What is lupus?

Lupus¹ is a chronic autoimmune disease that can affect multiple regions of the body. The most commonly affected areas are the joints, skin, blood vessels, brain, lungs, and kidneys.

There are several types of lupus, including:

• Systemic lupus erythematosus (SLE)²: The most common form of lupus, SLE causes symptoms in the joints, skin, lungs, kidneys, brain, and blood vessels. SLE is also the most widespread and chronic form of lupus.

• Subacute cutaneous lupus: This form of lupus causes skin rashes or lesions that are often photosensitive (worsen when exposed to sunlight).
• Drug-induced lupus: This condition occurs as the result of an adverse reaction to certain medications. The symptoms of drug-induced lupus are similar to those seen in SLE and typically subside when the offending medication is removed.
• Neonatal lupus: This form of lupus occurs when a mother with SLE passes autoantibodies (immune cells that wrongly target the body’s own cells instead of foreign bodies or infections) to her baby. Infants with neonatal lupus can develop skin, liver, heart, or blood problems.

How common is lupus?

Lupus affects an estimated 5 million people worldwide³ and 1.5 million people in the United States. The vast majority (90%) of cases of lupus in adults affect women.

Current data indicates that about 16,000 new cases of lupus are diagnosed each year. Additionally, one in three people with lupus also has another type of autoimmune disease.

What causes lupus?

Lupus is an autoimmune disease. Autoimmune diseases occur when the immune system mistakenly targets health tissues in the body, rather than foreign invaders (pathogens).

Researchers believe that lupus is caused by a combination of inherited (genetic) and environmental factors. Certain environmental triggers may cause the symptoms of lupus to develop or worsen (flare-up) in those with a genetic predisposition for the disease.

Potential triggers⁴ of lupus include:

• Medications: Some antihypertensives (medications used to treat high blood pressure), antibiotics, and anti-seizure medications, have been known to trigger episodes of lupus (referred to as drug-induced lupus). The symptoms that occur during these episodes typically subside once the use of the triggering medication(s) has been discontinued.
• Sunlight: People with lupus may be more photosensitive (sensitive to sunlight). As a result, prolonged sun exposure can result in rashes or skin lesions.
• Infections: Infections typically cause an immune response, which can trigger an episode of lupus.
• Stress or overwork: Some people find that additional life stress or not getting enough rest can trigger⁵ lupus flares.

• Discontinuing use of your lupus medications: Medications can help keep episodes of lupus at bay. Discontinuing the use of these medications can cause flare-ups to occur. (It’s possible, however, for patients to still experience flares when they are fully compliant with their medications.)

Risk factors for lupus

Both genetic and environmental factors⁶ can contribute to an increased risk of developing lupus. Some of these factors include:

• Age: Lupus most frequently develops between the ages of 15 and 44. Only 15% of people with lupus develop symptoms prior to the age of 18.
• Sex: Women are much more likely to develop lupus than men.
• Race/ethnicity: Lupus is seen more commonly in people from non-Caucasian backgrounds. In the United States, the incidence of lupus is higher in African Americans, Asian Americans, Native Americans, Hispanics/Latinos, Pacific Islanders, and Native Hawaiians.
• Family history: Your risk of developing lupus is 5–13% higher if you have a relative with the condition. If that relative is your mother, however, your risk is closer to 5%.

Lupus symptoms

The symptoms of lupus can vary from person to person. Signs of the condition can develop slowly over time or come on suddenly. They may go away quickly or persist for a long period of time.

The signs and symptoms of lupus characteristically occur in episodes known as flares. Flares (also called flare-ups) are periods of time during which the symptoms of the disease temporarily worsen.

Common signs and symptoms⁷ of lupus include⁸:

• Rashes (commonly a key characteristic of lupus, featuring a butterfly-shaped rash across the cheeks and nose)
• Fatigue
• Joint pain, stiffness, or swelling
• Fever
• Skin lesions that are sensitive to sun exposure (photosensitive)
• Chest pain
• Shortness of breath
• Fingertips and toes turning white or blue during exposure to cold temperatures or stress (Raynaud’s syndrome)
• Headaches
• Confusion
• Memory loss
• Hair loss
• Swelling around the hands, feet, abdomen, or eyes
• Brown or foamy urine
• Sores in the mouth
• Anxiety, sadness

If you experience any of the signs or symptoms of lupus, see a healthcare provider as soon as possible. They will be able to determine whether your symptoms are the result of lupus and provide you with the proper treatment.

Lupus symptoms in women

The majority (9 out of 10) cases of lupus in women occur between the ages of 15 and 44⁹. Women with lupus may experience certain health problems earlier in life than those without the disease, such as:

• Osteoporosis: Osteoporosis, a disease that causes a loss of bone density and quality, occurs in women as they age. Some medications that treat lupus can accelerate bone degeneration, which may cause early osteoporosis.

Additionally, while regular exercise can help prevent or slow the loss of bone density, the symptoms of lupus may prevent some women from being able to lead an active lifestyle.

• Heart disease: People with lupus are at an increased risk of coronary artery disease (CAD), the most common form of heart disease. Lupus is associated with several known risk factors for CAD, including high blood pressure, high cholesterol, swelling or inflammation, and type 2 diabetes.

As with osteoporosis, the risk of heart disease can be lowered with regular physical activity; however, this may not be possible during periods of severe lupus episodes. In part due to this reason, women with lupus between the ages of 35 and 44 are fifty times more likely¹⁰ to have a heart attack than women in this age group without lupus.

• Kidney disease: Over 50% of people with lupus also develop inflammation of the kidney (a condition known as lupus nephritis). Kidney inflammation can be very dangerous—because it doesn’t always cause painful symptoms, some patients may not know they have lupus nephritis until it has progressed to a late stage.

Patients with lupus nephritis typically begin to develop the disease within five years of the onset of their lupus symptoms. If you have lupus, your healthcare provider will likely administer regular urine and blood tests to check your kidney function.

Lupus¹¹ affects women of color more commonly than Caucasian women. African-American women, for example, are three times more likely to develop lupus than Caucasian women.

In addition, African-American women and Hispanic women tend to develop lupus symptoms at an earlier age, and the symptoms themselves tend to be more severe than those seen in racial groups. Non-Caucasian patients also tend to experience more related complications, such as stroke, heart swelling, seizures, and other heart problems.

Lupus rash

Lupus that causes symptoms only affecting the skin is generally known as chronic cutaneous lupus. While not all patients experience skin-related symptoms alone, roughly 60–70% of people with lupus will develop some form of skin rash¹² or lesion.

While there are different types of rashes associated with lupus, the most common rash is known as the “butterfly rash” or malar rash.

Malar rashes occur more frequently in patients with SLE than in those with other forms of lupus. These rashes are reddish in color and may resemble rosiness or blushing. They usually cover both cheeks and the bridge of the nose, but may sometimes appear blotchy. While butterfly rashes aren’t usually painful, they can sometimes be raised and itchy or feel warm to the touch.

About 20% of people with SLE experience a different type of rash known as discoid lupus erythematosus (DLE). This is characterized by thick, scaly, slightly reddish patches. These patches may leave scars when they heal.

Subacute cutaneous lupus erythematosus (SCLE) has its own distinct rash formation, characterized by red, splotchy, scaly patches that can cluster and increase in size. These rashes are typically seen on the areas of the body most frequently exposed to sunlight. Rashes of this type typically do not leave scars when they heal but may leave some hyperpigmentation.

Lupus nephritis

Lupus nephritis¹³ is a condition seen in roughly half of people with SLE (the most common form of lupus). Nephritis, commonly known as kidney inflammation, occurs when antibodies (in this case, those associated with lupus) attack the parts of the kidneys responsible for waste filtration.

Though women are more likely to develop lupus in general, men with lupus are more likely to have lupus nephritis. Non-Caucasian patients also tend to develop lupus nephritis more frequently than Caucasian patients.

Kidney inflammation can cause a number of serious symptoms, including:

• Blood in the urine
• Foamy appearance to the urine
• Hypertension (high blood pressure)
• Edema (swelling) of the hands, feet, or ankles
• High creatinine levels in the blood

Complications from lupus

People with lupus may experience both short- and long-term effects from the condition. The earlier the condition is diagnosed, however, the greater the chances of being able to continue living a normal lifestyle with minimal complications.

People with lupus may experience changes in their mental, physical, and social functioning. Having lupus can also increase your chances of developing other health conditions.

These complications and conditions¹⁴ may include:

• Lung problems: Lupus can cause difficulty breathing, bleeding around the lungs, and pneumonia. It may also increase your risk of developing pleurisy (inflammation of the lining of the chest cavity).
• Blood vessel problems: Lupus can lead to anemia (low red blood cell count), an increased risk of bleeding, vasculitis (blood vessel inflammation), and blood clotting problems.
• Kidney problems: Lupus may cause kidney damage, which can ultimately lead to kidney failure. Failure of the kidneys to function properly is one of the leading causes of death in people with lupus.
• Heart problems: People with lupus may be at increased risk for pericarditis (inflammation of the heart muscle, arteries, or tissue membrane surrounding the heart), cardiovascular disease, and heart attacks.
• Brain and central nervous system problems: When lupus affects the brain, the resulting symptoms can vary, depending on the area of the brain affected. Common central nervous system symptoms include headaches, dizziness, vision changes, and behavior changes. In some cases, stroke or seizures may occur.
• Cancer: Though the risk is small, having lupus seems to increase the risk of developing cancer.
• Infection: People with lupus tend to be more susceptible to infection. This is due to the fact that both the condition itself and the treatments used to manage it can weaken the immune system.
• Complications in pregnancy: There is an association between lupus and miscarriage, preeclampsia (increased blood pressure during pregnancy), and preterm birth. Healthcare providers generally recommend waiting until lupus has been well-managed for at least six months before trying to become pregnant.
• Avascular necrosis (bone tissue death): Lupus may diminish the supply of blood flowing to the bones. This can cause small fractures to form, ultimately causing the bones to collapse.