Systemic lupus erythematosus, more commonly referred to as “lupus,” is a chronic autoimmune condition. It affects multiple organs including the joints, skin, kidneys, brain, and lungs. Let’s find out everything there is to know about this condition, including its causes, risk factors, symptoms, and treatments.
What is Lupus?
Autoimmune diseases occur when our own immune system malfunctions and begins attacking different tissues in our body. Lupus is one of the common multi-system autoimmune diseases, causing inflammation in many organs. This inflammation results in various symptoms depending on the organ(s) involved.
In the United States alone, 1.5 million people have a form of lupus. Worldwide, at least 5 million people suffer from lupus. Lupus is 9 times more common in women. It is two to three times more prevalent in African-American, Asian, or Latina women than in Caucasian women. The disease is most frequently diagnosed in people aged 15 to 44 years, with 16,000 new cases of lupus diagnosed every year.
Causes
The causes of lupus are complex and poorly understood. There are some known genetic risk factors which play a small role. But these factors interact with multiple environmental risk factors that trigger the auto-immunity response, leading to lupus.
Risk Factors
Although the definitive cause of lupus is not well understood, there are multiple known risk factors that increase your chances of having lupus:
- Genetic. There are few known genetic variants related to immune function that could increase your risk of lupus.
- Gender. Females are nine times more likely to have lupus, which raises the possibility of hormonal factors leading to lupus.
- Race. African-American, Asian, and Hispanic women are more likely than Caucasian women to develop lupus. They also tend to get a more severe form of lupus.
- Environmental factors. Exposure to ultraviolet B light is a known environmental trigger for lupus. Other environmental factors that trigger lupus include diet, smoking, and vitamin D deficiency.
- Medications. There are some medications that are known to trigger lupus, for example hydralazine, procainamide, isoniazid, and minocycline.
Symptoms
Lupus is a chronic multisystem disease with relapsing and remitting symptoms. The symptoms of lupus vary depending on the organs involved. There is significant variation in the symptoms among patients. Here are the most common symptoms of lupus:
- Fatigue
- Fever
- Weight loss
- Skin rash (sensitive to light and worsens with sun exposure)
- Joint pain, swelling, and significant morning stiffness (more than 1 hour)
- Sores in the mouth or nose
- Chest pain due to inflammation around the heart (pericarditis) and lungs (pleurisy)
- High blood pressure
- Changes in urination (blood or foam in the urine)
- Seizures
Diagnosis
Lupus is diagnosed based on a combination of factors. In a patient with the above-described symptoms, a detailed clinical assessment (medical history and physical exam) by a clinician is the first essential step in the diagnosis of lupus.
Multiple blood tests can help with diagnosis and improve the understanding of the severity of the disease. Antinuclear antibody (ANA) is an essential blood test for the diagnosis of lupus. Other tests include a complete blood count (CBC), chemistry, complements, inflammatory markers, and urine tests. Certain abnormal antibodies including anti-double-strand DNA (called anti-dsDNA), anti-Smith (referred to as anti-Sm), or antiphospholipid antibodies will often be checked. Biopsy of the kidney or skin also helps with the diagnosis.
Treatment
The treatment of lupus depends on disease severity and the organ(s) involved. The primary goal of lupus treatment is to reduce inflammation, which may lead to decreased function, in the affected organs. Early establishment of care with a rheumatologist could help get the right treatment and prevent further organ damage.
Many of the medications prescribed for treatment of lupus could have significant side effects which require close monitoring with a rheumatologist.
- Hydroxychloroquine. This is an important medication in the treatment of lupus. It can relieve muscle and joint pain, improve skin rashes, and minimize fatigue and fever.
- Glucocorticorticoids. These are strong anti-inflammatory medications used to control inflammation due to lupus. They are used for a short period of time to quickly reduce the inflammation and provide immediate symptom relief.
- Immunosuppressant drugs. There are multiple immunosuppressive medications used for various forms of lupus. Some of the common ones include imuran, methotrexate, mycophenolate, and cyclophosphamide. The choice of the medication depends on the organ involved and the severity of the disease. Your rheumatologist will help you find the right medication for your type of lupus.
- New therapies.
- In 2011, the FDA approved belimumab (Benlysta) for the treatment of lupus. Benlysta is different than conventional treatments because it is a biologic agent that targets specific immune cells in the body rather than suppressing the entire immune system. It has minimal side effects. The medication may not be suitable for all lupus patients, but it marks a breakthrough in lupus treatment and paves the way for more effective drug therapies.
- Voclosporine was approved for lupus nephritis in 2021. This oral medication can be used in combination with background immunosuppressants to improve kidney function.
- Saphnelo is an FDA-approved treatment that blocks type 1 interferon (IFN-1) activity, which plays a key role in how lupus attacks your body.
Living with Lupus
Lupus is a chronic illness, and its symptoms can significantly affect your quality of life. Living with lupus can be challenging, frustrating, and stressful. Here are some tips that can help you manage your disease and improve your quality of life.
Learn about Lupus
Understand your disease. The more you know about your disease, the more you feel in control of your health. Talk with your doctors to learn more about your disease and learn the ways you can control the disease and manage your symptoms. Regular communication with your rheumatologist will help to adequately control the disease and identify side effects of lupus medications early. It’s okay to investigate lupus on the internet, but be sure to check with your doctor to verify that what you’ve learned is true.
Change your Lifestyle
Some of the simplest ways to manage lupus are within your control.
- Avoid flares. Sometimes symptoms get worse or “flare.” Some of the warning signs of flare include fever, worsening fatigue, worsening joint pain, and a new skin rash. Early treatment of a flare could help prevent long-term organ damage, so let your doctor know if you are experiencing a flare. Some things that can cause a flare include stress, sun exposure, infection, and missing your medications.
- Avoid sunlight. Exposure to ultraviolet light could lead to flares. Use protective clothing such as a hat and long sleeves. Don’t forget sunscreen.
- Take your medications on schedule. Let your doctor know if you suspect you have side effects from the medications.
- Eat a healthy and well-balanced diet. This will help your overall health and decrease your risk of heart disease.
- Reduce stress. Stress could cause a flare. Ways to manage your stress include meditation, yoga, Tai Chi, and deep-breathing techniques. Talk to your family members, counselors, or your healthcare team to get help with anxiety or depression.
- Get enough sleep. Make resting a priority.
- Stop smoking. This helps reduce your risk of heart disease, which is common in patients with lupus.
- Get help from friends and family. Your family and friends have an important role in helping you deal with the various physical and mental issues that come with the diagnosis of lupus. Keep open communication with your loved ones and ask for help. Belonging to a support group could help you understand lupus and provide you with a sense of community. If you’re not open to the idea of being in a group, try one-on-one counseling.
Prognosis and Complications
Lupus has no cure, but with treatment the disease can be well controlled. The Lupus Foundation of America estimates that 80 to 90 percent of people with lupus won’t have any reduction in lifespan, and, for the vast majority of patients, lupus is not fatal.
Complications, however, may arise, either from the disease itself or from medications used to manage it. These may include:
- Increased risk of cardiovascular disease (heart attacks, stroke)
- Reduced kidney function leading to kidney failure and need for dialysis
- Complications during pregnancy (miscarriage, preterm birth)
- Joint damage due to uncontrolled arthritis
- Increased risk of infection
- Weak bones (osteoporosis)
These complications may be prevented or minimized with adequate control of the disease and close monitoring by your doctor. Regular consultations with your rheumatologist are essential.
Summary
Lupus is a chronic autoimmune disease with a waxing and waning disease course. It is a multisystem disease, affecting many different organs with varying symptoms and signs depending on which organs are involved. Due to its chronic nature, regular consultations with a rheumatologist are important to monitor disease activity and prevent major side effects related to the lupus medications.
Written by Natan Rosenfeld
References
- Lupus facts and statistics
- Risk factors for developing lupus
- Genetic Factors Predisposing to Systemic Lupus Erythematosus and Lupus Nephritis
- Lupus
- Benlysta: What you need to know
- FDA Approves First Oral Therapy Voclosporin for Lupus Nephritis
- Lupus Prognosis and life expectancy
- Lupus Complications and Prognosis
