process your body’s waste materials. Testing a sample of urine (called a “spot urine” test) can reveal problems with the way your kidneys are functioning.
The most common urine tests look for cell casts (bits of cells that normally would be removed when your blood is filtered through your kidneys). They also look for protein being spilled into your body because your kidneys are not filtering the waste properly (proteinuria). A collection of your urine over a 24-hour period can also give important information.
A biopsy procedure involves the removal of a small bit of tissue that the doctor then examines under a microscope. Almost any tissue can be biopsied.
The skin and kidney are the most common sites biopsies in someone who may have lupus.
The results of the biopsy can show the amount of inflammation and any damage being done to the tissue. Further tests on the tissue sample can detect ANA and determine whether lupus or another factor such as infection or medication is responsible.
What are the Types of Lupus?
When people talk about lupus, they’re usually talking about systemic lupus. But there are four kinds of lupus:
- Systemic lupus erythematosus (SLE), the most common form of lupus
- Cutaneous lupus, a form of lupus that is limited to the skin
- Drug-induced lupus, a lupus-like disease caused by certain prescription drugs
- Neonatal lupus, a rare condition that affects infants of women who have lupus
Systemic lupus erythematosus (SLE) is the most common form of lupus — 70 % of people with lupus have it. It’s what most people mean when they refer to “lupus”.
How is SLE different from other forms of lupus?
SLE can cause inflammation of multiple organs or organ systems in the body, either acutely or chronically. In contrast, cutaneous lupus (CLE), is limited to the skin, although in some patients, it may eventually progress to SLE. Drug-induced lupus can be caused by certain prescription medications. It has many of the same symptoms as SLE, but rarely affects major organs and disappears about six months after the medication is stopped. Neonatal lupus occurs only in newborns and is not true lupus. Most of the symptoms of neonatal lupus will disappear after six months.
What causes SLE?
Experts don’t know what causes systemic lupus erythematosus, but lupus and other autoimmune diseases do run in families. Women ages 15 to 44 and certain ethnic groups—including African American, Asian American, Hispanics/Latino, and Native American—are at higher risk for developing SLE than the rest of the population. Read more lupus facts and statistics and learn what the possible causes of the disease are.
What are the symptoms of SLE?
Symptoms of systemic lupus erythematosus (SLE) vary from person to person and they may come and go and change over time. Lupus shares symptoms with other diseases, which can make it difficult to diagnose. The most common symptoms include:
- Skin rashes
- Pain or swelling in the joints (arthritis)
- Swelling in the feet, and around the eyes (typically due to kidney involvement)
- Extreme fatigue
- Low fevers
Below is a brief description of some of the more serious complications of systemic lupus erythematosus involving major organ systems.
- Inflammation of the kidneys—called lupus nephritis—can affect the body’s ability to filter waste from the blood. It can be so damaging that dialysis or a kidney transplant may be needed.
- Inflammation of the nervous system and the brain can cause memory problems, confusion, headaches, and strokes.
- Inflammation in the brain’s blood vessels can cause high fevers, seizures, and behavioral changes.
- Hardening of the arteries or coronary artery disease—the buildup of deposits on coronary artery walls—can lead to a heart attack.
- Inflammation of the skin can cause rashes, sores, and ulcers throughout the body. About half of all people with systemic lupus erythematosus will develop a malar rash — a butterfly-shaped rash mostly seen across the cheeks and nose that can get worse in the sunlight.
What is Cutaneous Lupus?
Approximately two-thirds of people with lupus will develop some type of skin disease, called cutaneous lupus erythematosus. Skin disease in lupus can cause rashes or sores (lesions), most of which will appear on sun-exposed areas such as the face, ears, neck, arms, and legs. 40-70 percent of people with lupus will find that their disease is made worse by exposure to ultraviolet (UV) rays from sunlight or artificial light.
A dermatologist (a physician who specializes in caring for the skin) should treat lupus skin rashes and lesions. He or she will usually examine the tissue under a microscope to determine whether a lesion or rash is due to cutaneous lupus. Taking the tissue sample is called a biopsy.
There are 3 forms of lupus skin disease
Chronic cutaneous (discoid) lupus
Discoid lupus appears as disk-shaped, round lesions. The sores usually appear on the scalp and face, but sometimes they will occur on other parts of the body as well.
Approximately 10 percent of people with discoid lupus later develop lupus in other organ systems, but these people probably already had systemic lupus with the skin rash as the first symptom.
Discoid lupus lesions are often red, scaly, and thick. Usually, they do not hurt or itch. Over time, these lesions can produce scarring and skin discoloration (darkly colored and/or lightly colored areas). Discoid lesions that occur on the scalp may cause the hair to fall out. If the lesions form scars when they heal, the hair loss may be permanent.
Cancer can develop in discoid lesions that have existed for a long time. It’s important to speak with your doctor about any changes in the appearance of these lesions.
Discoid lupus lesions can be very photosensitive, so preventive measures are important:
- Avoid being out in the sunlight between the hours of 10 a.m. and 4 p.m.
- Use plenty of sunscreen when you are outdoors
- Wear sun-protective clothing and broad-brimmed hats
- Limit the amount of time spent under indoor fluorescent lights
Subacute cutaneous lupus
Subacute cutaneous lesions may appear as areas of red, scaly skin with distinct edges or as red, ring-shaped lesions. The lesions occur most commonly on the sun-exposed areas of the arms, shoulders, neck, and body. The lesions usually do not itch or scar, but they can become discolored. Subacute cutaneous lesions are also photosensitive, so preventive measures should be taken when spending time outdoors or under fluorescent lights.
Acute cutaneous lupus
Acute cutaneous lupus lesions occur when your systemic lupus is active. The most typical form of acute cutaneous lupus is a malar rash–flattened areas of red skin on the face that resemble a sunburn. When the rash appears on both cheeks and across the bridge of the nose in the shape of a butterfly, it is known as the “butterfly rash.” However, the rash can also appear on the arms, legs, and body. These lesions tend to be very photosensitive. They typically do not produce scarring, although changes in skin color may occur.
What is drug-induced lupus?
Drug-induced lupus is a lupus-like disease caused by certain prescription drugs.
The drugs most commonly connected with drug-induced lupus are:
- Hydralazine (used to treat high blood pressure or hypertension)
- Procainamide (used to treat irregular heart rhythms)
- Isoniazid (used to treat tuberculosis)
Drug-induced lupus is more common in men because they are given these drugs more often; however, not everyone who takes these drugs will develop the disease.
The symptoms of drug-induced lupus
Specific criteria for diagnosing drug-induced lupus have not been formally established. However, symptoms often overlap with those of systemic lupus erythematosus (SLE). These include:
- muscle and joint pain sometimes with swelling
- flu-like symptoms of fatigue and fever
- serositis (inflammation around the lungs or heart that causes pain or discomfort)
- certain laboratory test abnormalities
While the symptoms of drug-induced lupus are similar to those of systemic lupus, only rarely will any major organs be affected.
How quickly does drug-induced lupus develop?
It usually takes several months or even years of continuous therapy with the medication before symptoms appear. For people treated for one to two years as currently used doses of the high-risk drugs, approximately 5% of those taking hydralazine and 20% of those taking procainamide will develop drug-induced lupus. With most of the other drugs, the risk is less than 1% and usually less than 0.1% that those taking the medication will develop drug-induced lupus.
How long will drug-induced lupus continue?
The lupus-like symptoms usually disappear within six months after these medications are stopped.
What is neonatal lupus?
Neonatal lupus is not true lupus. It is a rare condition associated with anti-SSA/Ro and/or anti-SSB/La antibodies from the mother that affect the fetus. At birth, the baby may have a skin rash, liver problems, or low blood cell counts, but these symptoms typically disappear completely after six months with no lasting effects.
The most serious symptom is congenital heart block, which causes a slow heartbeat. Although very rare, newborns of women with lupus are at greater risk for developing this potentially life-threatening complication. Congenital heart block is usually detected when the fetus is between 18 and 24 weeks old. The condition does not disappear, and affected infants will eventually need a pacemaker.
With proper testing, physicians can now identify most at-risk mothers, and the infant can be treated at or before birth. Most infants of mothers with lupus are entirely healthy.