What Is Lupus? Symptoms, Causes, Diagnosis, and Treatment

Lupus is an autoimmune disease — meaning the immune system doesn’t function properly and attacks a person’s own body by mistake — that affects multiple organs throughout the body.

In healthy individuals, the body produces proteins called antibodies to fight off foreign invaders, such as viruses, bacteria, and fungi. But in people with lupus, antibodies can’t distinguish between foreign invaders and the body’s own cells and tissues.

This lifelong, chronic disease can affect almost every organ system in the body, although not all systems are affected in any one person. The first thing patients need to know is that lupus varies in symptoms and severity from person to person, says Stuart Kaplan, MD, a rheumatologist at Mount Sinai South Nassau in Oceanside, New York. Some people have active disease, while others have mild disease. It’s important to note that lupus is not contagious.

Lupus can cause any or all of these symptoms — and new symptoms can appear at any time. “The vast majority of lupus patients at some point will have joint pain with inflammation, some form of skin rash, and fatigue,” says Roberto Caricchio, MD, a rheumatologist at UMass Memorial Health in Worcester, Massachusetts.

• Fever
• Headaches
• Sun sensitivity
• Swelling of the hands, arms, feet, legs, and around the eyes (edema)
• Mouth or nose sores
• Hair loss
• Chest pain when taking deep breaths
• Raynaud’s syndrome, in which the small blood vessels in the fingers and toes spasm, limiting circulation, especially in cold weather or with stress

There are several types of lupus:

Systemic Lupus Erythematosus (SLE)

Cutaneous Lupus Erythematosus

Drug-Induced Lupus Erythematosus (DIL)

Some drugs can cause conditions very similar to lupus, resulting in symptoms such as rash, arthritis, hair loss, and fever. “Once medications are discontinued, the symptoms go away,” and thus, it is not SLE, says Dr. Caricchio.

Neonatal Lupus

Technically, neonatal lupus is not a form of lupus. The condition is the result of autoantibodies passing from a pregnant woman with lupus (or related condition) through the placenta and to the baby developing in the womb, causing mostly temporary symptoms, explains Virginia Pascual, MD, the director of the Drukier Institute for Children’s Health at Weill Cornell Medicine in New York City.

Some infants are born with symptoms such as a skin rash, liver problems, or low white blood cell counts. But those symptoms disappear within a few months, leaving no lasting effects.

So, what causes lupus? “We do not know,” says Caricchio. Research has yet to pinpoint the exact conditions or triggers that lead to the disease, he explains — “however, the condition appears to be multifactorial,” or can be impacted by several different things:

Genetics

Environment

Researchers suspect environmental factors may increase the risk of developing lupus. For example, exposure to sun can cause a lupus rash and some systemic lupus activity, says Stacy Ardoin, MD, an associate professor of rheumatology and immunology at the Ohio State University College of Medicine in Columbus, Ohio. Other environmental factors that may contribute to lupus include certain drugs, viral infections, exhaustion, stress, and anything that can cause physical stress on the body (such as surgery, physical harm, injury, pregnancy, or childbirth).

Hormones

Diagnosing lupus “can be very challenging,” Dr. Ardoin says. This is because symptoms can range from a mild rash and arthritis to kidney failure and seizures — “with a whole spectrum in between.” Symptoms may mimic other diseases and conditions, such as infections or cancer.

Other tests for lupus depend on the symptoms patients are experiencing, says Kaplan. For example, chest X-rays and echocardiograms may be necessary to investigate fluid around the lungs and the heart. If doctors suspect nephritis is present, the patient may need a kidney biopsy. Early diagnosis and treatment can help to avoid complications, he adds.

An accurate diagnosis of lupus can take time, says Caricchio. However, the availability of good lupus classification criteria can reduce the chance of receiving a wrong diagnosis, he adds. If doctors suspect you have lupus, they'll use classification criteria —  a set of symptoms and signs — to determine the type and severity of your case.

Fortunately, increased awareness among the general public and primary care physicians may mean patients may see a rheumatologist sooner rather than later for diagnosis, says Caricchio.

Prognosis of Lupus

While a lupus diagnosis can be overwhelming, if the disease is controlled, people living with the condition should be able to “go about life as normally as people who don’t have lupus,” Dr. Luk says.

Individuals should go on pursuing activities or hobbies they enjoyed before diagnosis, adds Caricchio. And, he says, women with lupus can have children. “We don’t discourage having kids by any means,” he says. “But it is recommended that women become pregnant when lupus is not active, so that the chance of flare is minimal.”

Lupus is a chronic, lifelong disease. However, most people don’t experience symptoms continuously. Instead, there are different times when symptoms appear, known as lupus flares. How long flares last varies from patient to patient, but symptoms can typically persist from a few days to weeks at a time. “There’s no way to predict when a flare will occur,” says Luk.

Flare symptoms can also be wide-ranging. Some people may experience relatively mild flares that include rash and arthritis, while others have severe flares, such as kidney inflammation, adds Luk.

Rheumatologists typically manage lupus with a variety of drugs, says Caricchio, adding that treatment regimens are tailored to each patient based on his or her symptoms.

Medication Options for Lupus

Some chemotherapy drugs and transplant drugs may be used, too, to treat patients with lupus nephritis or other organ problems, says Caricchio.

Why Monitoring Lupus Is Important

While lupus is more treatable than it used to be, the disease can still be life-threatening, Ardoin notes. “It’s hard to predict the course of illness, so it’s important for patients to check in regularly with their rheumatologist,” she says.

After people are diagnosed and begin treatment, they need to see their rheumatologists regularly for blood draws to monitor blood counts, systemic inflammation, and disease activity.

Blood tests also help doctors monitor levels of complement proteins, which boost the body’s immune response to infections. (Levels of complement proteins, as well as some others, are low when the disease is active, explains Ardoin.)

Blood and urine tests are also needed to check kidney function. “It’s important to regularly follow up with a rheumatologist to make sure the disease is not starting to become more severe,” says Luk. If kidney problems are not detected early, the risk of renal failure and death is higher.

Alternative and Complementary Therapies

In addition to treatment, certain lifestyle and dietary adjustments can help people with lupus. There’s no specific “lupus diet,” but incorporating foods that fight inflammation can be beneficial. Maintain a healthy balance of fresh vegetables such as dark leafy greens, whole grains, and protein from fish and beans, and watch out for sugar and unhealthy fats. Limit high-fat and processed foods. And avoid alfalfa, which has substances that can activate your immune system.

Dietary changes may also be necessary depending on which medication you’re taking for your treatment.

There’s no known way to prevent lupus itself, but there are steps you can take to help prevent flares of symptoms. You can’t entirely predict when a flare will happen, but it’s important to stick to your treatment and identify — and then avoid or reduce — triggers such as stress, infections, and sunlight.

Maintaining overall health is key, too. Because lupus is associated with an increased risk of cardiovascular disease, whether due to inflammation or genetics, maintaining a healthy lifestyle is particularly important, says Caricchio. He recommends getting enough sleep, maintaining a healthy body weight, exercising, and eating a nutritious diet.

Additionally, people with lupus should be mindful to avoid sun exposure as much as possible by wearing sunscreen and protective clothing, says Kaplan. A photosensitive rash can become worse in UV light.

Good social relationships and support groups can also be important for maintaining mental health, says Ardoin.

• Nephritis, or inflammation of the kidney, which can be mild or severe
• Fluid around the heart (pericardial effusion) and the lungs (pleural effusion), which can cause shortness of breath or chest pain
• Blockages of the blood vessels leading to the brain, causing stroke
• Inflammation of the heart, or myocarditis, which can cause chest pain and heart disease
• Inflammation of the brain, which can cause confusion, seizures, and psychosis
• Depression and problems with short-term memory
• A higher risk of miscarriage and preterm labor in pregnant women
• Long-term cardiovascular effects

• Lupus is diagnosed in about 16,000 people each year.

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• An estimated 204,000 people have SLE in the U.S. What’s more, 9 in 10 adults with lupus are women, and most people develop lupus between the ages of 15 and 44.
• It’s also two to three times more prevalent (and more severe) among Black and American Indian/Alaskan Native women than white women.

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Lupus in Children

Lupus in children tends to be more aggressive than in adults, says Dr. Pascual. The exact reasons for this are not understood. One theory is that people are born with a genetic susceptibility to the disease that may be triggered by environmental factors such as a virus. “Children with the condition may have inherited a more complex set of predisposing genes,” she says. But this theory has yet to be proved.

Children undergo the same diagnostic testing as adults, and treatments are similar. “There are few clinical trials in children, so there is no option but to treat children based on the adult experience. We use the same drugs,” says Pascual, with doses adjusted according to the child’s weight.

Doctors should closely monitor children for drug side effects. Steroids, for example, can delay growth and cause high blood pressure. Chemotherapy can make children more prone to infections, says Pascual. Fortunately, life expectancy in pediatric lupus has improved dramatically in the last 15 years, she says.

BIPOC and Lupus

The data from Dr. Somers’s group was a first step into gathering more reliable, detailed data on the issue, using a standardized way of defining cases.

"It’s important to have a firm understanding of baseline rates of this disease to flag trends in the population and how they’re changing over time,” Somers said.

The research also quantified, for the first time, the prevalence of lupus in men with direct comparison between ethnic groups. Lupus rates are highest in Black men, followed by Hispanic, Asian, and white men.

Somers’s group, funded by the Centers for Disease Control and Prevention, continues its work by looking at why there are such stark disparities in lupus between genders and race. Using the data from the five registries, the researchers are also looking at patients’ experiences with lupus and how the disease evolves.

Common overlapping conditions include:

• Sjögren’s syndrome
• Raynaud’s syndrome

Lupus may also occur with the chronic autoimmune conditions myasthenia gravis and scleroderma; antiphospholipid syndrome, which encourages the formation of blood clots; polymyositis and dermatomyositis, which can cause swelling in the muscles and skin.

• Lupus is an autoimmune disease where your body mistakenly attacks its own tissues, leading to inflammation and other complications like arthritis, rashes, and kidney damage.
• Cutaneous lupus affects the skin, and systemic lupus affects multiple organs.
• It’s important to actively manage the disease and regularly check in with your doctor to control symptoms and identify your personal triggers.