What Is Lupus?

With its constellation of quirky symptoms—many of which can mimic other conditions—lupus is a complex disease to diagnose, leading some to call it"the great imitator."Scientists don't know exactly what causes lupus, and most people know very little about the disease.

Lupus is an autoimmune disease that can attack the skin, joints, organs, nervous system, blood cells, kidneys, or some combination of body systems. Most cases strike women, although lupus can occur in men, too.

It helps to know how the immune system works. A healthy body is hardwired to produce antibodies that fight off germs and other foreign substances. Lupus occurs when this system goes haywire. Instead of defending against enemy invaders, like viruses and bacteria, it produces antibodies that target healthy tissue, leading to inflammation, swelling, and damage.

The term "lupus" commonly refers to systemic lupus erythematosus, or SLE, which accounts for 70% of all lupus cases. According to the Centers for Disease Control and Prevention (CDC), there were approximately 204,295 people in the US with SLE in 2018. Some people also have cutaneous lupus erythematosus (CLE), a type of lupus that affects the skin.

Signs and Symptoms of Lupus

People with lupus may experience an array of symptoms. Some common ones include unexplained fever, fatigue, joint pain, or rash. All of these can easily be confused with any number of illnesses. If not closely managed, lupus can leave a trail of damage in its wake.

The type and severity of lupus symptoms can vary, as can the frequency of flares. Some people have symptoms that persist over long stretches of time, while others see their symptoms subside or disappear for a while (this is considered a remission), only to flare again later on. In other words, one person's experience with the disease can be entirely different from someone else's.

In some people, lupus affects a single body system—like the skin or joints. In others, lupus leaves its mark across multiple body systems, such as the kidneys, heart, lungs, blood, blood vessels, and brain.

A butterfly-shaped rash stretching from cheek to cheek across the bridge of the nose is a hallmark of lupus.

The kidneys are particularly vulnerable in people with lupus.When the kidneys aren't functioning properly, people can develop swelling around the ankles and eyes (edema), blood in the urine, or weight gain.

Some people have pain when breathing, a possible sign of inflammation of the lining of the chest.

Signs of lupus include:

• Fatigue or malaise
• Joint pain, stiffness, or swelling
• Muscle pain or weakness
• Fever with no known cause
• A red, butterfly-shaped rash across the nose and cheeks
• Other rashes, particularly on areas of the body exposed to the sun
• Pain in the chest when taking a breath
• Swelling in the feet, legs, fingers, or around the eyes
• Hair loss
• Mostly painless mouth ulcers, particularly on the roof, gums, and sides of the mouth or inside the nose
• Anemia
• Pain or purple fingers or toes due to cold or stress
• Sun sensitivity
• Dry eyes
• Headaches, confusion, and dizziness
• Blood clots
• Stroke
• Seizures or psychosis

Lupus Brain Fog

People with lupus are more likely to have lapses in memory, difficulty concentrating, and confusion. A 2019 study published in the Annals of Rheumatic Diseases reported that up to 90% of patients with SLE experience cognitive dysfunction, also known as "brain fog." Lupus-related brain fog usually ebbs and flows but doesn't get progressively worse.

In some people, cognitive-related symptoms are bad enough to interfere significantly with daily life. Talk to your healthcare provider about lupus treatments that can address your symptoms and ways to circumvent your memory issues.

What Causes Lupus?

Scientists don't know exactly what causes lupus, but they think genetics may play a role. More than 100 genes have been linked to lupus, yet no single gene or combination of genes has been identified as the culprit. Some people appear to be at increased risk of developing lupus if a family member has it. It can also occur in people with no family history, although some family members may have other autoimmune conditions. The disease is more common in certain ethnic groups, especially African Americans, Hispanics, Native Americans, Asians, Alaskan Natives, Native Hawaiians, and other Pacific Islanders.

One theory is that environmental factors flip the switch that turns lupus on in people who are already genetically susceptible. Researchers have yet to pinpoint the exact factors, but a study in the Annals of the Rheumatic Diseases cites possible triggers as:

• Ultraviolet light
• Certain infections
• Certain drugs, including sulfa-based medicines, tetracycline, and antibiotics
• Exhaustion or emotional stress
• Physical stress from bodily injury, surgery, pregnancy, or childbirth

Scientists reported in the journal PeerJ that they suspect hormones—the body's chemical messengers—may be involved in the disease process in some way. About 90% of patients with lupus are women, mostly in their childbearing years, and many of them experience more lupus symptoms during pregnancy or before their menstrual periods. However, more research on this is needed.

Risk Factors

The key risk factors for lupus are gender, age, and geographic location. Nine out of 10 cases involve women, according to the LFA, although men get lupus, too. Most signs and symptoms of the disease appear between the ages of 15 and 45, but younger and older people can develop lupus as well. African-American women are more likely to have lupus than white women. The disease is also more prevalent in Hispanic, Asian, Native American, Alaskan Native, Native Hawaiian, and other Pacific Islander women.

How Is Lupus Diagnosed?

There is no single diagnostic test to confirm whether someone has lupus. Doctors rely on a combination of tools, including medical history and physical exam, blood tests, urinalysis, and kidney biopsy. Because of this, it can take months or years for someone to get a lupus diagnosis.

The American College of Rheumatology (ACR) has identified several common signs of lupus to help doctors diagnose the condition. A person with four or more of these problems, either currently or in the past, may have the systemic form of lupus:

• Butterfly and discoid rashes
• Photosensitivity (meaning a skin rash that develops due to sun exposure)
• Mouth or nose sores
• Arthritic pain with tenderness or swelling in two or more joints
• Swelling in the lining of the heart or lungs
• A neurological problem (such as seizure or psychosis)
• A kidney disorder (such as excessive protein in the urine)
• A blood disorder (such as anemia)
• Other blood abnormalities

Blood tests can detect the presence of certain antibodies associated with lupus. An antinuclear antibody (ANA) test, for example, screens for proteins that cause the body to begin attacking itself. However, a positive ANA test doesn't necessarily mean someone has lupus.

Lupus Treatment

Since there's no cure, getting a lupus diagnosis and starting treatment as soon as possible is the key to keeping lupus under control. Since every person with lupus experiences the disease differently, treatment regimens are tailor-made to the patient. In general, doctors prescribe medication to alleviate symptoms, prevent flare-ups, and minimize organ damage. These include:

• Non-steroidal anti-inflammatory drugs (NSAIDs) such as aspirin, ibuprofen (Motrin and Advil),and naproxen sodium (Aleve) are recommended to ease pain and fever, for example.
• Corticosteroids such as prednisone may be given to lessen the pain and swelling of inflammation in the body.
• Medicines for treating malaria have been shown to be effective in decreasing the production of antibodies that attack the body's organs and tissues.
• Immune-suppressing drugs are helpful when steroids fail to control symptoms or when patients cannot tolerate high doses of steroids. People taking immunosuppressants must be closely monitored because these drugs reduce the body's ability to fight infections.
• Low-dose aspirin or prescription warfarin or heparin may be prescribed to people at risk of blood clots.
• Biologics have also shown to be helpful in treating lupus. Benlysta (belimumab) and Saphnelo (anifrolumab-fnia) are two of the biologics available for lupus. Both are types of monoclonal antibodies and are given either intravenously or by injection.

When to See a Doctor

Many people struggle for years with vague or erratic symptoms before finally getting a diagnosis of lupus. Once you have been diagnosed with lupus, regular doctor visits are a must.

Managing lupus often entails having a team of doctors and nurses by your side. You may see a rheumatologist, who specializes in treating joint, soft tissue, and autoimmune diseases, or an immunologist versed in immune system disorders. Depending on your symptoms and complications, you may also see physicians who specialize in treating heart, kidney, lung, blood, hormone, skin, and nervous system issues.

You should see a doctor as soon as possible if your symptoms change or worsen, or if the medication your doctor prescribed isn't making you feel better. See a doctor immediately if you have severe abdominal pain; chest pain or shortness of breath; seizures; a new unexplained fever or high fever; excess bruising or bleeding; confusion or mood changes; or a combination of symptoms, such as severe headache, stiff neck, and fever.

The National Resource Center on Lupus offers tips for preparing for doctor visits. Start by knowing your medical history; keeping a journal of your symptoms and when they first appeared can help. Be prepared to answer specific questions about your symptoms. Know the names of all the medications you take—not just lupus medication—and bring copies of medical records from other physicians, including imaging test results. Ask for a summary of your doctor's orders and take notes. Ask questions and bring up any problems or concerns you have.

Lupus in Children

Lupus in children is as much of a mystery as in adults. In the beginning, the signs of trouble may be vague. Symptoms can appear and then vanish, making it difficult to pinpoint the cause. Children can suffer for a longer period of time than adults before they get a diagnosis, and, as a result, they may experience more health problems.

Depending on the body systems affected, lupus looks very different from one child to another. Some symptoms, like hair loss or skin rashes, are visible and may be scary for children. Other symptoms, including aches and pains or memory loss, are less obvious but just as concerning.

Raising a child or teen with lupus poses numerous challenges. You and your child will make regular trips to the doctor to keep the disease in check.It's up to parents to make sure their kids eat a healthy diet, get adequate sleep, and take their medicines as directed.

Children should learn to recognize their symptoms and how to prevent symptom flare-ups. That may mean slathering on sunscreen and wearing hats and long-sleeved shirts to avoid sun exposure—a potential trigger of lupus symptoms.

Prevention

There isn't a way to prevent lupus, but people with lupus can take measures to help prevent flares. It's important for people to partner with their healthcare provider about treatment plans. Medications that ease pain, clear up rashes, reduce inflammation, and quell the immune system can help many people living with lupus prevent and manage symptoms and stave off complications.

Maintaining a healthy lifestyle can also help people with lupus live life to the fullest. If you smoke, quitting can help since smoking can worsen the effects lupus has on the heart and blood vessels and may complicate treatment. Likewise, a heart-healthy diet may be beneficial.

Getting adequate rest is important, too, since fatigue is a chronic problem for many people with lupus. The National Sleep Foundation recommends seven to nine hours of sleep each day for adults; young children and teens require even more shuteye.

According to the Lupus Foundation of America (LFA), sun protection is another important step in managing lupus since ultraviolet rays can trigger a symptom flare.

Regular exercise, time management, and relaxation strategies like meditation can help keep stress—another possible lupus trigger—at bay, the LFA advises.