Lupus, as defined by the Centers for Disease Control and Prevention, is defined as a chronic, autoimmune disease that can damage roughly any part of the body, from the organs to the skin. It’s a disease that is common among women, particularly ones who are in childbearing age. It causes the immune system to malfunction, causing inflammation throughout the body. To date, there is no known cure for lupus; there are only treatments and medication to suppress and manage its symptoms.
Typically, the human body’s immune system has white blood cells tasked with defending the body against foreign intruders, like bacteria, viruses, and other pathogens. When a person has an autoimmune disease, the body’s defensive systems target and attack healthy tissues. These attacks cause inflammation, and can potentially lead to damage throughout the body.
Lupus has afflicted people for a very long time. The first documented case of it ever occurring was in 400 BC when Hippocrates recorded evidence of the disease. The history of lupus is so extensive that its occurrences fall into three periods: Classical, Neoclassical, and Modern.
In the Classical period the name lupus was coined (Latin for “wolf,” after the facial lesions resembling a wolf’s bite), along with the clinical descriptions of lupus vulgaris, lupus profundus, and discoid lupus. The Neoclassical period brought the earliest mention of lupus as a systemic disorder, and the classification of two types of lupus erythematosus: the discoid form and the disseminated or systemic form. This was also the period when the first signs and symptoms of the systemic form of lupus were identified. Finally, it was in the Modern period when Dr. Malcolm Hargraves and his colleagues discovered LE (lupus erythematosus) cells, which are special types of cells that have engulfed the nucleus of another cell. The discovery brought on a new era in immunology and the study of lupus as it is known today as an autoimmune disease. Also, cortisone has been discovered as a treatment, along with immunologic markers associated with the disease.
Lupus is difficult to diagnose because of its myriad of signs and symptoms, the majority of which could be attributed to other diseases. According to experts, its most distinctive sign is a facial rash that resembles butterfly wings across both cheeks. This symptom occurs in many but not all lupus cases. There are many other ways that lupus can affect the body. Among the complications is damage to numerous body systems, including the immune system, cardiovascular system, kidneys, lungs, and nervous system.
The considerable range of lupus’ symptoms and the different effects it might have on the body has led scientists and researchers to identify four different types of lupus:
- Systemic lupus erythematosus (SLE)
This is by far the most common and widely recognized form of lupus. The symptoms that it creates range from mild to severe, but it remains capable of producing severe complications among the different major organ systems. This includes inflammation of the kidneys (lupus nephritis), inflammation of the nervous system and the brain, inflammation in the brain’s blood vessels, and coronary artery disease.
- Cutaneous lupus erythematosus
This form manifests on the skin by causing different types of rashes or lesions. The most common is called discoid rash, which is raised and scaly but not itchy, appearing in the form of disks. Another typical example is the well-known “butterfly rash” that spreads over the nose bridge and across both cheeks. About 10% of people with cutaneous lupus erythematosus will go on to develop systemic lupus.
- Drug-induced lupus erythematosus
This is a lupus-like disease that has been triggered by prescription drugs. It has symptoms similar to that of systemic lupus, but it doesn’t often affect significant organs in the body. Drug-induced lupus erythematosus is associated with drugs such as hydralazine (blood pressure medication), procainamide (a medicine for irregular heart rhythm), and isoniazid (tuberculosis treatment). This form is more common among men than women as they are more often prescribed these drugs.
- Neonatal lupus
As the name suggests, this rare condition affects infants, particularly those of women who have lupus. It’s caused by the mother’s antibodies acting upon the infant even while still in the womb. Once born, the infant might have liver problems, low blood cell counts, or a skin rash. However, this type of lupus is not considered a true form of lupus, and the symptoms may disappear completely after several months and have no lasting effects.
Lupus is a disease that can affect practically any organ in your body. Symptoms can differ significantly between people. A person may not have the distinctive lupus butterfly rash, while another may have it prominently. Furthermore, new symptoms or different symptoms may manifest only as time passes with the disease.
Lupus is a chronic disease, so the symptoms may come and go. The condition occurs in a cycle of flare-ups and remissions. This means that there will be occasions when the symptoms are at their peak and manifest in varying levels from mild to severe. Following proper medication and treatment of the symptoms, the signs will recede for some time. This could be for months or years. But lupus will always remain with the body, and it’s a lifelong cycle of addressing the symptoms and treating them over time. The earliest signs of lupus typically manifest around the teen years to a person’s 30s. Upon the first appearance, symptoms may be mild, severe, sporadic, or continual as well.
These are common signs and symptoms of lupus, though some people with lupus will not have all symptoms.
- Extreme fatigue – Up to 80% of people who have lupus may experience fatigue, sometimes to the point that it becomes debilitating. It’s not clear why so many people who have lupus experience this fatigue, but the level of fatigue they experience depends on different factors. These include the severity of their disease at the time, their medications, their pain levels, and even mental or physical health. Fatigue may also be the sign of an impending flare of lupus.
- Pain – Pain and swelling in joints are another prominent symptom of lupus. Muscle and joint pain cause a feeling of stiffness. Swelling of joints may accompany the pain. People with lupus experience these aches, also called myalgias, as the disease starts to affect and inflame specific muscle groups, referred to as myositis. More than 90% of people who have lupus experience this type of pain during their time with this disease. Over 50% also report pain as a primary symptom.
- Fever – Fevers higher than 100 degrees Fahrenheit are prominent among people with lupus. They experience a reoccurring, low-grade temperature that may also indicate the approach of further symptoms or an onset of a lupus flare. The fever may also be a precursor to inflammation.
- Rashes – As mentioned earlier, a butterfly-shaped rash is one of the most significant indicators that a person may have lupus or might develop systemic lupus later on. At least two-thirds of people with lupus will also get a form of skin condition, and 40 to 70% of these cases will find that exposure to UV light will exacerbate the rash. The rash may appear on constantly sun-exposed areas such as the face, ears, neck, and limbs.
- Chest pain – Lupus may also trigger pain in the chest area as it affects the lining of the lungs. People with lupus may experience pain when breathing deeply as the disease affects the pulmonary system. Lupus may affect the lungs through inflammation of the pleura, which is the lining outside the lungs (pleuritis) or the lining inside the lungs (pneumonitis).
- Photosensitivity – As UV light exacerbates the skin condition of people who have lupus, they also become photosensitive. They are affected by both sunlight and fluorescent light. Excessive exposure becomes a common trigger for both cutaneous lupus and systemic lupus.
- Hair loss – Hair loss can be a side effect of both lupus as well as the medications used to treat it. Nutritional deficiencies, illnesses, and thyroid problems may also contribute to hair loss.
- Headaches – Lupus may also affect the nervous system and the brain. As the nervous system and brain become affected, people with lupus experience headaches. There may also be some memory problems among some sufferers.
- Mouth sores - These ulcers often appear on the roof of the mouth but may appear around gums, inside the cheeks, or the lips.
- Sjogren’s syndrome – Around 10% of people with lupus may experience this secondary autoimmune disease. Sjogren’s syndrome causes the glands that produce tears and saliva to malfunction. This results in dryness of the eyes, the mouth, and the vagina. There may be gritty sensations in the eyes, especially in the morning.
Gastrointestinal problems – Heartburn and gastroesophageal reflux is also another common symptom for lupus. People may develop peptic ulcers due to the medication used in lupus treatment.
From what doctors understand, lupus is caused by a complex mix of genetics, hormones, as well as several environmental factors. In terms of prevalence, the Lupus Foundation of America has estimated that about five million people worldwide are afflicted with the disease or have some form of lupus. Of this number, 1.5 million of them are Americans. There are roughly 16,000 new cases of lupus identified every year.
Various factors may influence whether or not a person may get lupus:
Lupus primarily affects women around childbearing age. However, even men, children, and teenagers could be diagnosed with lupus as well. At least 90% of lupus patients are women. The disease typically develops between the ages of 15 to 44 years old. The first symptoms to appear can be extreme fatigue, pain, cognitive issues, hair loss, and other physical impairments.
Race or Ethnicity
Lupus is particularly prevalent (roughly 2-3 times more common) among women of color compared to Caucasian women. At-risk races and ethnicities include African Americans, Hispanics, Asians, Native Americans, Native Alaskans, Native Hawaiians, and Pacific Islanders. According to research, one in every 537 African American women has lupus. They are also more likely to experience issues in their organ systems as well as a more active disease.
At least 20% of people with lupus may have a parent or a sibling who already has it, and about 5% of them are children born of parents who have the disease. Typically, when people showing lupus symptoms are diagnosed, their family histories are investigated to determine if they have an immediate family member or relative who has lupus or other autoimmune conditions. MHC Genes in particular (MHC Class II and III) are families of genes that are associated with lupus. These genes impact the group of proteins that affect the immune complexes.
Researchers have also found that there may be links between lupus and several common toxins. This includes cigarette smoke, silica, and mercury. There are also concerns about infectious diseases like the Epstein-Barr virus (which causes mononucleosis), the herpes zoster virus (which causes shingles), and cytomegalovirus. There may also be drugs that cause lupus (as mentioned in the drug-induced type of lupus). Finally, ultraviolet light and stress may exacerbate symptoms but have not been directly proven as a cause.
Diagnosis & Testing
It is very difficult to diagnose lupus since it has so many symptoms, and patients have varying complaints. Physicians must take extra care when studying someone’s symptoms as well as their comprehensive medical history. As a result, diagnosing lupus generally takes a longer period of time. There is no single finding that becomes the absolute qualification for a person to have systemic lupus, and therefore doctors generally eliminate all other possible causes of the symptoms. The person must also be monitored over time, with their lab tests and symptoms studied.
Doctors may use different approaches to determine if an individual has lupus:
- Comprehensive medical records – The person’s lab tests, results, signs, symptoms, and responses to medication must be carefully recorded, studied and analyzed for the telltale signs of lupus.
- Laboratory tests:
- Complete blood count measures the number of red blood cells, white blood cells, and platelets. The results may indicate if the person has anemia, which is common in lupus.
- Erythrocyte sedimentation rate determines how fast blood cells settle. If it’s faster than average, it may indicate systemic disease.
- Kidney and liver assessment through blood tests may tell how well the kidney and liver are functioning, as lupus can affect those organs.
- Urinalysis is used to sample urine to determine the red blood cell and protein levels.
- Antinuclear antibody (ANA) test is used to test for the presence of the immune system’s antibodies to see if the immune system is working correctly or not.
- Imaging tests:
- A chest x-ray is used to determine if there are abnormal shadows that indicate inflammation in the lungs.
- Echocardiogram produces sound waves that create images of the heart’s beating. This is used to check for problems in the heart and see if it’s affected by lupus.
Biopsy – Depending on the type of damage already present in the body that might be potentially due to lupus, doctors may take a sample of tissue from inside the body to biopsy and analyze.
- Nonsteroidal anti-inflammatory drugs such as ibuprofen and naproxen sodium are used to treat the pain, swelling, and fever caused by lupus.
- Antimalarial drugs decrease the risk of lupus flare-ups as well. Hydroxychloroquine, which affects the immune system, is commonly prescribed.
- Corticosteroids such as prednisone and steroids like methylprednisolone control severe symptoms of the disease if it has affected the kidneys and brain.
- Immunosuppressants such as azathioprine, mycophenolate mofetil, and methotrexate, suppress the immune system in more severe cases of lupus.
Diet & Lifestyle:
It’s essential for people who have lupus to maintain regular visits to their doctor for checkups, especially if there are signs that symptoms are worsening. The doctor will be able to keep a close eye on the progression of symptoms. People living with lupus also should avoid excessive exposure to sunlight as ultraviolet light can trigger a flare-up. Smoking also increases the risk of cardiovascular disease in lupus patients, which is the number one cause of death among lupus patients, so smoking and secondhand smoke must be avoided.
A healthy diet, with vitamin D and calcium supplements, is recommended for people with lupus. A healthy diet emphasizes fruits, vegetables, and whole grains. Meanwhile, supplements may be used to keep bones healthy to avoid osteoporosis.
Therapy and Procedures:
Many treatments and procedures for lupus depend on drugs and medication as the focus is on treating symptoms and reducing inflammation. There is no cure for lupus available at this time, but medication can be prescribed by doctors, depending on the severity of the flare-ups and the disease. A healthcare provider can provide the right prescriptions and adjust these treatments over time as symptoms change or develop.
Research & Advancement
Therapies and treatments in lupus have advanced significantly. The first therapies in the late 19th century involved the use of quinine, and then later, the use of salicylates. In the mid-20th century, cortisone and corticosteroids were introduced. As of today, corticosteroids are the primary therapy for nearly all individuals who have the disease.
Today, multiple medical organizations such as the Lupus Research Alliance continue to look for better treatment and medication for patients. They also continue to look into the probable causes or risk factors in the disease.
Clinical trials are a core part of the research process to ensure that new treatments are both effective and safe. In addition to clinical trials, researchers are increasingly making use of real-world evidence, which is information that is collected in the normal course of care for people with these cancers.
Reviewing medical records and patient histories is particularly important for lupus. Because lupus takes time to diagnose, comprehensive medical histories are necessary to consider the symptoms and to make a correct diagnosis. These comprehensive patient medical histories of what it’s like to live with lupus provide researchers with clues of what to look into or what to target next.
Can people die of lupus?
Most people have a normal life expectancy with lupus, thanks to improved methods of diagnosis, as well as developments in treatment and medication. Around 10-15% of people who have lupus may die prematurely due to the complications of the disease.
Is lupus contagious?
No, many possible risk factors increase the chances of a person developing lupus, but you cannot catch lupus from someone else.
Can I still get pregnant even with lupus?
Women with lupus can still get pregnant and give birth, but this is considered a high-risk pregnancy, as there is a risk for complications such as lupus flares, preeclampsia, high blood pressure, and diabetes.
Is it preventable?
For most varieties, the disease is not preventable, except for drug-induced lupus. However, to reduce the likelihood of developing lupus, avoiding direct sunlight, practicing stress management, and infection prevention techniques, as well as plenty of rest, will help.
Part of understanding and finding better treatments for lupus is through the careful study of patient records and tests. PicnicHealth makes it easier to get your complete medical records to aid in managing lupus. Visit PicnicHealth.com/lupus to sign up for a free PicnicHealth account if you have lupus.