What is Systemic Lupus Erythematosus (SLE)?
Systemic lupus erythematosus (SLE) is an autoimmune disease whereby the body’s immune system attacks itself causing damage.
Our immune system, when functioning normally, creates antibodies to protect us from viruses, bacteria and germs. These antibodies also give the body immunity against further attacks from microorganisms which are unfamiliar.
When the body becomes autoimmune, the immune system cannot tell the difference between harmful invaders and healthy tissue in the body. It creates auto-antibodies which attack and destroy healthy tissue. (1)
Systemic lupus erythematosus (SLE) can affect many areas of the body including: joints, skin, kidneys, central nervous system, lungs and the system which forms blood cells (hematopoietic system).
It is thought to affect anywhere between 322,000 to 1.5 million people in the United States. The many signs and symptoms of SLE can resemble those of other disorders, making diagnosis difficult. (2)
The symptoms include:
Extreme tiredness and lethargy could be one of the first symptoms noticed when suffering from systemic lupus erythematosus (SLE).
Even after a good night’s sleep a person may not feel refreshed and this feeling persists throughout the day. Fatigue does not go away easily and can last for weeks. (3, 4)
Malaise describes a general feeling of illness and lack of energy, or an uncomfortable feeling that something is wrong but you don’t know what. This symptom is associated with SLE. (5)
A fever is classified as an increase in core temperature above what is normal for an individual. It is usually caused by disease and is a symptom of systemic lupus erythematosus (SLE). (6, 7)
Loss of Appetite and Weight Loss
Loss of appetite and weight loss for no apparent reason are also associated with this condition. (8)
Joint pain and or muscle discomfort and weakness are further signs of SLE. These often occur in the same joints on either side of the body. (9)
Systemic lupus erythematosus (SLE) can result in skin rashes and photosensitivity. In fact 66 percent of people with this condition will develop an associated skin disease.
There are three types of skin rash that manifest in lupus. These are:
Chronic Cutaneous Lupus (Discoid Lupus)
This skin condition is characterized by round, disc shaped sores, generally on the face and scalp. They can however also occur elsewhere on the body.
The rash will be thick, red and scaly but usually does not hurt or itch. Over a period of time, scarring and skin discoloration (in the form of dark or light colored areas) can appear.
When these discoid lesions are on the scalp they can cause hair loss which might, on occasion, be permanent.
It is also possible for cancer to develop in persistent discoid sores.
This type of skin rash can be very sensitive to sunlight and fluorescent lighting (photosensitive). Consequently protection from these light sources is important. (10)
Subacute Cutaneous Lupus
The sores in this type of skin rash are red and shaped like a ring, or red and scaly, with defined edges.
They generally appear on skin areas that are exposed to the sun, like arms, shoulders and neck, as well as other parts of the body. These lesions tend not to scar or itch, they are however photosensitive. (11)
Acute Cutaneous Lupus
Acute cutaneous lupus rash tends to occur when systemic lupus erythematosus (SLE) is active. This manifests as a flat red rash across the bridge of the nose and the cheeks. It is sometimes referred to as the “butterfly rash” due to its shape.
While it is typically seen on the face, this rash can appear on other parts of the body like the arms, legs and torso.
Once again this is very photosensitive and might leave skin discoloration. (12)
Other Skin Issues
As well as skin rashes, there are other issues which affect the skin associated with this condition.
Calcium deposits can build up under the skin, appearing as yellowish or white lumps (calcinosis). These might leak a white fluid and can be painful.
Inflammation of blood vessels in the skin can be seen as small reddish-purple spots or lumps, often on the lower legs. Called cutaneous vasculitis lesions they can on occasion develop into ulcers.
These lesions can also appear as raised sores, spots, or small reddish lines on the fingertips or around the fingernails.
Cutaneous vasculitis can lead to significant damage of the skin tissue. Dead skin patches might appear as small black spots or as sores near the fingertips or on the toes. The result of this is loss of blood supply to these soft tissues leading to gangrene.
Raynaud’s phenomenon is a condition associated with lupus where inflammation of blood vessels and nerves affects the fingertips and toes. Particularly in cold weather, they turn blue, white or red and are numb or painful.
Abnormal blood flow through blood vessels in the skin can cause a bluish mottled appearance, often on the legs.
Petechiae are very small red spots on the skin, generally again on the lower legs. They are caused by a low number of clotting agents (platelets) in the blood, leading to bleeding under the skin. (13)
Ulcers can appear in the mouth or inside the nose, occasionally in soft vaginal tissue, and tend to be painless. (14)
Systemic lupus erythematosus (SLE) can cause hair loss, which is usually temporary. The hair can become weak and break easily, however new hair can grow to replace what is lost. (15)
Swelling will often be seen in the lower extremities, the hands or the areas around the eyes. (16)
Chest pain is not uncommon when taking a deep breath. (17)
One of the effects of systemic lupus erythematosus (SLE) is kidney damage. Referred to as lupus nephritis, a sample of kidney tissue can indicate how far the disease has progressed.
The kidney contains filters called glomeruli, which are blood vessels that filter waste from the blood. Cells (mesangial cells) help the glomeruli work efficiently. (18)
The World Health Organization issued classification of this disease mainly based on the function of these two factors. (19)
Glomeruli appear normal and there is minimal mesangial damage.
Mesangial cells are mildly impaired and the glomeruli become inflamed.
Mild or moderate changes in the mesangial cells leading to active and chronic scarring. Glomerular tissue death in a specific area can happen as a result of inflammation.
Widespread inflammation of the kidney and glomeruli. Severe changes in the mesangial cells leading to active and chronic scarring.
The immune system is now attacking the glomeruli causing widespread scarring. This is called membranous lupus nephritis.
At this stage the scarring of the kidneys is advanced and their function impaired. (20)
Systemic lupus erythematosus (SLE) is a disease which can be active and then go into remission. The aim of treatment is to manage associated life threatening conditions, minimize flare ups and control and manage day to day symptoms.
Treatments can be variable and will be tailored to best meet the needs of each individual.
Many of the treatments are immunosuppressive in nature and include medication to treat associated symptoms. These include hydroxychloroquine for joint pain, fatigue and skin conditions, and non-steroidal anti-inflammatories for joint and muscle pain.
Oral steroids or steroid injections could be administered for both mild and severe symptoms.
Kidney disease (lupus nephritis) is a common complication of this condition and will often need treatment. It is worth noting that many of the drugs used to treat this might have side effects.
Immunosuppressive treatments, including cyclophosphamide and azathioprine, might be used when the central nervous system is affected. (21)
People who have photosensitive symptoms might benefit from limiting their exposure to either sunlight or fluorescent lighting. Wearing protective clothing and high factor sunscreens can help. (22)
What is systemic lupus erythematosus (SLE)? Systemic lupus erythematosus (SLE) is an autoimmune disease whereby the body’s immune system attacks itself causing damage.
What are the signs of systemic lupus erythematosus (SLE)? The signs of this condition can involve many parts of the body. They include joint, muscle and chest pain, fatigue, skin conditions and ulcers.
How do you develop systemic lupus erythematosus (SLE)? The precise cause of this condition is not known, however it is thought that lupus is a genetic condition. Factors like exposure to sunlight, drugs and infections might trigger activation of the disease in predisposed individuals. (23)
How are your diagnosed for systemic lupus erythematosus (SLE)? Diagnosis of this condition can pose a problem for medical professionals. It affects so many parts of the body and can be mistaken for other conditions. There are set criteria surrounding symptoms that enable diagnosis. (24)
What is the best treatment for systemic lupus erythematosus (SLE)? Treatment for this condition is varied and will be tailored to each individual’s needs.
What are the long term complications of systemic lupus erythematosus (SLE)? This disease can affect many organs in the body including the kidneys, heart and central nervous system. While it can get worse and then get better, over time gradual deterioration can be life threatening.
Is systemic lupus erythematosus (SLE) considered a disability? This condition is listed as a disability, however there are caveats. It must affect at least two body systems or organs and be accompanied by other consistent symptoms. (25)
Is there a cure for systemic lupus erythematosus (SLE)? While we have mentioned that this condition can go into remission, there is no cure. (26)
Is systemic lupus erythematosus (SLE) life threatening? This condition can be managed, but the long term prognosis is not good and it is life threatening. (27)
Systemic lupus erythematosus (SLE) is a degenerative autoimmune disease whereby the body’s immune system attacks itself causing damage.
The condition can pose a problem for doctors to diagnose. However, once identified there are treatments available which help manage the symptoms.