Lupus erythematosus: causes, symptoms, treatment

What is lupus erythematosus?

Lupus erythematosus (also called lupus or LE for short) is one of the rheumatic diseases. The name of this disease comes partly from the Latin (lupus = wolf), and partly from the Greek (erythema = redness). Perhaps the typical reddish skin changes on the face of many sufferers reminded the first writers of this disease to the bite of a wolf. An earlier name of the LE was therefore also "wolf disease".

Two forms of lupus erythematosus can be distinguished. While in the systemic form of the LE (short SLE) next to the skin and other organs are affected, occur in the cutaneous (lat. Cutis = skin) form of lupus erythematosus (CLE) only skin lesions.

Lupus erythematosus mainly affects women of childbearing age. The disease typically breaks out between the ages of 20 and 30 years. On average, women are affected about ten times more frequently than men. General statistics on the frequency of the disease are rarely reported, as there are large differences between different countries and ethnic groups. For example, It is known that African American women have about three times the incidence of disease compared to white women in the United States of America.

A disease with many faces

The course of a lupus erythematosus can vary greatly from person to person. While some become seriously ill, others rarely notice any symptoms. The skin changes can take on different forms. They have a different size, scar, or are scaly in the center and sensitive to touch. The reddish spots can occur here not only on the face, but on the whole body. In particular, body parts are affected, which are particularly exposed to solar radiation, such. the shoulders, the arms or the neck.

Apart from the skin, especially the kidneys, but also other organs can be affected. Furthermore, joint and muscle pain and inflammation of the pleura or the pericardium may occur. Likewise it can come to an infestation of the nerves and to disturbances of the blood coagulation. Most sufferers also complain of tiredness, shivering, fever and fatigue.

In most people with LE, times when symptoms are mildly alternate with times when the disease is on the rise. In this context, periods of increased symptoms are also referred to as so-called relapses.

Due to the range of different symptoms that are similar to other diseases, a diagnosis of lupus erythematosus is often very difficult. It can sometimes take several years for a diagnosis to be certain.

The importance of the immune system

To combat pathogens, our body produces certain proteins called antibodies. Antibodies usually bind pathogens and render them harmless. In lupus erythematosus, the immune system is misdirected to produce antibodies that bind to endogenous tissue instead of pathogens. Antibodies that bind to the body's own tissue are called autoantibodies, with the prefix "auto" meaning "self" or "own" from the Greek. Autoantibodies trigger inflammation, activating immune cells that destroy connective tissue in lupus erythematosus.

The results of today's research suggest that in addition to the immune system but also the influence of hormones and genes is crucial for the disease process. Thus, lupus erythematosus occurs frequently in families, suggesting a genetic background. However, the disease is not directly inherited, but the predisposition only increases the risk of getting sick. In addition, the fact that lupus erythematosus occurs significantly more often in women than in men suggests an important role for sex hormones.

Risk factor UV light

It is known that ultraviolet (UV) light causes severe relapses of the disease. Often this remains unnoticed at the beginning of the disease, as symptoms often occur after a time in the sun with a time delay. Consistent sun protection is particularly important for people with lupus for this reason. It should be noted that windows are permeable to UVA radiation.A corresponding sunscreen should therefore be used e.g. even when staying in vehicles.

Main therapeutic goal: suppression of inflammatory processes

Lupus erythematosus can not be cured today, but treated well. As with many other rheumatic diseases, the sooner the therapy is started, the better the chances of a favorable course and a long life expectancy.

Since the main goal of treating a LE is to suppress the inflammatory processes, depending on the severity of the disease and tolerability, appropriate medication for each individual affected. However, a drug that is almost always used is chloroquine or hydroxychloroquine. It can reduce inflammation in the body and reduce the likelihood of relapses.

causes

The exact cause of a LE is largely unknown. On the one hand there is a malfunction of the immune system, in which one's own body tissue is suddenly recognized as foreign and is attacked by immune cells; On the other hand, environmental factors such as UV radiation or certain genes also seem to influence the development and course of the disease.

Accumulations of immune complexes

Normally, during their development, immune cells learn to distinguish pathogens from their own tissues. Nevertheless, cells that recognize and attack the body's own tissue, they are destroyed immediately.

In autoimmune diseases, including lupus erythematosus, this mechanism no longer works completely. The tissue of the skin and internal organs is mistakenly recognized and destroyed by autoimmune cells as foreign. Certain proteins, so-called autoantibodies, are formed. If these autoantibodies bind to their target structures, so-called immune complexes are formed. Accumulations of immune complexes can eventually attract inflammatory cells that damage the tissue. In a LE, the blood vessels are affected. Such inflammation of the vessel walls is called in the jargon vasculitis.

Inflammations mainly affect the blood vessels

Once the body's own tissue is declared an enemy, it stays that way for a lifetime, i. The malfunction of the immune system can not be reversed with the currently available drugs.

As a result of the immune reactions, inflammation can occur throughout the body, either persisting without interruption or in the form of relapses. The causes of such relapses may include sunlight, infections, psychological stress and medications. In addition, it is known that contraceptives containing hormone preparations or pregnancies can enhance LE. Smoking also seems to worsen the course of the disease, as it narrows the blood vessels and thus promotes circulatory disorders and hypertension.

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symptoms

In lupus erythematosus (LE), an all-skin form, cutaneous lupus erythematosus (CLE), can be distinguished from a systemic LE (SLE) that affects not only the skin but also other organs. However, a CLE can not always be easily distinguished from a SLE because the cutaneous form can transition to the systemic shape. In addition, the individual subforms of a CLE can not always be clearly distinguished from each other, so that the transitions between different subforms are partially fluid.

1. Symptoms of Cutaneous Lupus Erythematosus (CLE)

Cutaneous lupus erythematosus currently has four different subtypes:

  • the acute cutaneous LE (ACLE)
  • the subacute cutaneous LE (SCLE)
  • the chronic cutaneous LE (CCLE)
  • and the intermittent cutaneous LE (ICLE).

All four subforms are characterized by an inflammation of the skin between the epidermis and the dermis beneath it. Under the microscope, at this point an accumulation of inflammatory cells can be detected, which normally do not belong there.

Acute cutaneous lupus erythematosus (ACLE)

An ACLE can be local or generalized. The more common local form is typically characterized by reddish changes on the bridge of the nose and cheeks, which are symmetrical and sharply defined. Because of their appearance, these rednesses are also known as so-called "butterfly erythema" (Greek: erythros = red) and may possibly be misinterpreted as sunburn at the beginning of the illness. Butterfly erythema usually heals without scarring or discoloration of the affected areas of the skin.

While in the local form the lesions on the cheeks and nose are limited, in the generalized form of the ACLE the skin changes occur on the entire body. Often, hands and feet are affected. In the area of ​​the nails, redness may occur due to dilated blood vessels.

Another characteristic feature of an ACLE is that transition from the cutaneous to the systemic form occurs most frequently here.

Subacute Cutaneous Lupus Erythematosus (SCLE)

A subacute cutaneous LE only becomes systemic in about 10-15% of those affected. When this happens, it affects the joints and muscles. Typical skin lesions of the SCLE are characterized by annular and scaly redness, which may fuse together and assume characteristic garland-like shapes. They can easily be confused with another disorder, psoriasis. The face is usually spared by the redness. They occur mainly on the neck and the arms. The course of the subacute cutaneous LE is in many cases relapsing-recurring.

Chronic cutaneous lupus erythematosus (CCLE)

With this subform of the CLE further forms can be distinguished:

  • the discoid LE (DLE)
  • lupus erythematosus profundus (LEP)
  • and chilblain lupus erythematosus (CHLE).

Discoid lupus erythematosus (DLE)

The most common variant of CCLE is the so-called discoid lupus erythematosus. As the name suggests, this disease causes discoid redness with a firm, whitish scaling that is found on the face, scalp and auricles in the majority of sufferers. Only about 20% of the redness occurs in addition to the upper body, the arms and legs. After the lesions have healed, light scars remain with a darker pigmented rim. In the scalp area, these scars remain hairless. A DLE only changes to a systemic LE in about 5% of those affected.

Lupus erythematosus panniculitis (LEP)

This subform of CCLE is very rare and is characterized by inflammation of the subcutaneous tissue. Doctors speak in this context of a so-called panniculitis. Typical skin lesions of a LEP include well-defined knots below the skin around the arms and shoulders, thighs or buttocks. These may be in conjunction with the overlying skin. During the course of the disease, these nodes heal, resulting in deeply retracted scars. In about 70% of cases, LEP can also occur with a DLE.

Chilblain lupus erythematosus (CHLE)

The Chilblain LE is also a very rare subform of the CCLE. The term "chilblain" comes from the English and means "frostbite". The bluish pressure-sensitive swellings are in fact very similar to chilblains, and are usually indistinguishable even under the microscope. They occur mainly in the cold and humid seasons and are mainly found on the fingers and toes, nose and ears.

Intermittent cutaneous lupus erythematosus (ICLE)

Since 2004, the ICLE has been considered as a separate form of cutaneous LE, based on the latest scientific evidence, independent of the subtypes of the CCLE. Characteristic of this disease are red plateau-shaped swellings with a smooth surface that look very similar to hives. They mainly occur on body parts that are exposed to solar radiation. These lesions can persist for several months and heal without scarring. Usually, an ICLE does not transition into a systemic SLE and is associated with a good prognosis.

2. Symptoms of Systemic Lupus Erythematosus (SLE)

Not only the symptoms of SLE, but also the severity of the disease vary greatly from person to person. While there are hardly any limitations in everyday life for some people affected, the disease can also end in life-threatening deaths for others.

Although it is difficult to give exact numbers, experience has shown that the most common initial symptoms of SLE are quite nonspecific (such as fatigue, fever, or weight loss) or skin changes and joint discomfort. In about 80% of those affected SLE is associated with the cutaneous form, the CLE.

The mean frequency of various symptoms during the course of SLE, as determined in several studies, is shown below:

  • Non-specific symptoms: 90-95%
  • Changes in the skin and mucous membranes: 80-90%
  • Complaints in the locomotor system, e.g. Joint or muscle inflammation: 80-90%
  • Inflammations of the abdominal and lung skin and the pericardium: 50-70%
  • Inflammation of the kidney: 40-60%
  • mental and nervous disorders, e.g. Depression, Psychosis, Strokes or Epileptic Seizures: 40-60%
  • altered blood picture, e.g. Iron deficiency, deficiency and platelets: 20-30%

The course of a SLE can look very different. An acute course is characterized by a sudden onset of high fever, skin changes and inflammation at various points in the body. A chronic course is when symptoms such as e. Skin lesions, increased photosensitivity or joint pain begin at first creeping, spontaneously regress, and then again and again occur intermittently.

A special form of the SLE is also the drug-induced SLE dar. Drugs cause mainly problems with the kidneys and the brain.If these drugs are discontinued, the symptoms return to normal. If you have a drug-induced SLE, it's best to discuss it in detail with your doctor.

Skin lesions and nonspecific symptoms

Nonspecific symptoms such as Fever, weight loss, fatigue and general malaise, but also skin lesions are amplified by ultraviolet (UV) light in a LE. In this context, one speaks of a so-called "photosensitivity". This often occurs characteristic redness v.a. on the face, in the neck and on other, exposed to the sun body parts. Depending on the subform they are differently pronounced.

In addition, damage to the oral mucosa and nail fold can occur. In more rare cases, in about 5% of those affected, it may come to a so-called "Raynaud's syndrome", which is characterized by painful, seizure-like circulatory disorders of the hands.

Complaints in the movement system

a) joints

Joint pain (arthralgia) and joint inflammation (arthritis) are very common symptoms of SLE. They occur in up to 90% of the patients in the course, but can also regress. Mostly the knees and wrists of both hands and feet are affected.

In contrast to arthritis in rheumatoid arthritis, X-rays in SLE usually do not cause any damage to the joints. Often, however, are changes in the connective tissue in the joints, which are associated with inflammation.

If the vessels are damaged, the bone tissue can no longer be well supplied with oxygen and nutrients. In some cases, the result may be a painful death of tissue called necrosis.

b) muscles

While muscle pain (myalgia) is common in SLE, muscle inflammation (myositis) is rare. In most cases, myositis causes pain and weakness in the arm and leg muscles, v.a. near inflamed joints. It is important to distinguish here whether the muscle pain is caused by myositis or by medications such as myositis. Glucocorticoids are caused because the treatment is different depending on the cause.

Inflammation of the abdominal and pleura and the pericardium

Both the body cavities (the thoracic or abdominal cavities) and some organs, e.g. the heart or the lungs are lined by a special layer of cells, which allows movement and movement of the organs. In the lungs one speaks of a so-called lung and pleura (pleura) and in the heart of a pericardium (pericardium). The abdominal organs are surrounded by a peritoneum.

If the pericardium (pericarditis) or the pleura (pleuritis) becomes inflamed, the affected people usually do not notice it. On the other hand, inflammation of the peritoneum (peritonitis) often causes abdominal pain, vomiting and nausea. In addition, an inflammation of the heart's lining (endocarditis) and the heart muscle (myocarditis) can occur at the heart, which can lead to further sequelae.

Inflammation of the pulmonary vessels

If there is an acute inflammation of the blood vessels in the lungs, it causes fever, shortness of breath and coughing. The symptoms are similar to those of pneumonia. If the inflammation is chronic, connective tissue is increasingly formed. As a result of the inflammatory process, scar tissue (pulmonary fibrosis) and hypertension may develop. Those affected usually suffer from dry coughing and dyspnea under stress.

kidney damage

An important function of our kidneys is to filter the blood, while draining metabolic end products and pollutants from the body through the urine. This function is ensured by so-called renal corpuscles (glomeruli), which consist of small blood vessels. If these blood vessels ignite in the glomeruli, this is called glomerulonephritis.

Kidney damage is very common in SLE. Although only about 50% of sufferers experience symptoms of nephritis, studies show that up to 90% of kidney damage can be found even if there are no symptoms. Since the onset of kidney damage is gradual and initially causes no symptoms, regular check-ups of renal function should be performed.

If symptoms arise, it may initially lead to a restriction of renal function, which can end after several years with kidney failure. Much less common is acute glomerulonephritis, which manifests itself as a rapid increase in blood pressure, fluid retention in tissues, and a sudden decrease in urine output.

Diseases of the nerves

In the course of a SLE, blood vessels in the brain can become inflamed. This can cause a large number of different symptoms, such as Headache, mood swings, blurred vision and hearing and balance disorders. Very seizures also occur.

Often, however, it is difficult to distinguish whether these signs of disease actually stem from an active SLE disease, or are caused by other, co-occurring diseases, e.g. Metabolic disorders or infections have been caused. Little is known about the cause and the course of the disease.

Enlarged lymph nodes, spleen and liver

Usually, the nature of the lymph nodes in people with SLE is especially during a push, i. during an active phase of the disease, changed. Characteristically, enlarged but non-pain sensitive lymph nodes are v. A. in the area of ​​the neck, armpits and groin. However, other lymph nodes can be affected throughout the body. In rare cases you can find an enlarged spleen.

Increased coagulation tendency

In some patients, so-called anti-phospholipid autoantibodies can be detected, which lead to an increase in blood clotting after binding to their target structures. As a result, clots (thromboses) can develop in the blood vessels of the legs.

Thrombosis is a problem, for example, when they detach from the vessel walls and are flushed through the circulation in the lungs, where they can trigger closures (pulmonary embolisms). Due to the increased coagulation tendency, vascular occlusions can also occur within the maternal placenta, which increase the risk of miscarriage.

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Questions and answers about the symptoms

diagnosis

Since there are many different diseases characterized by symptoms similar to those of lupus erythematosus, the diagnosis of this disease is often a great challenge to the attending physician. It is based on the onset of symptoms, a thorough physical examination, blood tests and other examination methods to check organ function.

1. Physical examination methods

After a thorough record of the medical history, the doctor will check your skin for changes such as Check for redness on the face and upper body. If necessary, skin samples (biopsies) can be taken.

If the skin shows no obvious changes, a so-called photo provocation test can be performed. In this case, a specific skin area is irradiated with UVA and UVB light on several consecutive days. Subsequently, it is checked over a period of a few weeks whether the exposed skin areas due to the irradiation have changes similar to those of an LE.

Another part of the physical examination is checking the joints for swelling and restricted mobility.

2. Blood tests

Autoantibodies

The lupus erythematosus is one of the autoimmune diseases, which is why the detection of so-called autoantibodies is of great importance and provides important information for the diagnosis. At a first suspicion, it is first determined whether anti-nuclear antibodies (ANA) are present. These are autoantibodies that bind to structures of the cell nucleus (lat. Nucleus = cell nucleus) of a cell. If these antibodies can not be detected (negative ANA value), it is very likely that no lupus erythematosus is present. However, if the ANA value is positive, there may be an LE, but it does not have to, as people with other illnesses or completely healthy individuals may be ANA positive.

For this reason, if the ANA value is positive, more detailed blood tests are performed to check for the presence of various autoantibodies that recognize different constituents of the nucleus. This is important because some of these antibodies, v. A. the anti-dsDNA autoantibodies are very specific for a LE. That is, in a positive anti-dsDNA test, it is very likely that the disease actually exists. In addition, some autoantibodies are associated with certain symptoms. This means that the presence of such characteristic autoantibodies increases the risk of developing certain symptoms as the disease progresses.

The most important autoantibodies are listed below:

  • anti-dsDNA (occurring in 60% of sufferers, this antibody is almost only detectable in SLE, the antibodies are often associated with kidney problems)
  • anti-Smith (occur in 60% of those affected, and this antibody is almost only detected in SLE)
  • anti-Ro / SSA (occurring in 30% of individuals, associated with subacute cutaneous lupus erythematosus (SCLE) and increased sensitivity to UV light)
  • anti-La / SSB (present in 20% of patients) associated with subacute cutaneous lupus erythematosus (SCLE) and increased sensitivity to UV light)
  • anti-phospholipid (occurring in 30% of those affected, associated with blood clots and increased miscarriage rate)

complement

The complement system is made up of certain proteins in the blood that support the activity of the antibodies and thus normally protect against infections. In lupus erythematosus, the concentration of various complement proteins is decreased and gives an indication of whether the disease is in an active state.

inflammatory markers

The erythrocyte sedimentation rate (ESR) or erosion rate (ESR) indicates the rate at which the red blood cells (erythrocytes) in a blood sample in a measuring tube fall. BSG may, but is not necessarily, elevated in inflammation and especially lupus erythematosus. The same applies to the C-reactive protein (CRP), another inflammatory value.

kidney function

Here, both the blood and urine can be examined to see if the kidneys are functioning properly. Blood or proteins in the urine can already indicate an impairment of kidney function at a very early stage, even if no symptoms have yet occurred.

3. Studies of organ function

In addition to the physical examination and the blood tests, different examinations can be performed to check the function of the internal organs. Ultrasound (sonography), an electrocardiogram (ECG), computed tomography (CT) or magnetic resonance imaging (MRI) can be used for this purpose, for example. It is important that in the course of a lupus erythematosus investigations of the organ function take place at regular intervals in order to diagnose in the course of the disease a deterioration of internal organs in time.

treatment

By taking medication and avoiding certain trigger factors, lupus erythematosus can now be well controlled. While there is a very broad range of drugs available for the treatment of SLE, drugs specifically designed to treat a CLE are still in development. However, many substances that are involved in SLE and other rheumatic diseases may also be prescribed for CLE in individual cases.

In addition to a drug treatment, you can also do many things yourself to alleviate your symptoms. Since it is a disease in which the symptoms improve and sometimes worsen over the course of the disease, each affected person can try to find out for themselves which trigger factors cause a worsening or improvement of the disease. For example, it helps some people to rest and reduce stress, stay away from the sun, or protect themselves from infection as much as possible.

Finally, it is important that people with lupus erythematosus have general and nonspecific symptoms, e.g. Take fatigue, shivering, fatigue or mood swings seriously and discuss with your doctor. The attending physician may try to change the combination or dose of the drugs to be more effective but at the same time better tolerated.

1. Therapy of Cutaneous Lupus Erythematosus (CLE)

In many cases, inflammation of the skin in cutaneous lupus erythematosus can be well controlled locally by drugs. In addition, many symptoms improve by the sole avoidance of risk factors, e.g. Sunlight or smoking.

However, if local therapy does not help and there is a high risk of scarring and pigmentation, early systemic (internal) treatment is needed. For the systemic treatment of a CLE there is currently no drug that is officially approved. For this reason, in individual cases, if necessary, prescribed medications that are normally used in other diseases, but can also act in a CLE.

a) Local therapy of the CLE

glucocorticoids

In order to reduce inflammation locally on the skin and prevent scarring, creams or ointments with a cortisone preparation (glucocorticoids) are used. The use of such preparations is especially necessary in the area of ​​the hairy scalp as well as the soles of the feet and palms. Glucocorticoids suppress the immune system and thus limit the inflammation. Especially on the face, the application should not take place over a longer period due to the side effects.

Calcineurin inhibitors

The calcineurin inhibitors tacrolism (0.1% ointment) and pimecrolism (1% cream) also show good efficacy. Although onset of treatment often results in burning on the treated skin, calcineurin inhibitors cause fewer side effects than glucocorticoids, making them particularly suitable for facial skin use. Usually, an improvement in symptoms can be seen within a few days.

camouflage

If it has already come to scarring or changes in skin pigmentation, a special, strongly covering make-up can help, a so-called camouflage.Patients can get advice from the doctor or pharmacist if they have questions.

b) Systemic therapy of CLE

Chloroquine / hydroxychloroquine in CLE

In the case of severe disease, some CLE sufferers also use medications that are usually formally used in SLE or malaria: hydroxychloroquine and chloroquine. In consultation with the doctor, the dosage and length of the application can be adjusted. In addition, regular eye and liver and kidney examination is important to prevent organs from being damaged by therapy.

Further therapy options

In case of intolerance to antimalarials or if therapy does not respond, alternative medicines are available that are also prescribed for other rheumatic diseases. Some studies have shown that so-called retinoids, e.g. The acitretin and isotretinoin may have similar efficacy to antimalarials. However, these studies have so far shown meaningful results only in DLE and SCLE and for this reason should currently only be used in these diseases.

Other medicines that can be used for SCLE include the cytostatic methotrexate or azathioprine.

2. Therapy of Systemic Lupus Erythematosus (SLE)

The choice of a suitable therapy meets the treating rheumatologist depending on the severity of the inflammation and the impairment of affected organs. Depending on the activity of the disease and on the individual's life situation (for example a pregnancy) the treatment can be adjusted.

a) Light forms of a SLE

Mild forms of SLE with a predominantly skin and joint involvement are generally treated with disease-controlling drugs or disease-modifying drugs (DMARD). In addition, a therapy with glucocorticoids can take place.

Chloroquine / hydroxychloroquine in SLE

The antimalarials chloroquine and hydroxychloroquine (trade name Quensyl®) are the most commonly prescribed DMARD. Although these drugs affect the immune system, they do not suppress it.

Before starting treatment, the attending physician will usually check liver and kidney values ​​and check for any retinal disease in the eye. These examinations are necessary to ensure that the drug is well tolerated. Since chloroquine / hydroxychloroquine can rarely damage the retina, regular follow-up examinations should be carried out to detect changes in time and to discontinue treatment if necessary.

Chloroquine and hydroxychloroquine are not only particularly effective in joint inflammation, but also prevent the recurrence of relapses and reduce damage in the course of the disease. In addition, studies have shown a reduced risk of complications.

According to the current expert opinion, antimalarials in low dosage can also be taken during pregnancy and lactation. The optimal effect usually occurs after 3-6 months, with no smoking, as cigarette smoke has been shown to reduce efficacy.

When SLE is mainly prescribed hydroxychloroquine, being switched to inactivity or intolerance to chloroquine.

glucocorticoids

In case of a sudden worsening of the symptoms, additional cortisone preparations (glucocorticoids or steroids) can be prescribed, which are taken in the form of tablets. Glucocorticoids act like natural steroids, which are also produced in the body. They inhibit inflammation and reduce swelling and pain.

Cortisone tablets are usually taken once a day. The dose depends on the symptoms and weight of the person. If the symptoms improve, the intake can be stopped again. It is important to ensure that glucocorticoids are not stopped at once, but that the dose is reduced in small increments to avoid side effects. Sometimes it also helps to take glucocorticoids over a longer period of time in a low dose to reduce the likelihood of further relapses while avoiding side effects.

The most common side effects are stomach aches, indigestion, weight gain, fluid and fat deposits. In addition, there is a degradation of bone, which leads to an increased probability of fractures. One speaks in this context of a bone loss or osteoporosis. In order to avoid osteoporosis, calcium and vitamin D supplements should therefore be taken with glucocorticoid therapy.

b) Difficult course of a SLE

In a severe, life-threatening course of SLE with impaired multiple organs are initially prescribed drugs that suppress the immune system strong, so-called immunosuppressants. They are designed to inhibit overactive immune cells and prevent tissue damage.

If the symptoms improve after treatment with the immunosuppressants, treatment with less aggressive and better tolerated drugs or medications is initiated at a lower dose to reduce the likelihood of further relapses.

Cylcophosphamide (CYC)

By default, a combination of high-dose glucocorticoids and injections of the drug cylcophosphamide (CYC) is currently being administered for immunosuppression. Cyclophosphamide works by destroying cells that divide quickly. The effect is delayed, however. It can usually take up to six weeks to see an improvement in symptoms.

A common side effect of CYC is nausea. In addition, it can lead to inflammation of the bladder and changes in the blood picture. Regular examinations of the blood and urine are essential for this reason.

The need for treatment with CYC should first be discussed with the attending physician, as this drug is highly toxic to sperm and egg cells and can lead to infertility. Pregnancy and breast-feeding should also be avoided during treatment with CYC as it is harmful to the unborn or newborn child.

Mylophenolate mofetil (MMF)

Alternatively, the medication mycophenolate mofetil (MMF) may be prescribed for moderate kidney inflammation, causing fewer side effects and generally better tolerability. It should be noted that the effects on MMF are delayed, as is the case with CYC. It can take up to three months for this medicine to get better.

The most common side effects seen with mycophenolate are nausea, diarrhea, vomiting and abdominal pain. In addition, the susceptibility to infections is increased, so if you experience fever, sore throat and other typical symptoms of a cold or flu the doctor should be consulted.

Methotrexate (MTX)

Another drug to suppress the immune system is methotrexate (MTX), which is also prescribed for other rheumatic diseases. It has a good effect on an infestation of the joints and is generally well tolerated. However, blood levels should be monitored at regular intervals during treatment to prevent damage to the liver, kidneys and bone marrow. Side effects can be alleviated or prevented by taking folic acid.

biologics

It is now known that in the course of the immune response in a SLE certain immune cells, called B cells, are activated by mistake. B cells produce antibodies and therefore play a crucial role in the course of the disease. Recently, therefore, so-called biologics are under development to curb the activation of these B-cells.

The first biologic already approved for the treatment of SLE is belimumab. This drug binds to a messenger called BLyS, which normally stimulates B cells. If the antibody now binds to BLyS, BLyS can no longer fulfill its function. The activation of overactive B-cells is thus reduced and the symptoms improve.

At the moment, biologics are rarely used in people with very severe SLE outcomes, when other medications do not work. However, it is still being researched which patients benefit most from the therapy with biologics and how side effects can be minimized in this form of therapy.

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Important question about the treatment

Avoidance of risk factors

Symptoms of lupus erythematosus or lupus-like symptoms may be aggravated or even triggered by certain drugs, ultraviolet radiation or other environmental factors. In addition to taking medication, you can improve your symptoms by avoiding these triggering factors.

sun protection

In lupus erythematosus, it has been known for many decades that ultraviolet (UV) radiation can worsen symptoms and trigger relapses. This can be seen in both CLE and SLE. In humans with CLE, UV radiation can in rare cases also cause a systemic form, the SLE.

Both sunlight and artificial UV light, e.g. in tanning salons, should be avoided. It is advisable to consult a doctor or pharmacist here for detailed advice on which products are suitable and how they are used correctly.

Contraception and pregnancy

It is known that hormone changes, such as in pregnancies or taking estrogen-containing contraceptive methods such as. of the pill or the hormonal spiral, can exacerbate lupus erythematosus. In addition, not all medications prescribed for LE are suitable for pregnant women and their unborn children. Therefore, when choosing to have a baby, women should consult their rheumatologist carefully.

Smoke

Smoking is considered a risk factor for lupus erythematosus. Studies have shown that cigarette smoke not only favors vascular damage, but also inflammatory responses. In addition, cigarette smoke can reduce the effectiveness of the antimalarials chloroquine or hydroxychloroquine. People with lupus erythematosus should therefore try to avoid smoking altogether and avoid passive smoking.

infections

Most of the medicines taken in lupus erythematosus lower the activity of the immune system and therefore lead to a higher susceptibility to infections.Doctors and stakeholders should therefore together look for ways to minimize the risk of infection with pathogens.

For this purpose, in lupus, the use of drugs that suppress the immune system (immunosuppressants) should be done in such a dosage that the immune system is still able to defend itself sufficiently against pathogens.

It is also advisable to regularly check the vaccination status and to vaccinate if necessary. In addition to the annual flu vaccine, a vaccine against pneumococci should also be considered. In any case, vaccinations with so-called live vaccines should be avoided. These vaccines contain small amounts of attenuated but functional germs. If live vaccines are used in people whose immune system, such as lupus erythematosus, is suppressed by certain drugs, it can lead to an uncontrolled spread of the germs and complications.

stress management

Although stress may not directly affect the course of the disease, emotional stress can cause symptoms of the disease to be more intensively affected by those affected. It makes perfect sense to seek advice and support from family and friends. Many sufferers also help relaxation techniques or the participation in self-help groups or the exchange with other stakeholders.

cold

In a special form of CLE, the so-called chilblain lupus erythematosus, those affected should pay attention to a corresponding protection of the hands and feet. Regular exercise is also important to stimulate the circulation and improve blood circulation.

Disease-causing drugs

Another special form of lupus erythematosus, the so-called drug-induced lupus erythematosus, can be triggered by a variety of different drugs. These medications can trigger an autoimmune reaction that causes similar symptoms to an LE.

There are about 40 drugs that can trigger a drug-induced LE, such as the antihypertensive drug hydralazine or the agent procainamide, which is taken in arrhythmias. Although many of these drugs are no longer prescribed today, a new class of compounds from the group of biologics has recently been added, which can also trigger a drug-induced LE. These are anti-TNF-alpha therapeutics (adalimumab or infliximab) which have been used in various inflammatory diseases, e.g. in rheumatoid arthritis or Crohn's disease.

Usually, the symptoms of a drug-induced LE are milder and usually disappear after discontinuation of the drug. Unlike an LE, neither the kidneys nor the nervous system are affected. Blood tests typically show the presence of so-called anti-histone antibodies. However, it is also possible to detect the anti-nuclear antibodies (ANA) characteristic of lupus.

Author: Dr. Julia Spengler

sources

RKI, Federal Statistical Office, Federal Health Reporting, Issue 49: Inflammatory rheumatic diseases.

Kuhn et al., J Dtsch Dermatol Ges 2007, Clinical Manifestations of Cutaneous Lupus Erythematosus.

Arthritis Research UK: http://www.arthritisresearchuk.org/

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Lela A. Lee and Victoria P. Werth. The Skin and Rheumatic Diseases. In: Firestein GS, Budd RC, Herris ED Jr, et al. eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012: chap 43.

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