Diagnosis

Diagnosis is based on certain criteria that must be met: At least four of these symptoms must be fulfilled in order to make the diagnosis of lupus erythematosus. Not all relevant symptoms have been listed here – this is only an excerpt. The diagnosis of lupus erythematosus is usually performed in several steps.

In particular, the presence of typical symptoms, which are revealed during a doctor-patient consultation (anamnesis) and an extensive physical examination, represents an important step in the diagnosis of lupus erythematosus. In addition, the affected organs are specifically examined. The preparation of X-rays of the joints as well as the performance of an ultrasound examination are regarded as particularly suitable methods in the diagnosis of lupus erythematosus.

Another important step in the diagnosis of lupus erythematosus is the performance of various laboratory tests. Blood must be taken from the patient in order to obtain special laboratory values typical for the presence of lupus erythematosus. Typically, people suffering from lupus erythematosus have antibodies directed against the body’s own structures.

These specific antibodies can be detected in the blood of the affected person. The detection of autoantibodies is therefore an important part of the diagnosis of lupus erythematosus. In addition, further abnormalities in laboratory parameters can be observed in most of the affected patients.

People suffering from lupus erythematosus usually have an increased blood sedimentation and a decreased number of white blood cells (leukocytes) and blood platelets (thrombocytes). While the so-called C-reactive protein (CRP) behaves completely normally in most cases, many people suffering from lupus erythematosus suffer from pronounced anaemia. In the course of the diagnosis of lupus erythematosus, the number of complement factors C3 and C4 is also examined.

In affected patients, this number is usually significantly reduced. The number of these factors also allows the activity of the inflammation in systemic lupus erythematosus to be monitored. In addition, the performance of tissue examinations can be useful.

For this purpose, the treating physician takes small tissue samples from the skin (see: skin biopsy) and kidneys and sends them to a special laboratory. Tissue samples in which a so-called lupus band can be detected will significantly advance the diagnosis. Especially in the area of sun-exposed skin, this lupus band is caused by deposits of immune complexes.

In addition, the tissue sample from the kidneys is considered an important method in the diagnosis of lupus erythematosus. In the course of the disease, a so-called “lupus nephritis”, an inflammation of the kidneys, often occurs. Red blood cells arranged in cylinders (erythrocyte cylinders) can then be detected in the blood.

In addition, the inflammatory reaction leads to the release of protein through the urine and to an increase in blood pressure. Due to an acute restriction of kidney function, the accumulation of fluid in the tissue (edema) can also often be observed. The presence of lupus nephritis has a decisive influence on the most appropriate therapy and the course of the disease.In order to finally make a diagnosis of lupus erythematosus, at least four of eleven possible criteria must be met.

In diagnostics, these are referred to as ACR criteria.

Butterfly erythema
Photosensitivity
Arthritis of at least two joints
Renal Participation
Participation of the central nervous system
ANAs (specific antibodies) in blood
Fluid in the pericardium or around the lungs (in the pleural space)

A very important diagnostic tool is the blood test of the affected person. Various abnormalities and changes in the blood can be indications of lupus erythematosus.

The blood count may show a reduction in the number of platelets (thrombocytopenia), white blood cells (leukocytopenia) and especially lymphocytes (lymphocytopenia). Furthermore, blood tests can reveal changes that indicate a so-called hemolytic anemia. Hemolytic anemia is characterized by the breakdown of red blood cells.

It is also characterized by an elevated LDH value, an elevated indirect bilirubin, an elevated reticulocyte value and possibly an elevated free hemoglobin. In the case of lupus erythematosus, the so-called Coombs test is then performed to detect the antibodies responsible for the decay of the erythrocytes. This test is positive for lupus erythematosus.

General inflammation values in the blood are also examined. This often reveals an increase in the so-called blood sedimentation rate (BSG) with a simultaneously normal CRP value on which is used as an indication of inflammation in the body. Furthermore, the complement factors C3 and C4 may be lowered.

These form important components of the immune system. In the diagnosis of lupus erythematosus, special rheumatological antibody diagnostics is performed in addition to these general blood tests. Specific laboratory procedures (e.g. immunofluorescence test) are used to determine antibodies that are very important for the diagnosis.

A very important value is the so-called ANA value. ANA stands for Antinuclear Antibody and refers to a value that is positive in about 95% of patients with lupus erythematosus. Repeated negative ANA values therefore tend to speak against lupus.

Furthermore, antibodies against double-stranded DNA, so-called anti-dsDNA antibodies, are determined. This very specific value is positive in about 70% of the patients affected. A positive test result in this test speaks very strongly in favor of a lupus.

The stronger the disease activity and the symptoms, the higher this value usually is. Furthermore, it is often associated with kidney damage as part of lupus erythematosus (lupus nephritis). There are other antibodies that are examined in rheumatological antibody diagnostics.

These include anti-C1q antibodies and anti-SM antibodies. These values are not so often positive, but if they are, this strongly indicates lupus. The so-called SS-A-antibodies are also only positive in about 60% of patients.

Positive SS-A antibodies are also associated with Sjögren’s syndrome, another autoimmune disease. Finally, in some cases, antibodies against important components of the blood’s coagulation system can be found. Antibodies against blood platelets (thrombocytes) are symptomatically often associated with pin-like bleeding of the skin and mucous membranes (petechiae).

Another important component of the coagulation system is factor 8, against which antibodies can also be detected. Symptomatically, this is often accompanied by extensive bleeding or joint swelling. Unfortunately, lupus does not always manifest itself in the same way and must therefore be diagnosed in a differentiated manner.

Classifications can be made. The disease of lupus erythematosus can be divided into three forms:

Cutaneous lupus erythematosus This form usually only affects the skin and has a good prognosis. The disease either occurs only in isolated areas of the skin (usually on the head) or affects the entire body (trunk, upper arms).
The skin symptoms have a reddish inflammatory border (thickened edge) and are dented in the middle due to tissue loss.
Subcutaneous (under the skin) LE This form is characterized by a general feeling of illness, joint and muscle pain, and skin changes. Rarely the kidneys are affected.
Systemic lupus erythematosus This lupus is characterized by certain symptoms and manifestations that are used for diagnosis (see below).The organs are always affected – especially the kidneys, which also determine the extent of the disease. If the kidneys are strongly affected, SLE has a rather poor prognosis – if the kidneys are only slightly affected, the prognosis is better.

Other forms of lupus erythematosus:

Lupus erythematosus tumidus
Lupus erythematosus dissminatus
Lupus erythematosus discoides
Lupus erythematosus visveralis

Lupus erythematosus tumidus is a special form of cutaneous lupus erythematosus and is often referred to as intermittent cutaneous lupus.

Cutaneous lupus mainly affects the skin. The lupus tumidus is mainly characterized by skin changes on the face, neck, décolleté, arms and shoulders. The reddish, approximately 0.5-5 cm large skin lesions, called plaques or papules, occur mainly after contact with sunlight.

The skin of those affected is very sensitive to light. Unlike other cutaneous forms of lupus, scaling of the skin is rather untypical. The skin changes heal without scarring.

The term “tumidus” means “bloated” and is derived from the appearance of the skin changes. The term lupus disseminatus is often used as a synonym for systemic lupus erythematosus. The lupus miliaris disseminatus faciei must be distinguished from this.

This chronic inflammatory disease of the skin must not be confused with lupus erythematosus, but represents an independent disease. It is characterized by an infestation of the skin, which is mainly accompanied by reddish-brown skin changes of the eyelids, forehead and cheeks, the cause of which is unclear. Lupus discoides, or chronic discoid lupus erythematosus (CDLE), is characterized by an almost exclusive affection of the skin.

The skin changes are usually provoked by sunlight and have a disc-shaped appearance. This form of lupus is therefore also called “discoid”. The disc-shaped skin lesions are sharply defined, slightly raised and have a scaly surface.

A brightening is often found in the middle. The changes are usually only found in one part of the body and rarely in several parts of the body at the same time. They heal scarred and lead to hair loss on the hairy scalp (scarred alopecia).

Systemic lupus erythematosus used to be called visceral lupus, but this term is outdated. Unlike cutaneous lupus, which only affects the skin, it is a form that can affect any organ. This is why it is called systemic lupus.

It is feared above all to damage the kidney or other serious organ damage, which can lead to multi-organ failure. However, since systemic lupus is treated with medication, complications can often be prevented. There is no cure for patients suffering from lupus erythematosus.

For this reason, the therapy of this disease focuses on alleviating the typical symptoms. The most appropriate therapy for lupus erythematosus depends on which organ systems are affected and to what extent the disease manifests itself. Accordingly, there is no fixed treatment regimen for lupus erythematosus.

Rather, the type and intensity of treatment must be determined on a patient-specific basis. Since lupus erythematosus leads to a pronounced malfunction of the body’s own defense system (formation of autoantibodies), it is extremely important to suppress the body’s defense reaction (immunosuppression). For this reason, the most important drugs used in the therapy of lupus erythematosus include all substances from the glucocorticoid drug group.

A classic example of such a substance is cortisone. However, these drugs must be administered in particularly high doses and used over a long period of time. Due to the high number of possible side effects, however, many of those affected are afraid of long-term use.

In the meantime, the antimalarial drug “Hydroxychloroquine” is considered an alternative in the treatment of lupus erythematosus. This drug is said to be particularly suitable when the skin and joints are affected. Stronger active ingredients such as cyclophosphamide or azathioprine are generally only used in severe forms of lupus erythematosus.

They are mainly used in patients who show a clear involvement of the kidneys (lupus nephritis), the central nervous system or the heart (heart valve inflammation). In addition, completely new drugs are now available for the treatment of lupus erythematosus.Artificially produced antibodies (Belimumab) are able to inhibit part of the immune cells in lupus erythematosus patients and thus alleviate the symptoms. In cases where no significant improvement can be achieved by the administration of classical drugs, the active ingredient mycophenolate mofetil is used.

As a so-called reserve drug, this active ingredient has not yet been officially approved for the treatment of lupus erythematosus. In specialist circles, this is known as “off-label use”. Furthermore, in some cases, the therapy can help to remove autoantibodies from the circulation by performing a blood wash (plasmapheresis).

Patients suffering from lupus erythematosus can also positively influence the course of the disease by taking other drugs. In particular, drugs to lower blood pressure and cholesterol-lowering agents that minimize the risk of cardiovascular disease should be taken regularly in the treatment of lupus erythematosus. Various painkillers can be used to relieve pain.

In addition, affected patients should refrain from nicotine consumption and attach particular importance to a calcium-rich diet. The intake of vitamin D3 is also recommended to prevent concomitant osteoporosis. Since tissue damage can occur in patients with lupus erythematosus due to the deposition of immune complexes in sunlit skin areas, those affected should consistently protect themselves from sunlight and other UV radiation.

Visiting sunbeds should generally be avoided. In addition, the use of sun protection creams with a particularly high sun protection factor is recommended. The therapy depends on the type of disease.

If, for example, lupus is caused by medication, these drugs are discontinued if possible. The focus is on cortisone and immunosuppressants. Cortisone is primarily intended to inhibit inflammation in the affected organs, while immunosuppressants are intended to suppress the body’s own defence system.

The latter can be explained by the fact that in lupus our immune system is directed against the body’s own cells. This undesirable effect must be curbed. In the case of cutaneous lupus (i.e.

a lupus that is restricted to the skin) the following are used: If the lupus is one of the most severe types, i.e. a systemic lupus erythematosus, the therapy is designed as follows In any case, a good blood pressure setting is very important in order to maintain the function of the kidney, which is already endangered by the disease itself. In less severe cases, where no organs are affected, painkillers such as ASA or Ibuprofen® plus hydrochloroquine are given to relieve the joint pain. Cortisone is only administered in inflammatory phases.

If there is a severe case with impairment of (vital) organs, the therapy is different. Here, high doses of cortisone are given and the body’s own defense system is suppressed by immunosuppressive agents. Cortisone and immunosuppressants suppress the body’s defense system.

This ensures that the immune complexes which want to fight the deposited DNA do not form in the first place. The body’s defence system is therefore so poor that the trigger of the disease cannot be fought at all. However, strong suppression (suppression) of the immune system must be handled with caution, as there is a high risk of infection for the patient.

Even the slightest cold can be dangerous for these patients. The now suppressed and non-working immune system is no longer able to fight viruses, bacteria and other pathogens.

Retinoids (vitamin A derivatives),
Creams with high sun protection factor and
Cortisone ointments