Lupus is a long-term autoimmune disease in which the body’s immune system becomes hyperactive and attacks healthy tissue. Due to its complex nature, lupus is sometimes known as the “disease of a thousand faces.”

According to the Lupus Foundation of America, people report around 16,000 new cases of lupus in the United States each year, and up to 1.5 million people may be living with the condition in the U.S.

There are several different kinds of lupus, including systemic lupus erythematosus (SLE), cutaneous lupus (such as discoid lupus erythematosus [DLE]), drug-induced lupus (DIL), and neonatal lupus.

A malar rash is a symptom of lupus. Image credit: Doktorinternet, 2013.Share on Pinterest
A malar rash is a key symptom of lupus. Image credit: Doktorinternet, 2013.

Systemic lupus erythematosus

SLE is the most common form of lupus, comprising 70% of lupus cases. It is a systemic condition. This means that it can affect multiple organs and systems throughout the body. For this reason, SLE tends to be a more severe form of lupus. The symptoms can range from mild to severe.

SLE can cause inflammation in the:

  • skin
  • joints
  • lungs
  • kidneys
  • blood
  • heart

This inflammation may happen in one of these areas or affect multiple areas at one time.

This condition typically goes through cycles of flare-ups and remissions. During times of remission, a person with lupus may have no symptoms. During a flare-up, the disease is active, and a person’s symptoms return, or new symptoms may appear.

Some people with SLE may have regular flare-ups. Others may only experience them every few years. Still others may experience long-term activity.

Discoid lupus erythematosus

In DLE, a type of cutaneous lupus, symptoms affect only the skin. DLE appears as circular lesions, typically on the scalp and face, although they can appear on other parts of the body, such as inside the ears.

The lesions tend to be red and may become thick and scaly. In some cases, the lesions lead to scarring and skin discoloration. If lesions scar on the scalp, hair may not regrow in that area.

DLE does not affect the internal organs, but around 10% of people with DLE go on to develop SLE, according to the Lupus Foundation of America. However, it is possible that these people already had SLE and their skin symptoms led to a DLE diagnosis first.

Subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body that have exposure to the sun. These lesions do not cause scarring but may cause skin discoloration.

Drug-induced lupus

DIL is an autoimmune condition that is caused by certain medications. The symptoms tend to be similar to those in SLE, but they are often less severe. More than 100 medications have been identified as potential causes of DIL.

Medications that are commonly associated with DIL include:

Symptoms of DIL tend to appear after months or years of continuous treatment with these medications. Approximately 5% of people taking hydralazine and 20% of those taking procainamide long term go on to develop DIL. Although other medications can induce DIL, the likelihood is far lower.

DIL typically goes away within 6 months of stopping the medication.

Neonatal lupus

Neonatal lupus is a condition that occurs in infants when their birth parent passes on certain antibodies through the placenta during pregnancy.

The antibodies that can cause neonatal lupus are ones associated with lupus and Sjögren’s disease. The birth parent may have either of these conditions or no symptoms at all but can still pass on these antibodies. When this occurs, the probability of the infant developing neonatal lupus is estimated to be 2%.

At birth, babies with neonatal lupus may have a skin rash, liver problems, and low blood counts.

The skin symptoms of neonatal lupus usually go away after a few weeks. However, some infants may have a congenital heart block, in which the heart cannot regulate a normal and rhythmic pumping action. This is a more serious complication, and the infant may need a pacemaker.

Most infants born to birth parents with SLE are completely healthy. However, a person with SLE may wish to speak with a doctor about how their condition may affect their pregnancy.

Lupus is an autoimmune condition, but the exact cause is unclear.

What goes wrong?

The immune system is a complex network made up of organs, tissues, and cells. It protects the body by fighting off foreign bodies such as viruses, bacteria, fungi, and toxins. It also clears away dead or faulty cells.

The immune system protects the body by producing Y-shaped proteins called antibodies. These antibodies either neutralize a threat or send a signal to other cells to remove it. Specialized white blood cells, called B lymphocytes, produce these antibodies.

When a person has an autoimmune condition, such as lupus, the immune system cannot differentiate between unwanted substances, or antigens, and healthy tissue. The body mistakes itself as foreign in error.

As a result, the immune system directs antibodies against both the healthy tissue and the antigens. This causes swelling, pain, and tissue damage.

Antinuclear antibodies (ANAs) are commonly present in people with autoimmune conditions such as lupus. They work by targeting the nucleus of the body’s own cells. The nucleus is the part of the cell that contains genetic material.

Why does the immune system go wrong?

Researchers do not yet entirely understand what causes lupus, but they believe it is caused by multiple factors.

One possible theory relates to cell death, a natural process that occurs as the body renews its cells. Research suggests that due to genetic factors, the bodies of people with lupus may not properly clear cells that have died.

The dead cells that remain may lead to the production of autoantibodies, such as ANAs, that go on to attack the body, causing lupus symptoms.

Lupus may develop in response to several factors. These may be hormonal, genetic, environmental, or a combination of these factors.

Hormones

Hormones are chemical substances that the body produces. They control and regulate the activity of certain cells and organs.

Hormonal activity may offer an explanation for risk factors such as sex and age.

The Centers for Disease Control and Prevention (CDC) state that females between the ages of 15 and 44 years are nine times more likely to have lupus than males.

Symptoms and diagnosis often occur between the ages of 15 and 45 years, during reproductive ages. However, 20% of cases appear after the age of 50 years.

Some research suggests that exposure to estrogen, a sex hormone that females tend to produce more of than males, can increase the risk of autoimmune conditions such as lupus.

This is further supported by many females experiencing more lupus symptoms before menstruation and during pregnancy, when estrogen levels are higher.

Although further research is required, the presence of certain sex hormones could explain why lupus is so prevalent in adult females.

Genetic factors

Although further research is needed, scientists have identified certain genes that play a role in the immune system response that may contribute to the development of lupus. Evidence suggests that there is a higher chance of a person developing lupus if a family member has it, further supporting the possibility that genetics may be a risk factor.

Twin studies also show that if a member of identical twins has lupus, the other has a 24% chance of also developing the disease.

Lupus can happen in people with no family history of the disease, but there may be other autoimmune conditions in the family.

People of any background can develop lupus, but it is two to three times more common in African American, Hispanic/Latina, Asian American, Native American, Alaska Native, Native Hawaiian, and other Pacific Islander females compared with white females.

Research suggests that for Women of Color, symptoms tend to onset earlier and be more severe. This research stresses the need for increased medical support, early diagnosis, and treatment.

Lupus risk factors include a combination of genetic and environmental factors. Lupus being more common in certain demographics may partially be due to genetics. However, differences in certain outcomes, such as disease progression and mortality, may be due to social factors such as inequalities in healthcare.

Environment

Environmental agents, such as chemicals or viruses, may contribute to triggering lupus in people who are already genetically susceptible.

Possible environmental triggers include:

  • Smoking: Exposure to toxic components of cigarette smoke may lead to genetic mutations and gene activations linked to SLE.
  • Exposure to sunlight: The radiation that comes from direct sunlight can worsen symptoms of preexisting SLE. However, further research is needed to examine whether it can be a risk factor for developing SLE. UV radiation can lead to cell damage and genetic mutations that may be involved in the development of SLE.
  • Infections: Infections such as the Epstein-Barr virus, also known as glandular fever, are much more common in people with SLE. This suggests that it may play a role in the development of the condition for those who are susceptible.
  • Pollutants: In a similar way to cigarette smoke, air pollution may be a risk factor for developing SLE.

Are children at risk?

Lupus can occur in people of any age. According to the American College of Rheumatology, 20% of people with lupus develop the condition before the age of 20. It is rare for people to develop lupus before the age of 5.

Lupus that develops during childhood tends to have more severe symptoms and frequently affects the kidneys, with up to 50% of children having kidney symptoms.

The symptoms of lupus occur in times of flare-ups. Between flare-ups, people usually experience times of remission, when there are few or no symptoms.

Lupus has a wide range of symptoms, including:

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Lupus affects people in different ways. Symptoms can occur in many parts of the body.

Signs of lupus in females

Lupus can present differently from person to person. Evidence suggests that lupus may present differently in males and females. Females tend to have less severe symptoms than males.

Symptoms that are seen more commonly in females include:

  • hair loss
  • sensitivity to sunlight
  • mouth ulcers
  • arthritis
  • malar rash

Signs of lupus in males

There is a common misconception that only females can have lupus. Although less common, males can also have lupus. Research suggests that males with lupus tend to have more severe symptoms.

Symptoms that occur more commonly in males include:

  • cardiovascular complications
  • low blood count
  • weight loss
  • kidney complications
  • chest pain

Effect on other body systems

Lupus can also affect the following systems:

  • Kidneys: Lupus can cause a kidney disease called lupus nephritis, where inflammation stops the kidneys from working properly. Symptoms can include swelling of the legs, feet, and face, frequent urination, and high blood pressure.
  • Lungs: Some people may experience inflammation of the lining of the lungs, known as pleurisy, that causes chest pain when breathing deeply. Some people may also develop pneumonia.
  • Central nervous system: Lupus can sometimes affect the brain or central nervous system. Symptoms may include brain fog (confusion and trouble concentrating), headaches, seizures, and strokes. Lupus cerebritis may also occur, causing an acute confusional state, cognitive dysfunction, mood changes, seizures, lethargy, and comas.
  • Blood vessels: Vasculitis, or inflammation of the blood vessels, can occur. This can affect circulation.
  • Blood: Lupus can cause anemia, leukopenia (a decreased number of white blood cells), or thrombocytopenia (a decrease in the number of platelets, which assist in clotting, in the blood).
  • Heart: If inflammation affects the heart, it can result in myocarditis and endocarditis. It can also affect the membrane that surrounds the heart, causing pericarditis. This may cause symptoms including chest pain. Endocarditis can damage the heart valves, causing the valve surface to thicken and develop holes. This can result in growths that can lead to heart murmurs.

Other complications

There are other complications that can arise from lupus.

Infection

Infection becomes more likely when a person has lupus because both the disease and its treatments weaken the immune system. Common infections include:

Bone tissue death

This occurs when there is low blood supply to a bone, resulting in small breaks developing. Eventually, this can lead to the bone collapsing. People with lupus may be at increased risk of this due to the condition itself and due to medications such as corticosteroids, which are used to treat lupus.

Pregnancy complications

People with lupus have a higher risk of pregnancy loss, preterm birth, and preeclampsia, a dangerous condition that includes high blood pressure. To reduce the risk of these complications, doctors often recommend delaying pregnancy until lupus has been under control for at least 6 months.

The following video explains how lupus causes symptoms.

The American College of Rheumatology uses a standard classification scheme to confirm a diagnosis. If a person meets 4 out of the 11 criteria, a doctor will consider a lupus diagnosis.

The 11 criteria are:

  1. Malar rash: A butterfly shaped rash appears across the cheeks and nose.
  2. Discoid rash: Raised red patches develop on the skin.
  3. Photosensitivity: A skin rash appears after exposure to sunlight.
  4. Oral or nose ulcers: Ulcers appear in a person’s mouth or nose.
  5. Nonerosive arthritis: This type of arthritis does not destroy the bones around the joints but does cause tenderness and swelling.
  6. Pericarditis or pleuritis: Inflammation affects the lining around the heart (pericarditis) or lungs (pleuritis).
  7. Kidney disorder: Tests show high levels of protein or cellular casts in the urine if a person has a kidney problem.
  8. Neurologic disorder: A person may experience seizures, psychosis, or problems with thinking and reasoning.
  9. Hematologic (blood) disorder: Blood may show a low red blood cell count (anemia), a low white blood cell count (leukopenia), or a low platelet count (thrombocytopenia).
  10. Immunologic disorder: Tests show that there are anti-double-stranded DNA antibodies, anti-Smith antibodies, or antiphospholipid antibodies (APLs).
  11. Positive ANA: A test detects high levels of ANA.

However, even this system sometimes misses early and mild cases of lupus. Underdiagnosis can occur because the signs and symptoms of lupus are not specific.

On the other hand, some blood tests can lead to overdiagnosis because people without lupus can have the same antibodies as those with the condition.

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A blood test can help to diagnose lupus.

Diagnosis can be difficult because of the varied symptoms that may resemble symptoms of other conditions.

A doctor will ask about symptoms, carry out a physical examination, and take a personal and family medical history. They will also consider the 11 criteria mentioned above.

The doctor may request some blood tests and other laboratory investigations.

Biomarkers

Biomarkers are antibodies, proteins, genetics, and other factors that can show a doctor what is happening in the body or how the body is responding to treatment. They are useful because they can indicate whether a person has a condition even when there are no symptoms.

Lupus affects individuals in different ways. This makes it difficult to find reliable biomarkers. However, a combination of blood tests and other investigations can help a doctor confirm a diagnosis.

Blood tests

Blood tests can show whether certain biomarkers are present, and biomarkers can give information about which autoimmune disease, if any, a person has.

  • Antinuclear antibody: Around 95% of people with lupus will have a positive result in the ANA test. However, some people test positive for ANA but do not have lupus. Other tests must confirm the diagnosis.
  • APLs: APLs are a type of antibody directed against phospholipids. APLs are present in up to 50% of people with lupus. People without lupus can also have APLs.
  • Anti-DNA antibody test: Around 30% of people with lupus test positive for these antibodies. The result is more likely to be positive during a flare-up. It is present in fewer than 1% of people without lupus, and it is rare in those with other rheumatic diseases. Therefore, it is a helpful test to confirm a lupus diagnosis.
  • Anti-Smith antibody: Around 20% of people with lupus have an antibody to Sm, a ribonucleoprotein that is present in the nucleus of a cell.
  • Anti-U1RNP antibody: Around 25–30% of people with lupus have anti-U1RNP antibodies, and fewer than 1% of people without lupus have them. This antibody may be present in other autoimmune conditions.
  • Anti-Ro/SSA and anti-La/SSB antibodies: Between 30–40% of people with lupus have anti-Ro/SSA and anti-La/SSB antibodies. These antibodies are not specific to lupus but can point to certain symptoms, such as extreme sun sensitivity.
  • Antihistone antibodies: Antibodies to histones are proteins that play a role in the structure of DNA. People with DIL usually have them, and people with SLE may have them. However, they do not necessarily confirm a diagnosis of lupus.
  • Serum (blood) complement test: This test measures the levels of proteins that the body consumes when inflammation takes place. If a person has low levels, this suggests there is inflammation in the body, and their condition may be active.
  • Nonspecific tests: Rheumatologists may choose to run tests that look for markers of inflammation, including C-reactive protein and erythrocyte sedimentation rate.

As well as blood tests, further tests may include:

  • Urine tests: Urine tests can help doctors diagnose and monitor the effects of lupus on the kidneys. The presence of protein, red blood cells, white blood cells, and cellular casts can all help to show how well the kidneys are working. For some tests, only one sample is necessary. For others, the person may need to collect samples over 24 hours.
  • Tissue biopsies: A doctor may also request biopsies, usually of the skin or kidneys, to check for any damage or inflammation.
  • Imaging tests: X-rays and other imaging tests can help doctors see the organs affected by lupus.

There are several treatments and home remedy options that may be helpful for the treatment or management of lupus.

Treatment

Treatment for lupus aims to reduce inflammation to protect organs from damage and prevent flare-ups. Each person is different, and their treatment plan may vary depending on the type of lupus, the extent of the inflammation, and the condition of their organs.

Common treatments for lupus include:

  • Hydroxychloroquine: This is an antimalarial drug that treats rashes and arthritis symptoms in lupus.
  • Corticosteroids and immune suppressants: These can include high-dose corticosteroids, such as prednisone, and medications that suppress the immune system, such as mycophenolate and methotrexate. This treatment aims to target a specific part of the immune system to control inflammation.
  • Belimumab: This is a biological agent approved by the Food and Drug Administration (FDA) to treat SLE.

A doctor may prescribe medications to treat complications that commonly arise in people with lupus, such as medications for seizures, antibiotics for infections, and vitamin D to help improve kidney function.

Home remedies

As inadequately treated lupus can lead to significant organ damage, doctors often recommend home remedies and lifestyle modifications in conjunction with medication.

One possible way a person may manage their lupus symptoms at home is through their diet. Although research is limited on how diet affects lupus, some evidence suggests it can play a role in disease management.

A person should aim to have a balanced and varied diet that contains fruit and vegetables, whole grains, and a moderate amount of protein. Lupus can vary greatly from one person to another, so a person with lupus should talk with a doctor about what diet may be best for them.

Some changes a person could make to their diet to help manage their condition include:

  • including omega-3 fatty acids
  • including less cholesterol and less saturated fats
  • including less sodium
  • including vitamins such as vitamin D and vitamin B

Further lifestyle changes a person can make to help manage their condition include:

  • Avoid smoking: Smoking can damage many organs in the body and lead to a worsening of lupus symptoms.
  • Consume less alcohol: Alcohol may lower the effectiveness of certain medications and affect liver health. A person should talk with a doctor about how alcohol may interact with their treatment and current health condition.
  • Get moderate exercise: Low impact exercises, such as walking, swimming, Pilates, and yoga, can allow a person to strengthen their body without the risk of aggravating joint inflammation. Moderate exercise may aid heart, lung, bone, and joint health and help a person’s mental health when they are dealing with the stresses of their condition.
  • Build a community: Research suggests that 43% of people with lupus are concerned about social isolation, and 30% feel that the emotional impact of their disease is misunderstood. A person with lupus can benefit from having an understanding support system around them. This may come from friends and family or from online communities.
  • Manage stress: Stress can trigger lupus flare-ups. Reducing stress and developing healthy life management skills can help reduce or prevent the incidence of flare-ups.

Lupus has a wide range of symptoms and may affect people in different ways. Some people may experience cycles of flare-ups and remissions, whereas others may have ongoing symptoms. The varied experiences of lupus can make it challenging for doctors to diagnose.

However, once a doctor has diagnosed the condition, there are several treatments that can manage a person’s symptoms, limit damage to their organs, and allow them to experience a greater quality of life.