What is lupus?

Lupus is a long-term autoimmune condition that hyperactivates the body’s immune system and destroys normal, healthy tissue. Symptoms involve swelling, inflammation and damage to joints, skin, kidneys, blood, heart, and lungs.

Due of its complex nature, people often call lupus the “1,000-face disease.”

In the United States, people record approximately 16,000 new cases of lupus per year, and up to 1,5 million people, according to the Lupus Foundation of America, may be living with this illness.

The Foundation claims lupus especially affects women, and is most likely to occur between the ages of 15 and 44.

Lupus gained public attention in 2015 following the announcement by the singer Selena Gomez that she was diagnosed in her late teens and sought treatment for the disease.

Lupus is not contagious. A individual can not have it transferred to another individual sexually or in any other way.

Nevertheless, women with lupus can, in rare cases, give birth to children who develop a type of lupus. This is called lupus neonatal.

Types

There are various types of lupus. This article will concentrate specifically on systemic lupus erythematosus (SLE), include discoid, drug-induced, and neonatal lupus are another forms.

Systemic lupus erythematosus

Malar rash is a major symptom of lupus. Image credit: Doktorinternet, 2013.
Malar rash is a major symptom of lupus. Image credit: Doktorinternet, 2013.

SLE is the most well recognized form of lupus. It is a state of systemicity. This means it is having an effect all over the body. Symptoms may be mild to severe.

It is more extreme than other forms of lupus, such as discoid lupus, as it can affect any organ or organ system in the body. This may cause skin, joints, lungs, kidneys, blood, pulse, or a combination of these inflammations.

This state is usually cyclical. The person should have no symptoms in moments of remission. The illness becomes active during a flare-up, and symptoms appear.

Discoid lupus erythematosus

Symptoms affect only the skin in discoid lupus erythematosus (DLE) — or cutaneous lupus —; There’s a rash on your nose, neck and scalp.

The areas raised can become thick and scaly and can result in scarring. The rash can last from several days to several years, and can recur.

DLE will not affect the internal organs but, according to the Lupus Foundation of America, about 10 percent of people with DLE will tend to develop SLE. However, it is not clear if these individuals already had SLE and displayed only clinical symptoms on the skin, or if there is improvement from DLE or SLE.

Subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus refers to skin lesions that occur on sun-exposed areas of the body. None of the lesions cause scarring.

Drug-induced lupus

Symptoms arise in around 10 percent of people with SLE due to a reaction to some prescription drugs. Some 80 medications can cause the condition according to the Genetics Home Reference.

They are some of the drugs people are using to control epilepsy and high blood pressure. Some thyroid medications, antibiotics, antifungals, and oral contraceptive pills also come in.

Drugs that are commonly associated with this form of lupus are:

  • Hydralazine, a hypertension medication
  • Procainamide, a heart arrhythmia medication
  • Isoniazid, an antibiotic used to treat tuberculosis (TB)

Drug-induced lupus typically goes away after the person stops taking the medication.

Neonatal lupus

The majority of babies born with SLE are healthy. Yet only 1% of women with lupus-related autoantibodies may have a baby with neonatal lupus.

The woman may have SLE, a condition of Sjögren, or no signs of disease at all.

Sjögren’s syndrome is another autoimmune disease often associated in lupus. Main signs include a dry mouth and dry eyes.

Babies with neonatal lupus at birth can experience skin rash, liver problems and low blood counts. About 10 percent would have anemia.

The lesions usually go away after a few weeks. However, some infants have a congenital heart block, in which the heart cannot regulate a normal and rhythmic pumping action. The infant may need a pacemaker. This can be a life-threatening condition.

To women with SLE or other associated autoimmune disorders it is essential to be under the care of a doctor during pregnancy.

Causes

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

What goes wrong?

The immune system defends the body and kill antigens including viruses, bacteria, and germs.

This does so by producing proteins known as antibodies. These antibodies are formed by white blood cells, or lymphocytesB.

The immune system can not distinguish between harmful substances, or antigens, and healthy tissue when a person has an autoimmune disorder such as lupus.

As a consequence, anticorps are guided toward both healthy tissue and antigens by the immune system. This causes damage to swelling, pain and tissue.

An antinuclear antibody (ANA) is the most common form of autoantibody that develops in humans with lupus. The ANA interacts with parts of the nucleus of the cell, the cell’s command center.

Such autoantibodies circulate in the blood but some of the cells of the body have walls that are sufficiently permeable to let certain autoantibodies pass through.

The autoantibodies will then target the DNA in those cells’ nucleus. Therefore lupus affects certain organs more than others.

Why does the immune system go wrong?

The development of SLE is possibly affected by various genetic factors.

Within the body certain genes help the immune system function. Changes in certain genes may stop the immune system from functioning properly in people with SLE.

According to Genetics Home Guide, one potential explanation relates to cell death, a normal cycle that happens when the body renews its cells.

Some scientists claim that the body is not getting rid of cells which have died due to genetic factors.

These remaining dead cells that release substances which cause malfunction in the immune system.

Risk factors: Hormones, genes, and environment

A variety of factors may cause lupus to grow in response. They may be hormonal, genetic, environmental, or a combination of them.

1) Hormones

Hormones are chemical substances which are produced by the body. We monitor and regulate the operation of different cells or organs.

The following risk factors may be explained by hormonal activity:

Sex: The U.S. National Institutes of Health states that females are 9 times more likely to develop lupus than males.

Age: Signs and diagnosis frequently arise during childbearing years, between the ages of 15 and 45. Nonetheless, according to Genetics Home Reference, 20 percent of cases occur after the age of 50 years.

As 9 out of 10 lupus cases affect females, researchers have looked at a possible connection between lupus and estrogen. All men and women are consuming estrogen but women are producing more.

In a study published in 2016, scientists observed that oestrogen in mice susceptible to lupus can affect immune function and induce lupus antibodies.

This can explain why women are more likely to get affected by autoimmune diseases than men.

In 2010 researchers who published a study in the journal Rheumatology on self-reported flares found that women with lupus experience more intense menstrual pain and fatigue. This indicates flares at this time might be more probable.

There’s no data to prove that lupus is caused by estrogen. If there is a correlation, lupus severity may be controlled by estrogen-based treatment. Nonetheless, it needs more work before physicians can recommend it as a therapy.

2) Genetic factors

Studies have not shown that lupus is caused by any particular genetic factor although it is more severe in some families.

Genetic factors may be the reason why the following are risk factors for lupus:

Race: people of any ethnicity that develop lupus, but in people of color it is two to three times more common compared to white people. This is also more common in women from Hispanic, Asian and Native American countries.

History of the family: A person who has a first or second degree relative with lupus will have a greater chance of developing it.

Scientists have established some genes that could lead to lupus development but there is insufficient evidence to prove they cause the disease.

In identical twin studies, one twin may develop lupus while the other does not, even though they grow together and have the same exposures to the environment.

According to a report published in Seminars on Arthritis and Rheumatism in 2017, if one member of a twin pair has lupus, the other has a 25 percent risk of developing the disorder. Identical twins have a greater risk of each developing the disease.

Lupus may occur in people who have no family history of the disease, but other autoimmune disorders may occur within the family. Examples of this include thyroiditis, hemolytic anemia and purpura thrombocytopenia idiopathic.

Some have suggested changes to the x-chromosomes may affect the risk.

3) Environment

Environmental agents — such as chemicals or viruses — can help to cause lupus in people who are genetically susceptible already.

Possible environmental causes include:

Smoking: an increase in the number of cases may be attributed to increased exposure to cigarettes in recent decades.

Sunlight exposure: Many say this may be a cause.

Medication: Around 10 percent of cases may be drug-related, according to Genetics Home Reference

Viral infections: These may trigger symptoms in people who are prone to SLE.

Lupus is not infectious, and can not be sexually transmitted by a human.

Gut microbiota

Scientists have recently looked into gut microbiota as a potential factor in lupus growth.

Scientists who published research in Applied and Environmental Microbiology in 2018 noted that in both people and mice with lupus, unique changes in the gut microbiota function.

They are demanding more reserch in this field.

Are children at risk?

Lupus is rare in children under the age of 15 unless the mother of their birth has it. In this case, a child may have problems with the heart, liver, or skin associated with lupus.

Children with neonatal lupus may have a greater risk of developing another autoimmune disorder later in life.

Symptoms

The lupus signs arise at flare-up periods. Individuals normally experience recovery periods between flare-ups, where there are little to no symptoms.

Lupus has a wide range of symptoms, including:

  • fatigue
  • a loss of appetite and weight loss
  • pain or swelling in joints and muscles
  • swelling in the legs or around the eyes
  • swollen glands, or lymph nodes
  • skin rashes, due to bleeding under the skin
  • mouth ulcers
  • sensitivity to the sun
  • fever
  • headaches
  • chest pain upon deep breathing
  • unusual hair loss
  • pale or purple fingers or toes from cold or stress (Raynaud’s phenomenon)
  • arthritis
Lupus affects people in different ways. Symptoms may be found in many areas of the body.
Lupus affects people in different ways. Symptoms can occur in many parts of the body.

Effect on other body systems

Lupus can also affect the following systems:

Kidneys: Kidney inflammation (nephritis) will make it harder for the body to efficiently absorb waste products and other toxins. Around 1 in 3 people suffering from lupus will have kidney problems.

Lungs: Some people develop pleuritis, an inflammation of the chest cavity lining that causes pain in the chest, particularly at breathing. May develop pneumonia.

Central nervous system: Occasionally, lupus may affect the brain or the central nervous system. Symptoms include headaches, dizziness, fatigue, memory loss, problems with vision, seizures, strokes or behavioral changes.

Blood vessels: Vasculitis, or blood vessel inflammation, can occur. This can have an effect on circulation.

Blood: Lupus can cause anemia, leukopenia (a decrease in the number of white blood cells) or thrombocytopenia (a decrease in the number of platelets in the blood that help to clot).

Heart: When the heart is compromised by inflammation it can lead to myocarditis and endocarditis. It can also affect the membrane surrounding the heart which causes pericarditis. It can result in chest pain or other symptoms. Endocarditis can damage heart valves and cause thickening and development of the valve surface. This can lead to growths which can trigger heart murmurings.

Other complications

Having lupus increases the risk of a number of health problems.:

Infection: Infection gets more likely as the immune system is compromised by both lupus and its treatments. Popular infections include urinary tract infection, respiratory infections, yeast infection, salmonella, herpes, and shingles.

Bone tissue death: This happens when blood flow to a bone is small. Minor breaks in the bone may grow. The bone could ultimately collapse. The hip joint is most often affected by this.

Pregnancy complications: People with lupus are at elevated risk of loss of pregnancy, premature delivery and preeclampsia, a disorder that requires high blood pressure. Doctors also suggest stopping the pregnancy until the lupus has been under control for at least 6 months to reduce the likelihood of such complications.

video


The following video explains how lupus causes symptoms.

Classification: 11 symptoms

When validate a diagnosis, the American College of Rheumatology makes use of a common classification system.

When a person meets 4 out of 11 requirements, a doctor may find the likelihood of having lupus.

The 11 parameters are those of:

  1. Malar rash: A butterfly-shaped rash appears across the cheeks and nose.
  2. Discoid rash: Raised red patches develop.
  3. Photosensitivity: A skin rash appears after exposure to sunlight.
  4. Oral or nose ulcers: These are usually painless.
  5. Non-erosive arthritis: This does not destroy the bones around the joints, but there is tenderness, swelling, or effusion in 2 or more peripheral joints.
  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: The person has seizures, psychosis, or problems with thinking and reasoning.
  9. Hematologic (blood) disorder: Hemolytic anemia is present, with a low white blood-cell count or low platelet count.
  10. Immunologic disorder: Tests show that there are antibodies to double-stranded DNA (dsDNA), antibodies to Sm, or antibodies to cardiolipin.
  11. Positive ANA: The test for ANA is positive, and the person has not used any drugs that might induce it.

Even this system, however, sometimes fails early and mild cases.

Underdiagnosis can occur because lupus signs and symptoms are not similar to each other.

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

Diagnosis

A blood test can help to diagnose lupus.
A blood test can help to diagnose lupus.

Diagnosis can be complicated due to the varied symptoms which can resemble symptoms from other diseases.

The doctor will inquire about the symptoms, conduct a physical examination and take a medical history of both personal and family. We will also consider the above 11 conditions.

The doctor can order blood tests, as well as other laboratory research.

Biomarkers

Biomarkers are antibodies, proteins, genetics, and other factors that may show a doctor what’s going on in the body, or how the body responds to treatment.

Symptoms are useful because they can show that a person has a disease even without symptoms.

Lupus affects people in different ways. This makes identifying accurate biomarkers difficult.

A combination of blood tests and other studies will also help a doctor confirm a diagnosis.

Blood tests

Blood tests can indicate whether certain biomarkers are present, and biomarkers can provide details on which, if any, a person has autoimmune disease.

1) Antinuclear antibody

In the ANA study, nearly 95 percent of people with lupus would have a positive result. Many people test positive for ANA, however, but don’t have lupus. Other tests must confirm the diagnosis.

2) Antiphospholipid antibodies

Antiphospholipid antibodies (APLs) are a type of phospholipid-directed antibody. In up to 50 percent of people with lupus, APLs are present. Individuals without a lupus can have APLs, too.

A person with APLs may have an increased risk of blood clots, stroke, and lung hypertension. There is also an increased risk of complications of pregnancy including a loss of pregnancy.

3) Anti-DNA antibody test

About 70 percent of people with lupus have an antibody called the anti-DNA antibody. In a flare-up the result is more likely to be optimistic.

4) Anti-dsDNA antibody

The anti-double-stranded DNA antibody (anti-dsDNA) is a particular form of ANA antibody that occurs in around 30 percent of lupus people. Fewer than 1 per cent of lupus-free people have this antibody.

If the test is positive, a person may have a more severe form of lupus, such as lupus nephritis, or lupus in the kidneys.

5) Anti-Smith antibody

Around 20 percent of people with lupus have an antibody to Sm, a ribonucleoprotein that is present in the nucleus of a cell.

It is present in less than 1 per cent of lupus-free people and is rare in those with other rheumatic diseases. A individual with anti-sm antibodies is likely to get lupus for this purpose. It is typically not present with lupus in the kidneys.

6) Anti-U1RNP antibody

Around 25 percent of people with lupus have anti-U1RNP antibodies, and fewer than 1 percent of people without lupus have them.

This antibody may be present in people with the syndrome of Raynaud, and Jaccoud’s arthropathy, a hand deformity due to arthritis.

7) Anti-Ro/SSA and anti-La/SSB antibodies

For people with lupus, between 30 and 40 percent have anti-Ro / SSA and anti-La / SSB antibodies. They also occur with Sjögren’s primary syndrome and in people with lupus who test ANA negative.

In around 15 per cent of people without lupus, they are present in small quantities and can occur with certain rheumatic disorders, such as rheumatoid arthritis.

If a mother has anti-Ro and anti-La antibodies, the risk of a baby born to her developing neonatal lupus is greater.

A person with lupus who wants to become pregnant has these antibodies undergoing testing.

8) Anti-histone antibodies

Antibodies to histones are proteins that play a role in the structure of DNA. People with drug-induced lupus usually have them, and people with SLE may have them. However, they do not necessarily confirm a diagnosis of lupus.

Serum (blood) complement test

Histone antibodies are proteins which play a role in DNA structure. Those with drug-induced lupus generally have them, and can have them for those with SLE. They may not automatically however validate a lupus diagnosis.

Serum (blood) complement test

serum complement test measures the levels of proteins that the body consumes when inflammation takes place.

If a person has low complement levels, this suggests that inflammation is present in the body and that SLE is active.

Urine tests

Urine tests can help to 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

The doctor may also require biopsies to test for any injury or inflammation, usually of the skin or kidneys.

Imaging tests

X-rays and other imaging tests can help doctors see the organs affected by lupus.

Monitoring tests

Ongoing tests can show how lupus continues to affect a person or how well their body is responding to treatment.

Treatment and home remedies

There is currently no cure for lupus, but people can manage their symptoms and flares with lifestyle changes and medication.

Treatment aims to:

  • prevent or manage flares
  • reduce the risk of organ damage

Medication can help to:

  • reduce pain and swelling
  • regulate the activity of the immune system
  • balance hormones
  • reduce or prevent joint and organ damage
  • manage blood pressure
  • reduce the risk of infection
  • control cholesterol

The precise diagnosis will depend on how personally the lupus affects. Flares may occur without care, which could have life-threatening consequences.

Alternative and home therapies

Exercise can help to reduce pain and relieve stress.
Exercise can help to reduce pain and relieve stress.

Apart from medication, the following may help to relieve pain or reduce the risk of a flare:

  • applying heat and cold
  • participating in relaxation or meditation activities, including yoga and tai chi
  • doing regular exercise when possible
  • avoiding exposure to the sun
  • avoid stress, as far as possible

Many people use the thunder-god-vine replacement. The National Center for Complementary and Integrative Health (NCCIH), however, warns that this may be dangerous. Until using it, it is crucial that you speak to a doctor.

Outlook In the past, people who had a lupus diagnosis would usually not survive for longer than five years.

Nevertheless, care now, according to the National Institutes of Health, will dramatically improve a person’s life span.

Effective therapy also allows lupus treatment possible, so that an full, healthy life can be lived by a person.

As scientists know more about genetics, physicians hope to be able to recognize lupus at an earlier stage one day. It would promote avoidance of accidents before they occur.

Often people want to enter a clinical trial, because this can include access to new medicines. For more information on clinical trials please click here.

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