Systemic lupus erythematosus: Frequency
In Europe, Systemic Lupus Erythematosus (SLE disease) affects approximately 25 to 68 per 100,000 people. Women are about ten times more likely to develop this autoimmune disease than men, and it usually occurs during childbearing years. SLE often appears following pregnancy.
The incidence of systemic lupus erythematosus varies among different ethnic groups. For example, Africans and Asians are affected significantly more often than Europeans.
Systemic lupus erythematosus: symptoms
Systemic lupus erythematosus can cause symptoms in various organs and organ systems. For example, blood vessels, skin, hair, lungs, heart, kidneys, and/or joints may be affected. In addition, the course of the disease can vary greatly from person to person. Accordingly, the clinical picture of SLE is varied.
General Lupus Symptoms
Lupus symptoms in different organs and organ systems.
Depending on which organs and/or organ systems are affected by the disease, more specific lupus symptoms occur. Some of them appear at the beginning, others only in the further course of the disease.
- Skin and hair: In areas of the skin exposed to the sun (such as the décolleté, face), rashes may develop suddenly or gradually. A typical feature is the butterfly-shaped reddening of the skin on the face, which increases with exposure to the sun. Other possible lupus symptoms include oral mucosal inflammation and circular hair loss.
- Joints: Very common lupus symptoms are painful and/or swollen joints. Joint pain occurs preferentially in the early morning. Inflammation often develops in many joints (polyarthritis), especially in finger, hand and knee joints. Tendon sheaths may also be affected by the inflammatory changes.
- Muscles: Sometimes SLE affects the muscles, which can lead to muscle inflammation (myositis), muscle pain, and muscle wasting (muscle atrophy).
- Kidneys: Many lupus patients develop kidney inflammation (lupus nephritis). It manifests with protein in the urine (proteinuria) and possibly water retention in the tissues (edema). The inflammation can worsen kidney function – even leading to kidney failure. Those affected are then dependent on regular blood washing (dialysis).
- Abdominal: Sometimes peritonitis develops in the setting of systemic lupus erythematosus. Signs include abdominal pain, nausea, and vomiting.
- Central nervous system: In some lupus patients, the brain is affected. Then, for example, epileptic seizures, migraine-like headaches, impaired concentration and memory, and/or psychological changes (such as depression, psychosis) may occur.
- Blood count: Often the blood count changes in systemic lupus erythematosus. All three blood cell lines may be decreased: red blood cells (erythrocytes), white blood cells (leukocytes) and platelets (thrombocytes). Possible consequences are anemia, susceptibility to infections and an increased tendency to bleed.
Systemic lupus erythematosus: Causes
Exactly what causes systemic lupus erythematosus is unclear. However, several factors seem to be involved, especially genetic changes. In combination with external factors that stress or stimulate the immune system, systemic lupus erythematosus can then break out. In addition, such factors may exacerbate an existing disease flare. They include:
- infections with viruses or bacteria
- intense sun exposure
- extreme climate change
- extreme psychological stress
- hormonal changes (e.g. during puberty, pregnancy and menopause)
Very rarely, certain medications trigger mild lupus (e.g., the antihypertensive drug methyldopa or the epilepsy drug carbamazepine). However, unlike “normal” SLE, this disappears within weeks to a few months after the medication is stopped.
Systemic lupus erythematosus: Diagnosis
If “systemic lupus erythematosus” is suspected, patients should therefore be examined by a specialist, i.e. a rheumatologist or pediatric rheumatologist. Because of the wide range of symptoms, cooperation with other specialists is advisable, for example with a dermatologist, a heart and kidney specialist or a gynecologist. The extensive examinations necessary for the diagnosis of “systemic lupus erythematosus” can also be performed as an inpatient in a hospital (for example, in children).
Interview and physical examination
The physician will first have a detailed discussion with the patient (with the parents in the case of children) about the medical history (anamnesis). This is followed by a physical examination.
Blood test
In addition, blood testing often reveals a deficiency of white blood cells (leukocytes) and platelets (thrombocytes), as well as anemia. The blood cell sedimentation rate (ESR) is accelerated in many cases.
Further examinations
Numerous other examinations allow the physician to assess the extent of systemic lupus erythematosus and the involvement of individual organs. For example, elevated blood pressure and protein in the urine may indicate involvement of the kidneys in the sense of inflammation of the kidneys (lupus nephritis). Further examinations such as an ultrasound of the kidneys can confirm this suspicion.
Depending on the symptoms, X-ray examinations may also be performed, the back of the eye examined and/or the patient’s lung function checked.
Criteria catalog
Accordingly, antinuclear antibodies (ANA) must first be detected at least once in a patient for SLE to be considered (obligatory entry criterion).
Secondly, further criteria with points from a list must be fulfilled, in fact so many that in total a number of points of at least ten results. These criteria are arranged in ten areas (domains), for example:
- Skin/mucosa: Hair loss, ulcers in the oral mucosa, SCLE, ACLE, DLE (three subtypes of cutaneous lupus erythematosus).
- Blood: deficiency of leukocytes, deficiency of platelets, autoimmune hemolysis (destruction of erythrocytes by autoantibodies)
- Constitutional: fever not caused by infection.
The individual criteria do not have to exist at the same time, for example, fever and hair loss. In addition, they are only scored if there is no more likely explanation for them than SLE.
Systemic lupus erythematosus: Treatment
Systemic lupus erythematosus cannot be treated causally – neither in children nor in adults. Only symptomatic therapy is possible, i.e. treatment of the symptoms of the disease. To this end, doctors draw up an individual therapy plan for each patient, depending on which organs are affected, how severely, and how active the disease currently is.
Medication
The aim of treating SLE is to curb the inflammation and the excessive activity of the immune system. For this purpose, various substance groups are available for internal (systemic) use:
- Non-steroidal anti-inflammatory drugs (NSAIDs): If systemic lupus erythematosus is only mild, such anti-inflammatory drugs (e.g., ibuprofen, diclofenac) are helpful. They also have an analgesic and antipyretic effect and prevent platelets from clumping together.
- Glucocorticoids (“cortisone”): They have a strong anti-inflammatory effect. They are often given in high doses over a short period of time (shock therapy or pulse therapy) to combat inflammatory episodes in otherwise mild SLE. They are also used in severe SLE.
- Immunosuppressants: They reduce the activity of the immune system, which is overactive in SLE. Examples include azathioprine, methotrexate, cyclophosphamide, and the bioengineered antibody belimumab. Such agents are considered in severe cases when other drugs do not help sufficiently.
Glucocorticoids can also be applied topically for skin symptoms, for example as an ointment.
Accompanying measures
The drug treatment of lupus described above can be supplemented by other measures. These include, for example:
- Cold applications for swollen, painful joints
- Respiratory therapy for breathing difficulties
- “blood thinning” medications for a tendency to blood clots
- Psychological procedures, for example to cope with pain
- vaccinations as protection against infections
To learn what to consider regarding vaccinations when the immune system is compromised (as in the context of SLE), see the article Immunosuppression and Vaccination.
Systemic lupus erythematosus: Course and prognosis
Systemic lupus erythematosus usually has a chronic, relapsing course. Months or years may pass between two successive episodes of the disease. In most patients, the attacks also become less frequent and weaker over time. SLE can therefore become less severe with increasing age.
Life expectancy
Most SLE patients today have a normal life expectancy. However, the quality of life of many patients is limited: Those affected suffer repeatedly or constantly from fatigue, skin changes and infections, or are dependent on regular blood washing (dialysis).
The most common cause of death in people with systemic lupus erythematosus is now no longer the disease itself: most patients die from complications of the disease.
Complications
Systemic lupus erythematosus can cause serious and sometimes life-threatening complications:
- Kidney inflammation: over time, it can cause kidney function to decline (renal insufficiency) or be lost completely (kidney failure). Those affected then need regular blood washing (dialysis) or a kidney transplant.
- Inflammation of the spinal cord: It triggers paralysis of the legs and (more rarely) the arms and can lead to paraplegia if not treated quickly.
- Inflammation of the optic nerve: If optic neuritis is not detected and treated in time, there is a risk of blindness.
- Increased risk of infection: SLE patients are particularly susceptible to infections with viruses, bacteria and fungi. If these are not treated consistently, there is a risk of organ damage.
- increased susceptibility to malignant diseases (cancer).
Systemic lupus erythematosus: Prevention
Systemic lupus erythematosus cannot be prevented – the causes of the chronic inflammatory autoimmune disease are not yet known. However, several factors have been identified that can trigger a disease flare-up. So to prevent an SLE flare-up, heed the following advice:
- Refrain from smoking.
- Drink alcohol only in moderation.
- Eat a balanced diet. Make sure you eat a mixed diet with sufficient vitamins and minerals (fresh fruit and vegetables, wholemeal bread, dairy products, etc.).
- Move regularly and exercise in moderation (even if you have symptoms such as joint pain).
- Avoid sources of infection, especially if taking immunosuppressants (these make you more susceptible to infections).
Psychological factors also influence the course of the disease. Especially chronic diseases such as systemic lupus erythematosus can depress the mood and make people depressed. This in turn triggers stress, which in the long term can have a negative impact on the hormone balance, the immune system and thus also the course of the disease.
