systemic lupus erythematosus Alternative Names
What is going on in the body?
Systemic lupus erythematosus (SLE) is an "autoimmune" disease that affects many parts of the body and can be serious, even fatal. A person's defence system, which normally attacks "foreign" bodies, begins to attack the body itself.
Antibodies are protein molecules that the body produces to help fight infection and cancer. An antigen is any substance that causes the production of antibodies and combines specifically with them. Normally, the body produces antibodies that only attach themselves to "foreign" antigens from outside the body, like viruses or bacteria. The antibody and antigen fit together like a lock and key. This process disables the virus or bacterium and protects the body.
In lupus, the body produces a number of "autoantibodies" that attack various parts of cells within the person's own body. These combinations of antibody and antigen are deposited in various tissues and organs throughout the body. These deposits cause swelling and damage to blood vessels in many organs. These include:
What are the signs and symptoms of the disease?
- brain and nervous system
- digestive system
- the eyes
- a variety of joints and muscles.
Since lupus affects so many different parts of the body, the disease has many signs and symptoms. These are best listed by organ system.
Symptoms that affect the whole body:
Symptoms that affect the skin:
- loss of appetite
- weight loss
Symptoms and signs that affect the muscles, bones, and joints:
- a red "butterfly rash" on the face, which extends over the cheeks and bridge of the nose in the shape of a butterfly
- red, raised rashes in areas exposed to the sun
- sensitivity to the sun
- loss of scalp hair
- ulcers of the mouth, nose, or vagina
Symptoms of kidney involvement:
- muscle pains and weakness
- joint pains, especially in the hands, wrists, and knees
- hand deformities
- a loss of blood supply to bone, especially hips and shoulders
Symptoms affecting the nervous system:
- protein in the urine
- blood in the urine
- white blood cells in the urine
- decrease in kidney function or kidney failure
Signs involving the blood:
- memory loss, problems with concentration
- headaches, including migraines
- bleeding in the brain
- loss of co-ordination
- a sense of numbness, pain, or "pins and needles" in the hands and feet
- problems with the major nerves in the head and face
Signs involving the heart:
- low red blood cell count
- low white blood cell count
- low blood platelet count
- problems with both blood clotting and bleeding
- enlargement of the lymph nodes ("glands")
Symptoms and signs involving the lungs:
- inflammation of the lining of the heart
- fluid collection in the sac around the heart
- inflammation and infection in the heart
- heart attack
- heart valve problems
Symptoms and signs affecting the digestive system:
- shortness of breath
- cough and coughing up blood
- inflammation of the lining of the lungs
- inflammation in the lung
- fluid in the lung
Symptoms and signs affecting the eye:
- pain in the abdomen
- nausea, vomiting, diarrhoea, loss of appetite
- swelling of the pancreas
- enlargement of the liver and abnormal liver function tests
- rectal bleeding due to ulcerations
- intestinal perforation due to inflammation of blood vessels
What are the causes and risks of the disease?
- conjunctivitis, which is inflammation of the outer lining of the eye
- damage to the retina and blindness
The cause of lupus is unknown. Lupus tends to run in families. A few drugs are known to cause the disease. These include procainamide, hydralazine, isoniazid, and chlorpromazine. Events that may trigger the disease include infection, stress, exposure to toxins, and sunlight.
Lupus has a prevalence in about 1 in 4000 of the population. It is 9 times more common in women than men. It is 9 times more common in Afro-Caribbeans and Asians than in Caucasian patients. The peolpe most affected are women of childbearing age.
The health risks of lupus are significant. It can cause serious problems in the kidney, blood, brain, heart, lung, eye, and digestive tract. Lupus can be fatal. The most common causes of death are kidney failure and heart attack.
What can be done to prevent the disease?
There are no known ways to prevent lupus.
How is the disease diagnosed?
A complete medical history and physical examination are important tools for diagnosing lupus. Blood tests also play an important role. Most patients have a positive antinuclear antibody (ANA) test. Other tests are often used to help in diagnosing lupus. These include a chest x-ray, electrocardiogram, CT scan, MRI scan, spinal tap, or kidney biopsy.
What are the long-term effects of the disease?
Lupus is a long-term disease that flares up, then quiets down, then flares up again. Persons with lupus often have a significant decrease in the quality of daily life. This is due to joint and muscle pain, fatigue, loss of appetite, and memory loss or trouble concentrating. Many complications of lupus put the person at risk. Many of the medications used to treat the disease also have significant side effects. Lupus can be fatal, often due to kidney failure, infections, or heart attack.
What are the risks to others?
There is no evidence that lupus is a contagious disease that can be passed from one person to another. However, lupus does tend to run in families. Pregnant patients with lupus have an increased risk of miscarriages, stillbirths, and premature infants.
What are the treatments for the disease?
Though there is no cure for SLE, there are things that can help prevent flare-ups. Persons with lupus should avoid intense sun exposure. Liberal use of sunscreens, long-sleeved shirts, and hats are helpful. Since infections are common, persons with lupus should seek prompt evaluation of fevers. A yearly flu Injection is recommended. If the spleen is removed, the person should receive the pneumococcal vaccine. Lupus can take an emotional toll on patients. A growing number of support groups provide individuals emotional support.
A number of medications are used to treat SLE.
What are the side effects of the treatments?
- Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen or naproxen, are often used to treat fever, joint aches, and muscle aches.
- Anti-malarial type medications, such as hydroxychloroquine, are often used to treat skin problems and arthritis.
- Corticosteroids, such as methylprednisolone, are useful in cases of active disease and can be life saving.
- Prednisone can cause prompt and complete resolution of symptoms in acute flare-ups. Once a flare-up is controlled, the doctor usually tapers down to the minimum dose possible. Such tapering can cause another flare-up of the disease.
- Powerful cytoxic medications which kill cells are often used to treat nephritis, a common and serious kidney problem in SLE. Sometimes these medications can decrease flare-ups and help reduce the need for high-dose corticosteroids.
- Patients with end-stage kidney disease may benefit from kidney dialysis or transplantation.
The medications used to treat lupus have significant side effects. Unfortunately, some of these side effects can mimic the symptoms of the disease itself.
NSAIDs can cause stomach irritation, headaches, confusion, itching, and decrease in kidney function.
Antimalarial medications can cause rash, muscle weakness, nerve problems, retinal toxicity, and birth defects.
Corticosteroids have a host of possible side effects. These include weight gain, high blood pressure, acne, easy bruising, bone loss, increased blood sugar, cataracts, glaucoma, and muscle weakness.
The cytotoxic medications can cause bone marrow suppression, bladder toxicity, gonadal failure, and an increased risk of cancer.
What happens after treatment for the disease?
Lupus is a chronic disease that fluctuates over time. The best way to manage it is by active participation in the process by the patient and the doctor.
How is the disease monitored?
An ongoing history and physical examinations help the doctor monitor the activity of the disease and determine possible complications. A variety of blood tests help in the monitoring of the disease as well.
Author: Minot Cleveland, MD
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Potential conflict of interest information for reviewers available on request