WHAT IS LUPUS

Lupus is a chronic, autoimmune disease caused when the immune system attacks its own tissues. It can affect the joints, skin, kidneys, blood cells, brain, heart, and lungs. Symptoms vary but common ones include fatigue, joint pain, rash, and fever. While there's no cure for lupus, current treatments focus on improving quality of life through controlling symptoms and minimizing flare-ups. It is a relatively common disease, affecting an estimated 1.5 million Americans. A majority of these sufferers are adult women between the ages of 19 and 60.  

Types of Lupus

There are several different types of lupus, including Systemic Lupus Erythematosus (SLE), Discoid lupus Erythematosus, Cutaneous Lupus, Neonatal Lupus, and Drug-Induced Lupus. When people use the term “lupus,” they are usually referring to SLE.

  • Systemic Lupus Erythematosus (SLE): so-named because it affects many different organ systems in the body. It is marked by chronic inflammation, especially of the kidneys, joints, and skin. The cardiovascular and nervous systems can also be affected. 
  • Cutaneous Lupus: also referred to as lupus limited to the skin. Three types of skin lupus exist: chronic cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and tumid lupus. A skin biopsy is usually obtained to diagnose skin lupus. 
  • Drug-Induced Lupus: certain drugs can cause lupus-like symptoms in people who do not have SLE. However, this form of lupus is temporary and usually subsides within months of the time that the medication is stopped. Medications known to induce lupus-like symptoms in some individuals include the blood pressure medications hydralazine and methyldopa, a heart medication called procainamide, and a drug called D-penicillamine, which is used in cases of metal poisoning. Other causes of drug-induced lupus include minocycline (used to treat acne) and anti-TNF (used to treat rheumatoid arthritis).
  • Neonatal Lupus: a type of lupus that affects babies of women with certain autoantibodies. About 1 in 1000 perfectly healthy women possesses either these antibodies and a mother who gives birth to a child with neonatal lupus may not have lupus herself. In total, only about 40% of women bearing children with neonatal lupus actually have lupus. Usually neonatal lupus involves only the baby’s skin and subsides on its own, even without treatment. 

To learn more visit Johns Hopkins Lupus Center.

LUPUS SYMPTOMS

Everyone reacts to Lupus differently. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus have mild disease characterized by episodes—called flares—when signs and symptoms get worse for a while, then improve or even disappear completely for a time.

The signs and symptoms of lupus experienced will depend on which body systems are affected by the disease. The most common signs and symptoms include:

  • Fatigue and fever
  • Joint pain, stiffness and swelling
  • Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose
  • Skin lesions that appear or worsen with sun exposure (photosensitivity)
  • Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud's Disease)
  • Shortness of breath
  • Chest pain
  • Dry eyes
  • Headaches, confusion and memory loss

Many of these symptoms occur in other illnesses. In fact, lupus is sometimes called "the great imitator" because its symptoms are often like the symptoms of rheumatoid arthritis, blood disorders, fibromyalgia, diabetes, thyroid problems, Lyme disease, and a number of heart, lung, muscle, and bone diseases.

Learn more at the Lupus Foundation of America.

A view of skin affected by Lupus. 

DIAGNOSIS

Lupus is complex and is often difficult to diagnose, as a result there is no single laboratory test that can determine if a person has lupus. To complicate matters, many symptoms of lupus are similar to those of other diseases, and can come and go over weeks and months. It can often take years for a diagnosis to be made. Doctors use the American College of Rheumatology's “Eleven Criteria of Lupus” to help make—or exclude—a diagnosis of lupus. Typically, four or more of the following criteria must be present to make a diagnosis of systemic lupus. The “Eleven Criteria” can be broken down as follows:

  1. Malar rash: butterfly-shaped rash across cheeks and nose
  2. Discoid (skin) rash: raised red patches
  3. Photosensitivity: skin rash as result of unusual reaction to sunlight
  4. Mouth or nose ulcers: usually painless
  5. Arthritis (nonerosive) in two or more joints, along with tenderness, swelling, or effusion. With nonerosive arthritis, the bones around joints don’t get destroyed.
  6. Cardio-pulmonary involvement: inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
  7. Neurologic disorder: seizures and/or psychosis
  8. Renal (kidney) disorder: excessive protein in the urine, or cellular casts in the urine
  9. Hematologic (blood) disorder: hemolytic anemia, low white blood cell count, or low platelet count
  10. Immunologic disorder: antibodies to double stranded DNA, antibodies to Sm, or antibodies to cardiolipin
  11. Antinuclear antibodies (ANA): a positive test in the absence of drugs known to induce it.

Learn more at the Lupus Research Alliance.

TREATMENT & MEDICATIONS

Due to its complex nature, a variety of treatments are often recommended for treating lupus. While there is no cure, the following treatments are suggested for managing symptoms: 

Anti-Inflammatories

Anti-inflammatory medications help to relieve many of the symptoms of lupus by reducing inflammation and pain. Anti-inflammatories are the most common drugs used to treat lupus, particularly symptoms such as fever, arthritis or pleurisy, which generally improve within several days of beginning treatment.

  • Aspirin
  • Acetaminophen (Tylenol®)
  • Non-steroidal anti-inflammatory drugs (NSAIDs)

Corticosteroids

Corticosteroids (also known as glucocorticoids, cortisone or steroids) are synthetic (man-made) prescription drugs designed to work like the body’s naturally occurring hormones produced by the adrenal glands, in particular cortisol. Cortisol helps regulate blood pressure and the immune system and it is the body’s most potent anti-inflammatory hormone. 

Steroids

Steroid medications work quickly to decrease the swelling, warmth, tenderness and pain that are associated with inflammation. They do this by lessening the immune system’s response. Prednisone is the most commonly prescribed steroid for lupus. Prednisolone and methylprednisolone are similar to prednisone. 

Antimalarials

Antimalarials are prescription drugs used in combination with steroids and other medications, in part to reduce the dose required of the other drugs. Antimalarials are most often prescribed for skin rashes, mouth ulcers and joint pain, but also can be effective in mild forms of lupus where inflammation and blood clotting are a concern. Antimalarials improve lupus by decreasing autoantibody production, protecting against the damaging effects of ultraviolet light from the sun and other sources and improving skin lesions.

Immunosuppressives (Immune Modulators)

Immunosuppressive medications are prescription drugs used to control inflammation and the overactive immune system, especially when steroids have been unable to bring lupus symptoms under control, or when a person cannot tolerate high doses of steroids. However, there can be serious side effects from these drugs. If you are being treated with immunosuppressives, you should be carefully monitored by your physician. Immunosuppressive drugs reduce your body’s ability to fight off infections and increase the chances that you could develop viral infections such as shingles (chicken pox or herpes zoster). It is extremely important that you pay attention to even the smallest cut or wound, and let your doctor know if any sign of infection begins, such as redness, swelling, tenderness or pain. These drugs may also increase your risk for developing certain types of cancer.

Anticoagulants

Because blood clots can be a life-threatening symptom of lupus, these drugs thin the blood to prevent it from clotting too easily. Anticoagulant medications include low-dose aspirin and prescription heparin and warfarin. In particular, if you are being treated with warfarin, you must be monitored by your doctor to be sure your blood does not become too thin. Anticoagulant therapy may be lifelong in some people with lupus. Very recent research shows that people’s genetic makeup may influence how they respond to warfarin; specifically, that people with variations in two genes may need lower warfarin doses due to differences in how the body breaks down (metabolizes) warfarin and regulates the ability of warfarin to prevent blood from clotting. 

Monoclonal antibodies (mAbs)

Benlysta® was developed to disrupt activation of B lymphocytes by interfering with BLyS, a protein required for B cell activity. Benlysta is the first and only drug specifically developed for and approved to treat lupus.

Repository Corticotropin Injection (H.P. Acthar Gel)

Acthar® contains a naturally occurring, highly purified hormone called ACTH, which stands for adrenocorticotropic (a-DRE-no-cor-ti-co-TRO-pic) hormone. One way Acthar is thought to work is by helping your body produce its own natural steroid hormones, such as cortisol. These hormones may assist your immune system by helping your body defend itself against inflammation.

Learn more at the Lupus Foundation of America

SUPPORT FOR LUPUS

The Lupus Foundation of America is the only national force devoted to solving the mystery of lupus, one of the world’s cruelest, most unpredictable, and devastating diseases, while giving caring support to those who suffer from its brutal impact. They envision a life free of lupus. The Lupus Foundation of America implements programs of research, education and advocacy in order to help solve the cruel mystery of lupus.

The Alliance for Lupus Research (ALR) is a national voluntary health organization based in New York City that was founded in 1999 and is chaired by Robert Wood Johnson IV, a member of the founding family of Johnson & Johnson. By committing millions of dollars to significant lupus research every year and holding nationwide lupus walks to secure additional funding, the ALR is proactively battling lupus.