There are approximately 5 million individuals who are affected worldwide and 16,000 new cases reported annually. In America, 1.5 million Americans have some form of lupus and many are of African American or Asian descent. It is more prevalent in women of childbearing age, but men, children and teenagers can develop it too. The most common age range is 15 to 44 years old. Most affected individuals have another autoimmune disease.

Many affected individuals with SLE have family members with autoimmune diseases. There are also a combination of factors that can cause SLE: hormones, genetics, and environmental triggers.

Hormones are required to regulate and send messages to other parts of the body. Women have more estrogen than men. and estrogen enhances the immune system. This is thought to be one reason why autoimmune diseases are more prevalent in women.

Genetics also play a role in lupus. There are many genes that have been identified and related to the development of lupus. Major histocompatibility complex (MHC) genes class II and III are known to be associated with lupus. These genes help regulate immune responses to foreign invaders. Lupus patients have low complement proteins. Other genes that play a role in complement protein receptors and antibody receptors are associated with lupus as well. Genetic studies also implicate tumor necrosis factor-alpha and interleukin-10. Genes that code for Fcy-receptors are associated with lupus nephritis in the kidneys.

Environmental factors have been linked to lupus. Toxins, such as cigarette smoke, silica, and mercury, and infectious diseases, such as infectious mononucleosis, shingles and cytomegalovirus (CMV) may also be contributing factors.

Drug induced SLE is caused by these drugs: procainamide, quinidine, minocycline, hydralazine, isoniazid, methyldopa, propylthiouracil, methimazole, terbinafine, and anti-TNF agents.

SLE can range from mild to severe symptoms. The symptoms can come and go, and affects different and several organs. The most common symptoms of SLE are:

• Butterfly-shaped rash on the cheeks and nose

• Headaches or migraines

• Painful and swollen joints

• Inflammation

• Fever

• Extreme fatigue or tiredness

• Swelling of the feet, hands, legs, and arms

• Puffy eyes or swollen eyes

• Anemia

• Chest pains

• Photosensitivity

• Hair loss

• Blood clots

• Raynaud’s syndrome or the fingers turning white or blue

• Ulcers in the mouth or nose

Lupus is commonly manifested as arthritis, but lupus nephritis (lupus in the kidney) occurs in half of affected individuals.

An affected individual is diagnosed with lupus through a clinical exam by a doctor. Lupus cannot be diagnosed based on lab tests alone. The doctor must assess the individual’s symptoms, lab tests, personal medical history, and family medical history.

In general, a diagnosis of lupus requires at least four of the following symptoms or characteristics:

• Butterfly rash

• Skin rash

• Photosensitivity

• Mouth or nose ulcers

• Arthritis

• Cardio-pulmonary involvement -- inflammation around the heart and/or lungs

• Neurologic disorder – seizure or psychosis

• Kidney disorder

• Hematologic (blood) disorder

• Immunologic disorder
• Antinuclear antibodies (ANA) positive

The healthcare provider can give affected individuals some lab tests to determine if the person has SLE or another condition with similar symptoms. There are several things in the body that can change if the person has lupus. There can be signs of swelling/inflammation indicating that the immune system is overworking. Complete blood cell count (CBC), autoimmune antibody tests, blood clotting analysis and complement tests are commonly performed.  Specifically, positive antinuclear antibody (ANA), positive anti-single stranded DNA (anti-ssDNA) positive anti-double stranded DNA (anti-dsDNA), positive anti Sm, positive antiphospholipid antibodies, low complement (C3, C4, CH50), and elevated acute phase reactants (ESR, CRP) are detected.  Other tests can be performed to assess organ health, such as kidney function or urine tests, or tissue biopsies.

Affected patients may consult a team of healthcare professionals--  doctors, nurses, physical therapists and other specialists. The autoimmune disorder ranges from mild to life threatening disease. However, with appropriate treatment, most lupus patients live full lives.

The focus of lupus treatment is symptom management and improving quality of life because there is no cure. Comprehensive lupus care prevents the immune system from attacking the body, protecting organs from damage by the condition or drug treatment.  

Non-drug treatments such as rest, exercise and smoking cessation are highly recommended. Affected individuals should avoid prolonged exposure to the sun and protect their skin with sunscreen.

Most affected individuals with mild to moderate symptoms can be managed with a Non-Steroidal Anti-Inflammatory Drug (NSAIDs), such as ibuprofen or naproxen. These medications are available by prescription or over-the-counter. Doses should be consulted with a doctor because NSAIDs can affect the heart, the kidney, and the gastrointestinal tract when dosed inappropriately.

Steroids can be used, but long term use is not recommended. Affected individuals should use a combination of other drugs to decrease exposure to steroids. Steroids can have many side effects, such as increased blood pressure, increased blood sugar, weight gain, osteoporosis, fluid retention, and mood changes. The use of steroids start at a high dose and require a gradual decrease to a lower maintenance dose.

Anti-malarial agents are commonly used in SLE. They can reduce inflammation, pain, blood flow, fatigue, and fever. Hydroxychloroquine is preferred because it has the least side effects. Some side effects include headache, nausea, upset stomach, and vision changes at high doses.

Cytotoxic agents are reserved for individuals that have severe SLE with a lot of flare-ups. Mycophenolate mofetil (CellCept) is the most company cytotoxic agents used in SLE, others are cyclophosphamide and azathioprine, and cyclosporine.

Intravenous biologic infusions, such as with Benlysta, can be used in individuals with failed oral drug therapy.

It is important for affected individuals to take measures to manage and prevent flare-ups. Awareness of medication side effects are also important. If he/she is experiencing side effects, it is best to let the doctor know so that the medication can be changed or decreased in dose. Many SLE individuals state that the flare-ups, pain and fatigue can impact daily life.

1. Lupus Foundation of America. https://www.lupus.org/. Accessed February 25, 2019.

2. Lupus LA. http://lupusla.org. Accessed March 5, 2019.

3. The Johns Hopkins Lupus Center. www.hopkinslupus.org. Accessed March 1, 2019.