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Lupus

  • An autoimmune disorder that causes inflammation in the skin, joints, and blood vessels
  • Symptoms include fatigue, joint pain, rash, leg swelling, chest discomfort
  • Treatment includes anti-malarial or anti-inflammatory medications
  • Involves rheumatology

Overview

Lupus, an autoimmune disorder, is often known as “the great imitator” because it can resemble and mimic many other diseases and conditions. 

Lupus causes inflammation in the skin, joints, and blood vessels and comes with symptoms that can include nearly anything from a skin rash to neurologic problems. This complex array of symptoms can make it hard for doctors to recognize lupus, meaning that people with lupus may suffer for years without a correct diagnosis. 

“Lupus patients are like snowflakes,” says Yale Medicine rheumatologist Fotios Koumpouras, MD. “No two people are quite alike, and everyone experiences the disease differently.” 

What are the symptoms of lupus?

Lupus can affect many different organs and systems of the body, so its symptoms can vary greatly from person to person.

However, many people with lupus experience any or all of the following symptoms:

  • Fatigue
  • Joint pain
  • Rash
  • Leg swelling
  • Chest discomfort
  • Symptoms made worse with exposure to sunlight

What are the most common types of lupus?

The two main forms of lupus are:

  • Cutaneous lupus: This is the most common form of lupus and causes inflammation only in the skin. It can lead to hair loss, pigment changes, rashes, and sores all over the body—especially on the face, neck, and scalp. One common type of rash, which appears as raised—often red—and sometimes scaly circles, is called discoid rash. Another type, which appears on the cheeks and bridge of the nose, is called a butterfly rash.
  • Systemic lupus: This type of lupus occurs in about 5% of cases; it causes external rashes, as well as inflammation in internal organs and blood vessels. It can be mild or severe. Severe cases can lead to serious complications, including kidney, nervous system, and cardiovascular damage.

In rare cases, women with lupus give birth to babies who have lupus-like symptoms, such as skin rash and liver problems. The condition is known as neonatal lupus, although it is not a true form of the disease, and the symptoms usually disappear within a few weeks to months. Neonatal cardiac lupus is also rare but can cause permanent damage to the fetal heart.

What are the risk factors for lupus?

Scientists aren’t sure what causes lupus, but they know there is a genetic component. It can run in families, but having a relative with lupus does not mean their family members will have it, too. 

“There are many genes involved in whether a person develops lupus, so it’s very difficult to predict or screen for genetic abnormalities,” says Dr. Koumpouras.

A person usually develops lupus as a result of an environmental trigger. This can be physical trauma, such as a motor vehicle accident, an infection, or even pregnancy. One illness that’s been linked to lupus is the Epstein-Barr virus.

More women than men develop lupus, and most people are diagnosed between ages 15 and 44. 

How is lupus diagnosed?

Getting a lupus diagnosis can be difficult and frustrating. “It can sometimes take patients ten years or more to get real answers for their symptoms,” says Dr. Koumpouras. “But going to a rheumatologist can make it a lot easier.”

Rheumatologists, doctors specializing in joint and muscle disorders, have experience diagnosing and treating lupus. Patients may be referred to them when they complain to their primary care doctor about joint pain. A blood test can confirm that an inflammatory condition is to blame.

People who see dermatologists because of a suspicious skin rash may also be referred to rheumatologists. Lupus can also cause blood clots, and rheumatologists can be called in when an unexplained clot is discovered.

No one lab test can confirm if a person has lupus, and doctors should first rule out other conditions that could be causing symptoms. To be diagnosed with systemic lupus, a person should meet at least four of the following criteria:

  • A butterfly rash or discoid rash
  • Sensitivity to the sun
  • Recurring ulcers in the mouth or nose
  • Stiffness and pain in the joints
  • Inflammation of the lungs or around the heart
  • Seizures or other neurologic problems
  • A kidney disorder, marked by protein in the urine
  • Anemia or abnormal blood cell counts
  • An immunologic disorder, marked by antibodies in the blood
  • A positive result on an Antinuclear Antibodies (ANA) blood test

How is lupus treated?

Lupus is usually treated with antimalarial medications. In people with malaria, these drugs kill the parasite that causes the disease. In people with lupus, they stop hyperactive immune cells from running out of control.

These drugs—such as Plaquenil (hydroxychloroquine), Aralen (chloroquine), and Atabrine (quinacrine)—can help to control lupus symptoms and prevent the disease from spreading to the kidneys and nervous system. The drugs are safe, side effects tend to be mild, and many lupus patients take them for the rest of their lives.

In addition to antimalarials, people with lupus may also take anti-inflammatory medicines, such as ibuprofen or naproxen, to relieve joint pain and stiffness.

Another drug, called Benlysta (belimumab), is a type of biologic that is administered intravenously. Some people with mild-to-moderate lupus may benefit from using Benlysta in addition to their other lupus medications.

In more serious cases, lupus patients may need to be treated with corticosteroids, chemotherapy, or immunosuppressant drugs. But those medicines have more side effects and are not used unless antimalarial drugs have not worked or if lupus has affected vital organs.

What makes Yale Medicine’s approach to treating lupus unique?

Newly diagnosed patients at Yale Medicine have the option of going through a comprehensive lupus education program. In four sessions spread out over a year, an advanced practice registered nurse teaches them about living with lupus, including how to avoid situations that might cause a flare-up and how to recognize flares.

Patients also have access to doctors with extensive lupus experience.

“We have physicians who are experts in kidney lupus and pulmonary lupus, for example,” says Dr. Koumpouras. “The program allows patients to see subspecialists who know their specific disease.”

Yale Medicine is also involved in several clinical trials on potential new drugs to treat lupus. “If individuals are not getting better with standard treatment, then they have the option of exploring new therapies being studied here,” says Dr. Koumpouras.