Lupus is an autoimmune disease in which the immune system mistakenly attacks healthy organs and tissues. There are four types of lupus, but the most common is systemic lupus erythematosus (SLE), affecting 70% of all people living with the disease. It’s a chronic condition characterized by the presence of a range of autoantibodies in the blood and systemic inflammation that can lead to serious damage in the kidneys, skin, joints, heart and brain over time.
While antibodies are proteins made by the immune system that protect the body against foreign substances, such as bacteria, fungi, viruses and toxins, autoantibodies or pathogenic antibodies mistakenly attack the body’s own cells and tissues instead of protecting them.
The course of disease for people living with lupus can be unpredictable. It typically triggers episodic flareups, causing symptoms that may be mild in one person and debilitating in another. Additionally, the toll of the disease doesn’t simply vary among individuals but has a strikingly heavier impact in certain patient populations.
SLE affects 3.5 to 5 million people worldwide, according to an article published in the medical journal Nature Reviews Rheumatology. It is estimated that approximately 200,000 people in the United States live with the disease, which disproportionately impacts nine times more women than men, often striking during childbearing years. Disease severity may vary according to ethnic background and is generally worse in patients of African ancestry and in Latin Americans. The difference in SLE prevalence and severity highlights the impact of race and ethnicity on the incidence of lupus.
“Lupus is a disease with huge inequalities in the way patients are diagnosed and how they are treated,” says Ashley Orillion, Ph.D., Scientific Senior Manager, Rheumatology & Maternal Fetal Immunology, Johnson & Johnson Innovative Medicine.
Johnson & Johnson has a long commitment to researching and developing potential therapies to treat autoimmune disorders. Below is an overview of SLE, plus a highlight of how Johnson & Johnson is working to help improve the lives of all people living with the disease.
What causes lupus?
The body’s normal response to threatening antigens is to produce chemicals, primarily antibodies, that help prevent infection and injury. However, the immune system of people with lupus overreacts to that threat, causing chronic inflammation. It’s this chronic inflammation that in turn damages organs and body systems.
Why some people develop lupus remains a mystery. “Experts aren’t sure exactly what triggers the immune system to overreact,” says Federico Zazzetti, M.D., Global Medical Affairs Lead for Rheumatology, Johnson & Johnson.
Evidence points to a few factors that may play a role. Genetics is one, says Dr. Zazzetti, as lupus can run in families. Exposure to ultraviolet light, previous viral infections, certain medications and a history of smoking could have an impact. Having another immune system disorder may also increase the odds. One in four individuals with an autoimmune disease will go on to develop another autoimmune condition.
Symptoms of lupus
One of the challenges in diagnosing SLE is that symptoms vary greatly depending on where the disease affects the body. “Every patient is different,” says Dr. Zazzetti. “The disease has a lot of different manifestations.”
Most patients experience fatigue and fever, he says. Some may develop seizures, inflammation of the kidneys, blood clots and memory problems, as SLE can damage the heart and brain. People living with SLE might also end up with arthritis, which causes joint pain, swelling and stiffness, thanks to the disease’s impact on the musculoskeletal system.
Lupus is a disease with huge inequalities in the way patients are diagnosed and how they are treated.
SLE can also affect the skin, causing red, thick and sometimes scaly rashes and sores that might burn or itch, says Dr. Zazzetti. Sometimes patients develop a rash across the nose and cheeks, which is known as a “butterfly rash.” Some people with SLE are sensitive to the sun, and a rash appears after sun exposure.
Skin changes associated with SLE are different in those with darker skin. The rash may cause thick, scaly, disc-shaped patches that are more likely to lead to scarring and discoloration.
Other symptoms can include gastrointestinal problems, headaches, depression, hair loss and Raynaud’s syndrome, which causes spasms in the small blood vessels in the fingers and toes, resulting in numbness and cold feelings in response to cold temperatures or stress.
One thing SLE symptoms have in common is that over time they impact a patient’s quality of life, limiting activity and making it more difficult to take on day-to-day tasks. Further, in rare cases, untreated SLE can be fatal.
How is lupus diagnosed?
The diagnosis of lupus involves a combination of clinical evaluation, lab tests and consideration of medical history. Despite many advances, no specific test alone can diagnose lupus. Since symptoms can be broad and overlap with those of other medical conditions, lupus is often misdiagnosed, says Dr. Zazzetti.
Doctors will go over a patient’s symptoms, then ask questions about their health, in part to find out if there’s a family history of autoimmune disease. They’ll likely administer a blood test to look for antinuclear antibodies, which are autoantibodies that can suggest something abnormal is happening with the immune system. Having a large number of antinuclear antibodies may signal an autoimmune disease. Doctors also often perform a complete blood count and a urinalysis to check for infections, says Dr. Zazzetti. The collective results of these varying tests can rule out other causes and point to lupus.
Treatment for lupus
Lupus is not considered curable at this time; doctors typically treat the disease by using a comprehensive approach aimed at managing symptoms, minimizing disease activity, preventing flares and reducing the risk of organ damage. The goal is to manage pain and discomfort, minimize and reduce the frequency of flares and help to prevent or slow organ damage, with a focus on improving quality of life. Treatment plans should be tailored to each patient based on his or her specific symptoms, severity of the disease and involvement of different organ systems.
Doctors may prescribe steroids, immunosuppressants, anti-inflammatory drugs or other medications, depending on availability of treatments and which organs and body systems are affected, plus how much damage has been sustained.
Making lifestyle changes can help some people deal with symptoms and minimize flares. These include maintaining a healthy diet, exercising a few times a week, limiting sun exposure, avoiding smoking and eating a heart-healthy diet.
Developing innovative therapies for lupus
Due to the complexity of lupus and its diverse effects on the body, scientists have faced significant challenges in developing new treatments. But Johnson & Johnson is working to help change that.
“We are researching a new wave of potential options for patients to be able to cope with the disease,” says Dr. Zazzetti. “Johnson & Johnson is dedicated to researching and innovating targeted therapies for patients with SLE. The company recognizes the huge medical need these patients have for additional treatment options that have a proven safety and efficacy profile.”
Addressing the health inequities of lupus
Johnson & Johnson is taking steps to address the health disparities related to the prevalence and treatment of lupus.
One group the company has partnered with is the Latin American Group for the Study of Lupus (GLADEL). Launched in 2019, this collaboration is collecting clinical data, biomarkers, treatment response and lifestyle information of more than 1,000 people living with lupus in Latin America, says Orillion. It will run through the end of 2025, and the data will be used to research and potentially develop more targeted lupus therapies, adds Dr. Zazzetti.
Another initiative is LupusNet, an ongoing collaboration that started in 2020. It aims to combine different data sources of people living with lupus from around the globe to create a unified global patient database that will be the largest in the world, says Dr. Zazzetti.
LupusNet currently includes five longitudinal observational registries of patients across North America, South America, Europe and Asia. The goal is to better understand the diversity of lupus globally, including how people access care in certain parts of the world.
“This is a way for us to take a global look at health inequities in lupus and understand how we can use this information to design the right trials for the right patients,” says Orillion.
