Systemic lupus erythematosus (SLE), also known simply as lupus, is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.
SLE is more common in women than men and it may occur at any age. However, it appears most often in people between the ages of 15 and 44. The disease affects African Americans and Asians more often than people from other races.
SLE may affect various parts of the body, but it most often manifests in the skin, joints, blood, and kidneys.
There are several different forms of lupus. SLE is the most common type and is the type of lupus that can lead to serious systemic complications. Other forms of lupus include:
- Cutaneous lupus erythematosus - Refers to lupus that is confined to the skin and does not affect other parts of the body. About 10% of people with this type of lupus go on to develop SLE.
- Discoid lupus erythematosus - A type of cutaneous lupus that produces a potentially scarring disc-shaped rash on the face, scalp, or ears.
- Drug-induced lupus - A temporary and mild form of lupus caused by certain prescription medications. They include some types of high blood pressure drugs (such as hydralazine, ACE inhibitors, and calcium channel blockers) and diuretics (hydrochlorothiazide). Symptoms resolve once the medication is stopped.
- Neonatal lupus - A rare condition that sometimes affects infants born to mothers who have SLE. Babies with neonatal lupus are born with skin rash, liver problems, and low blood counts and may develop heart problems.
SLE has a wide range of symptoms with the most common being joint pain, skin rash and fever. Many patients with SLE have "flares," in which symptoms suddenly worsen and then settle down for long periods of time.
Other common symptoms include:
- Chest pain when taking a deep breath
- Fever with no other cause
- General discomfort, uneasiness, or ill feeling (malaise)
- Hair loss
- Mouth sores
- Sensitivity to sunlight
- Skin rash: A "butterfly" rash appears in about half the people with SLE. The rash is most often seen over the cheeks and bridge of the nose.
- Swollen lymph nodes
Other symptoms depend on which part of the body is affected:
- Brain and nervous system: Headaches, numbness, tingling, seizures, vision problems, and personality changes
- Digestive tract: Abdominal pain, nausea, and vomiting
- Heart: Abnormal heart rhythms (arrhythmias)
- Lung: Coughing up blood and difficulty breathing
- Skin: Patchy skin color and fingers that change color when cold (Raynaud phenomenon)
- Kidney: Swelling in the legs, weight gain
Some people have only skin symptoms. This is called discoid lupus.
To be diagnosed with lupus, you must have 4 out of 11 common signs of the disease. Nearly all people with lupus have a positive test for antinuclear antibody (ANA). However, having a positive ANA alone does not mean you have lupus.
The health care provider will do a complete physical exam. You may have a rash, arthritis, or edema in the ankles. There may be an abnormal sound called a heart friction rub or pleural friction rub. Your provider will also do a nervous system exam.
Tests used to diagnose SLE may include:
- Antinuclear antibody (ANA)
- CBC with differential
- Chest x-ray
- Serum creatinine
You may also have other tests to learn more about your condition. Some of these are:
- Antinuclear antibody (ANA) panel
- Complement components (C3 and C4)
- Coombs test - direct
- ESR and CRP
- Kidney function blood tests
- Liver function blood tests
- Rheumatoid factor
- Antiphospholipid antibodies and lupus anticoagulant test
- Kidney biopsy
There is no cure for SLE. The goal of treatment is to control symptoms. Severe symptoms that involve the heart, lungs, kidneys, and other organs often need treatment from specialists.
Mild forms of the disease may be treated with:
- NSAIDs for joint symptoms and pleurisy
- Low doses of corticosteroids, such as prednisone, for skin and arthritis symptoms
- Corticosteroid creams for skin rashes
- Hydroxychloroquine, a drug also used to treat malaria
- Belimumab, a biologic drug, may be helpful in some people
Treatments for more severe SLE may include:
- High-dose corticosteroids
- Immunosuppressive drugs (drugs which dampen or suppress the immune system). These medicines are used if you do not get better with corticosteroids, or if your symptoms get worse when you stop taking them
- Blood thinners, such as Coumadin, for clotting disorders
Living with Lupus
Patients can make lifestyle changes to help cope with SLE. These include:
- Avoid excessive sunlight exposure, and wear sunscreen (ultraviolet light is one of the main triggers of flares).
- Get plenty of rest (fatigue is another common SLE symptom and flare trigger).
- Engage in regular light-to-moderate exercise to help fight fatigue and heart disease, and to keep joints flexible.
- Do not smoke and avoid exposure to second-hand tobacco smoke.