What Is Lupus?

Lupus is an autoimmune disease in which the immune system attacks the body's own tissues, causing inflammation, pain, and potentially damage. While lupus can affect any part of the body, it most commonly attacks your:

  • Skin
  • Joints
  • Heart
  • Lungs
  • Blood cells
  • Kidneys
  • Brain

The disease can take several forms, with systemic lupus erythematosus (SLE) being the most common. Currently, there's no cure for lupus, but treatment can help control the autoimmune response and reduce symptoms.

Around 1.5 million Americans have some form of lupus, with an estimated 16,000 newly diagnosed each year.  Anyone at any age can acquire the disease, but most people with lupus are female and between the ages of 15 and 44.

Types of Lupus
Verywell / Emily Roberts

Types of Lupus

Lupus has four main types. Even if you have the same type of lupus as someone else, your symptoms won't necessarily be the same, as the disease is highly individualized.

Systemic Lupus Erythematosus (SLE)

The most common form of lupus is systemic lupus erythematosus (SLE), which can affect many parts of the body, including the:

  • Joints
  • Skin
  • Blood vessels
  • Organs

This type affects around 70% of people with the disease and is typically what people mean when they talk about "lupus." Symptoms may come and go all at once and can range from mild to severe.

People with SLE may go through years of red rashes, extreme fatigue, painful or swollen joints, or fevers without ever noticing a pattern to the flares or without a doctor being able to diagnose their disease.

Drug-Induced Lupus

Drug-induced lupus is associated with similar symptoms, but they're specifically brought on by certain types of drugs that are usually taken over long periods of time. This type accounts for around 10% of lupus cases.

Many drugs have been known to cause this form of the disease, but several are considered primary culprits. They are mainly anti-inflammatories, anticonvulsants, or drugs used to treat chronic conditions such as heart disease, thyroid disease, hypertension (high blood pressure), and neuropsychiatric disorders. 

The three drugs mostly to blame for drug-induced lupus are:

Drug-induced lupus is completely reversible once the offending drug is discontinued, and symptoms usually go away within six months.

Cutaneous Lupus

Some people have only skin manifestations of lupus and are diagnosed with cutaneous lupus—a separate type of lupus that, alone, accounts for around 10% of lupus cases.

That said, cutaneous lupus lesions also occur in two-thirds of people diagnosed with SLE.

As with other forms of lupus, it's a case of your body attacking its own skin. The exact cause of this form is unknown, although women are more likely to have it and it can run in families. Cigarette smoking and sunlight have been shown to exacerbate the condition.

Cutaneous lupus, generally speaking, comes in three types, including:

Chronic Cutaneous (Discoid Lupus) 

In discoid lupus, the most common form of chronic cutaneous lupus, inflammatory sores develop on your:

  • Face
  • Ears
  • Scalp
  • Other areas of the body

These lesions can be crusty or scaly and often scar. They usually don't hurt or itch. Some people report lesions and scarring on the scalp, making hair re-growth impossible in those areas.

Most people with discoid lupus do not have SLE. Unlike many other forms, discoid lupus is more common in men than in women. 

Subacute Cutaneous

The skin symptoms of subacute cutaneous lupus are usually mild. People with this condition present with reddish-purple plaques, which are firm, raised, but flattened skin lesions.

These plaques can be found alone or in groups and range in size from 5 to 20 millimeters (mm), usually appearing on the trunk, including the upper chest and back.

About 10% of people with SLE have subacute cutaneous lupus. Certain drugs may also cause subacute cutaneous lupus. 

Acute Cutaneous

This is the type of skin flare that occurs when your SLE is active.

Lesions associated with acute cutaneous lupus appear as flattened areas of red skin on the face, reminiscent of a sunburn (the telltale butterfly rash). These lesions are photosensitive and can appear on the:

  • Arms
  • Legs
  • Body

Though the lesions may discolor the skin, they don't scar. Lesions typically appear during a flare or after sun exposure.

Note that both chronic/discoid and subacute cutaneous lupus may occur independently, or they may be manifestations of SLE, whereas acute cutaneous lupus does not occur outside of SLE.

Neonatal Lupus

Neonatal lupus is a rare form of temporary lupus affecting a fetus or newborn. Technically, it's not true lupus: It occurs when the mother’s autoantibodies are passed to her child in utero. These autoantibodies can affect the baby's skin, heart, and blood.

Many babies with neonatal lupus will have skin rashes at birth. The rest will break out typically within two to five months. Sun exposure tends to bring on the outbreak.

Rashes, on average, will disappear at about six months or soon after as the mothers' autoantibodies are gone from the baby's body.

Treatment for skin lesions is typically no more than ointments to help relieve the severity of the breakouts. And fortunately, infants born with neonatal lupus are not at an increased risk of developing SLE later in life.

Though it's rare, some babies of mothers with lupus can be born with a heart condition that is permanent but treatable using a pacemaker. This abnormality can be detected as early as the 18th week of pregnancy.

Lupus Symptoms

While each type of lupus can have some predominant symptoms, most overlap in some ways.

Initial symptoms of lupus often include:

  • Fever
  • Malaise, or general discomfort
  • Joint pain
  • Muscle pain
  • Fatigue

The fact that early and chronic lupus symptoms mimic the symptoms of several diseases can lead to misdiagnosis. It usually isn't until those symptoms are coupled with other, more suggestive signs that doctors tend to walk down the lupus path.

Those signs include, but are not limited to:

Inflammation (the primary feature of lupus) is at the root of resulting pain, heat, redness, swelling, tissue damage, and loss of function, either internally (certain organs), externally (primarily the skin), or both.

In Children

Childhood lupus affects somewhere between 5,000 and 10,000 children under 18 years of age in the United States. It is most often diagnosed between ages 11 and 15, but children of any age can have lupus.

As in adults, lupus expresses itself differently in each child, though children seem to have more organ involvement. This may be because children have often been sick for a longer period of time when they're finally diagnosed.

Symptoms in children are similar to symptoms in adults, the most common of which are fatigue and achiness. Obvious disease symptoms include fever, butterfly rash, and kidney involvement.

Causes

Normally, the immune system protects the body against microbial invaders like viruses and bacteria. With lupus (like all autoimmune diseases), this system malfunctions, causes a rise in inflammation, and begins to attack the body instead.

Despite knowing this, lupus is still a relative mystery to medical researchers. An exact cause of the disease remains unknown, and many still debate whether lupus is one disease or the combination of several similar diseases.

The consensus on the most likely ways in which lupus develops includes:

  • Genetics: You can inherit some risk factors from your parents.
  • Environmental triggers: These include medications, stress, viral infections, and/or exposure to sunlight.
  • Viral infections: These include cytomegalovirus (CMV), Epstein-Barr virus (EBV) which causes mononucleosis, and herpes zoster which causes shingles.
  • Reaction to drugs: This only applies to drug-induced lupus.
  • Hormones, such as estrogen: Lupus is more common in women during their childbearing years when estrogen levels are highest, and many women have lupus symptoms before menstrual periods or during pregnancy when estrogen production rises.

Diagnosis

If your doctor suspects that you have either cutaneous lupus erythematosus alone or in combination with systemic lupus erythematosus, several factors are considered, including:

  • Symptom history
  • Physical exam findings
  • Laboratory findings
  • Antibody tests
  • Tissue biopsy
  • Direct immunofluorescence

Lupus is often diagnosed using an anti-nuclear antibody blood test (ANA), which identifies autoantibodies that attack your body's own tissues and cells. They're found in the blood along with healthy antibodies, red blood cells, white blood cells, and other blood matter.

A positive ANA does not automatically mean you have lupus, however. That result is one piece of the puzzle of a lupus diagnosis.

Autoimmune diseases like lupus can be difficult to diagnose and differentiate. And ultimately, because of the wide range of symptoms, most people are first diagnosed five years after first developing symptoms.

If you feel that you (or your child) may be experiencing symptoms consistent with a diagnosis of lupus, be sure to visit your physician and express this concern.

Lupus Doctor Discussion Guide

Get our printable guide for your next doctor's appointment to help you ask the right questions.

Doctor Discussion Guide Woman

Treatment

Treatment for lupus often involves a combination of drug and non-drug therapies, as well as lifestyle changes.

For some patients who are mildly afflicted, lupus symptoms can be managed. However, the disease can be quite serious and even life-threatening for others.

Lupus does not follow a common path, so lupus patients often suffer unpredictable bouts (flares) followed by periods of remission—even with treatment.

Drug Therapies

Anti-inflammatory drugs and immune system modulators are often used for lupus, but the medication or combination of medications used will vary depending on your symptoms, parts of the body affected, and responsiveness to certain treatments. You have several options.

Over-the-Counter Pain Relievers

  • Aspirin can help manage pain and has anti-inflammatory and anticoagulant (blood-thinning) properties, which can be helpful given the increased risk of blood clots with lupus.
  • Tylenol (acetaminophen) may be used to manage pain or fever.
  • Non-steroidal anti-inflammatory drugs (NSAIDs) such as Motrin or Advil (ibuprofen) or Aleve (naproxen), are commonly taken to reduce inflammation and manage pain and stiffness.

Prescription NSAIDs

For higher doses of anti-inflammatories, prescription NSAIDs are available, including:

  • Indocin (indomethacin)
  • Relafen (nabumetone)
  • Celebrex (celecoxib)

Antimalarials

Antimalarials tend to be most effective in those with mild forms of lupus. Common ones prescribed for lupus are:

They can help reduce joint swelling, mouth ulcers, and skin rashes.

Corticosteroids

Oral corticosteroid medications are generally taken during flares as a fast-acting way to lessen the immune response and reduce pain and swelling. They include:

These medications are designed to work like your body's hormone cortisol, which helps regulate blood pressure and lessen immune response.

Due to their side effects, corticosteroids are often only used if NSAIDs and antimalarials aren't enough to manage symptoms. For lupus, corticosteroids are usually taken in pill form, but it also comes in injections, infusions, topical gels, and creams.

Treatment may need to be a little more aggressive for kids than adults, but doctors also have to be careful about the long-term side effects of medications, particularly corticosteroids like prednisone. With the right treatment, most kids lead a normal childhood.

Immunosuppressives

Medications that suppress the immune system, including the chemotherapy drugs, or that help prevent organ rejection after transplants may be prescribed for lupus, including:

  • Cytoxan (cyclophosphamide)
  • Rheumatrex (methotrexate)
  • Imuran (azathioprine)

Immunosuppressive therapies are often used in those with more severe forms of lupus that haven't responded to other treatments.

Anticoagulants

Low-dose aspirin or prescription blood thinners may be taken long-term to keep the blood from clotting too easily. These include:

  • Calciparine
  • Liquaemin (heparin)
  • Coumadin (warfarin)

Biologics

Biologic drugs are synthesized from living cells. One biologic—Benlysta (belimumab)—is frequently prescribed for lupus

Benlysta is a monoclonal antibody (mAbas), which means it attaches to only one substance in the body.

Specifically, this medication targets and blocks a protein needed for the activation of B cells (B lymphocytes), a group of white blood cells that play a key role in immune responses.

It's either injected with a syringe or given in an intravenous (IV) infusion and often used in combination with other medications for lupus.

Repository Corticotropin Injection

H.P. Acthar Gel contains adrenocorticotropic hormone (ACTH) that is injected under the skin or into a muscle to help reduce inflammation.

It may help the body produce more of its own natural steroids to assist the immune system.

Non-Drug Therapies

Several therapies can be used alone or in combination with drug therapies to manage symptoms or improve physical and mental health of those with lupus.

  • Cognitive behavioral therapy: Research shows that cognitive behavioral therapy, a type of talk therapy, can help lessen anxiety and depression in those with lupus who have high levels of daily stress.
  • Physical therapy: Physical therapy for lupus can help address stiffness and help restore or maintain mobility and physical function. Custom-tailored exercises may focus on strength, balance, coordination, or endurance.
  • Acupuncture: Preliminary research suggests that acupuncture may help manage the pain and fatigue of lupus.

Lifestyle Changes

Healthy lifestyle habits may help you manage or reduce symptoms of lupus and can even help lower the dose or number of medications needed.

  • Eat a healthy diet: There isn't a specific diet for lupus, but it's important to eat nutritious and well-balanced meals that include plenty of fresh fruits and vegetables.
  • Exercise: Regular exercise can help protect the heart, manage stress, and improve energy levels and mood.
  • Limit alcohol: Alcohol can interact with some lupus medications or increase side effects, such as the gastrointestinal risks of NSAIDs, so discuss alcohol use with your doctor.
  • Wear sunscreen and sun protective clothing: Lupus can increase sensitivity to UV rays and sunlight may trigger flares. Use at least SPF 30 sunscreen and wear hats and coverings when you're outdoors.
  • Don't smoke: As with most medical conditions, smoking can complicate or accelerate lupus so it's important to quit or limit smoking.
  • Manage stress with mind-body practices: Chronic stress may worsen your quality of life; about 25% of people with lupus have major depression and 37% have anxiety. Meditation and breathing practices may help lower stress, alter pain perception, and improve mental health.
  • Rest: You may need additional rest, especially during bouts of fatigue, and should aim for at least eight hours of sleep each night.

Coping

Lupus can bring all sorts of physical and emotional challenges, especially if you're newly diagnosed.

Learning to cope with your disease takes time and practice, and includes things like:

  • Educating yourself and your loved ones about your disease
  • Taking care of yourself by getting enough rest and eating well
  • Learning how to manage your flares
  • Finding support

Prognosis

The prognosis for those with lupus often depends on the amount of organ involvement.

Survival for lupus patients with central nervous system symptoms, major organ involvement, and/or kidney disease is likely to be shorter than those with only skin and/or joint disease related to lupus.

Studies on SLE have found that about 75% of people diagnosed early in their disease course live more than 20 years and their quality of life is comparable to those living with other types of chronic diseases. 

The most common cause of death early in the disease course is infection due to immunosuppression, caused either by SLE or the medications used to manage it.

Later on, accelerated heart disease is leading cause of death. Lifestyle interventions and the use of medications when needed can help minimize the added heart risks for those with lupus.

According to the National Resource Center on Lupus, about 80% to 90% of people with lupus have a normal life expectancy.

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Article Sources
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