New hope for people living with lupus

A diagnosis of lupus used to portend a dire future. But the outlook for people diagnosed with the chronic autoimmune disease today is improving with new treatments, and most patients can manage symptoms with medication and healthy lifestyle choices.

“We have many more treatments than we did a few years ago,” said Rheumatologist Martina Ziegenbein, MD, who practices at the Cape Cod Rheumatology Center in Hyannis.

What is lupus?

In lupus, the body’s immune system, which normally protects by destroying germs and damaged cells, instead targets healthy tissue. Lupus can be mild or life-threatening, and symptoms tend to flare up and then subside for a period of time. It can strike skin, joints, heart, lungs, kidneys, nerves and brain. Treatment varies upon severity and the organs or tissues involved. The cause is unknown and there’s no cure, according to the American College of Rheumatology.

While lupus strikes people of all ages and genders, women develop it 10 times as often as men, and one in five case are children, according to the American College of Rheumatology. Black and Hispanic women are twice or more likely to get lupus than white women, says the Centers for Disease Control and Prevention.

“Women of color are more affected, and we don’t know why,” said Dr. Ziegenbein, who completed a fellowship in lupus at Johns Hopkins School of Medicine’s Lupus Center in Baltimore, MD.

The severity of illness and effectiveness of some treatments also seems to vary according to whether or not the patient is a person of color, she added.

Symptoms and diagnosis

There is no test for lupus, but rather a diagnosis is made based on a collection of symptoms. Common symptoms include a butterfly-shaped rash across the cheeks and nose, joint pain and photosensitivity (exposure to sunlight or artificial UV rays triggers a flare-up of symptoms), Dr. Ziegenbein said. Lupus patients also often report hair loss, low fevers, fatigue, headaches and Reynaud’s syndrome (when fingers and toes turn white when cold). The federal Centers for Disease Control and Prevention have this complete list on their website.

If lupus is suspected, an ANA (antinuclear antibody) blood test may be ordered. According to the Lupus Foundation of America, 97 percent of people with lupus will get a positive result to this test, which looks for presence of antibodies that attack the nuclei of the body’s cells. However, a positive ANA test by itself does not mean you have lupus, as up to 25 percent of the general population may test positive, Dr. Ziegenbein said.

“It’s a very sensitive test,” she said.

After diagnosing a patient with lupus, Dr. Ziegenbein said she tests for protein in urine to gauge kidney function. Early stages of kidney disease may go unnoticed, she said. If a test indicates kidney problems, a biopsy would be performed to confirm whether there is inflammation and any permanent scarring.

“Kidney disease can be present in up to 40 percent of patients upon diagnosis,” she said.

Treatment

Treatment for all lupus patients should start with hydroxychloroquine, Dr. Ziegenbein said. This anti-malarial drug has long been used to reduce symptoms of fatigue, arthritis, mouth sores, rashes and flare-ups, according to the American College of Rheumatology. Hydroxychloroquine was in the news in 2020, when then-President Donald Trump promoted it as a protection against COVID, and the resultant surge in demand caused a third of lupus patients to have difficulty filling their maintenance prescriptions, according to the Lupus Research Alliance. Fortunately, that shortage has passed, Dr. Ziegenbein said.

Prednisone and other corticosteroids, drugs that suppress immune reactions and non-steroidal anti-inflammatory medicines (NSAIDS), such as ibuprofen can ease symptoms, though all carry side effects. Treatment requires balancing the benefits and risks of each drug, Dr. Ziegenbein said. Some young women are able to remain on a low dose of prednisone for many years, she said.

Patients with severe cases may travel to research centers to participate in clinical trials of drugs not yet approved by the FDA for use in lupus, Dr. Ziegenbein said. These might include rare cases with brain involvement, which can cause confusion and seizures.

New treatments

The Lupus Foundation of America cites these latest treatments:

• Anifrolumab-fnia (Saphnelo), a monoclonal antibody given by infusion. It targets messenger proteins in the immune system. In double-blind studies, it reduced lupus symptoms. The Food and Drug Administration approved it in August 2021 for treatment of people with moderate to severe cases of the most common form of lupus.
• Voclosporin (Lupkynis), a pill to reduce inflammation of the kidneys, or lupus nephritis, a serious complication of lupus. Approved by the FDA in January 2021.
• Belimumab (Benlysta), a monoclonal antibody given by injection or intravenous infusion that reduces creation of antibodies that attack healthy tissue. In December 2020, it became the first drug approved for treatment of lupus nephritis, when used in combination with other medications considered standard treatment. In March 2011 this same drug became the first drug in over 50 years to be approved by the FDA to treat lupus.

Lifestyle concerns

Awareness of health issues associated with lupus can help guide living with the disease. For example, lupus increases the risk of plaque buildup in arteries (atherosclerosis), making patients more likely to have a stroke or heart attack. It also heightens the risk of bone-thinning, or osteoporosis.

Blood disorders are common, as lupus can affect red blood cells, white blood cells and platelets. Anemia can result from low red blood cells. Lupus can heighten the chance of blood clots, so a blood thinner may be prescribed in those cases, Dr. Ziegenbein said. Ironically, lupus also can cause difficulty with clotting. Such variation requires individualized treatment.

“It’s really important to look at the whole patient.” she said.

A diet rich in vegetables and fruit, whole grains, lean proteins and healthy fats and low in salt, saturated fats and sugar can make it easier to manage lupus symptoms, according to the Lupus Foundation of America.

“We encourage people to be as active as they can be,” Dr. Ziegenbein said.

“Photosensitivity is one of the hallmarks of lupus – always apply a high SPF (sun block),” she added.