Systemic lupus erythematosus – causes, symptoms, diagnosis, treatment

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Systemic lupus erythematosus (lupus) is a disease that most often affects young women. What connects patron Piotr Korzecki, the beloved of the series Magda M., top model Mercedes Yvette and the black singer Seal? They all suffer from lupus.

Systemic lupus erythematosus (LUPUS) – characteristics

The Latin name of the disease is lupus erythematosus – lupus erythematosus. Lupus is also Latin for a wolf. There are theories that the name of the disease comes from the characteristic changes in the skin of the face, similar to the color of the hair on the wolf’s face, or from the disfiguring scars left over from skin lesions, resembling the bites of this forest predator.

The very course of systemic lupus erythematosus, often characterized by sudden and severe relapses, can also resemble insidious wolf attacks. Systemic lupus erythematosus is a disease of the connective tissue caused by autoimmunity, that is, by turning your own immune system against your own body.

Where does the term “systemic” come from? It is related to the damage of many tissues and organs during the disease. Symptoms of lupus most often appear on the skin, joints and kidneys. The disease itself has a very varied course, it can be both mild and severe – life-threatening. The exact causes of systemic lupus erythematosus are not fully understood, but there is talk of a certain genetic predisposition.

Also read about another variation of lupus: Drug-induced lupus – a rare complication of treatment

Systemic lupus erythematosus – causes

The exact causes of systemic lupus erythematosus are not known. One of the certain pieces of information is that this disease develops in people with a genetic predisposition to develop it with the simultaneous activation of another stimulus, including:

1. exposure to sunlight – ultraviolet radiation causes damage to epidermal cells, fragments of which are removed by macrophages. In the case of healthy people, this process runs smoothly, in the case of people with lupus, the removal of dead cells is disturbed, and the rest of the fragments become the “target” for autoantibodies. As a result, cascades of inflammation are triggered and specific skin lesions are formed. Additionally, internal organs are affected;
2. infection – a number of microorganisms are suspected, incl. human retroviruses, Epstein-Barr virus, certain bacteria;
3. hormonal factors – it should be mentioned that it affects more women than men, and it is related to the influence of sex hormones such as estrogens or prolactin. Interestingly, women taking hormone replacement therapy and oral contraceptives are also at greater risk;
4. leki – some drugs can induce so-called drug-induced lupus (including chlorpromazine, procainamide, methyldopa, interferon, isoniazid or hydralazine). The disease in most cases is mild, without affecting the internal organs and resolving after drug discontinuation;
5. other – Certain chemicals, such as organic solvents, aromatic amines, silicon compounds, a high-fat diet or smoking, can stimulate the development of autoimmune diseases. It is worth adding that unsaturated fatty acids and vitamin D have a potential protective effect.

See also: Experts: Patients with systemic lupus require comprehensive care

Systemic lupus erythematosus – symptoms

Systemic lupus most commonly affects young women between the ages of 20 and 40. Men also get sick, but 10 times less often. It is not a common condition, affecting two to eight in 100 people each year.

However, the insidious nature of the disease manifests itself in the multiplicity of its symptoms and high variability in its course. There is a variant of lupus erythematosus in which it is confined to the skin, but is much more systemic. Then not only the skin is affected, but also joints and internal organs.

1. Kidneys – in 1/3 of patients, nephritis develops, which may lead to kidney failure. It usually affects mainly young people at the very beginning of the disease (the first two years). Nephritis is one of the most important causes of increased mortality in patients with systemic lupus erythematosus. Unfortunately, the disease in its initial stage does not show that it affects the kidneys, therefore it is so important to control their functioning through laboratory tests (urine general examination and blood creatinine level). If kidney involvement is suspected, another test should be performed as well as a kidney biopsy. One of the symptoms of kidney involvement is swelling and weight gain from water retention;
2. Lungs – it is rare, but may develop severe lupus pneumonia (high temperature, shortness of breath, exhausting cough, sometimes haemoptysis), these symptoms require the exclusion of pneumonia caused by infection. It also happens that lupus is the cause of pulmonary fibrosis, which should be taken into account especially in the case of dry cough and shortness of breath after exercise. Other rare complications of lupus include, for example, pulmonary hypertension and “cramped” lung syndrome (when the disease is prolonged, the respiratory muscles become significantly weakened);
3. Serum membranes – inflammation of the lining of the lungs (pleurisy) and of the heart (pericarditis) is very common. The course is usually mild, asymptomatic, sometimes there may be pain in the chest, which will intensify during inhalation. Peritonitis can also develop, causing abdominal pain, nausea and vomiting;
4. The nervous system – during the course of lupus, various neurological and psychological symptoms may develop (neuropsychiatric lupus). Very often there are: mild cognitive impairment (planning, memory, attention or reasoning), anxiety disorders and mood disorders (e.g. irritability or depression). Unfortunately, there are also seizures, sensory disturbances, psychosis or paresis;
5. Heart – Libman-Sacks endocarditis is characterized by the presence of lumps (so-called vegetation) on the heart valves. Most often, there are no symptoms, and people with this problem are advised to use an antibiotic before planned procedures to prevent infective endocarditis. Lupus causes a significantly increased risk of coronary heart disease and heart attack, also in young people. Most often it is caused by the accelerated development of atherosclerosis, less often (in the period of high activity of the disease) it may be the result of inflammation of the coronary vessels;
6. Digestive system – in the case of lupus, abdominal discomfort (e.g. heartburn) or abdominal pain may occur. Some of the medications taken increase the risk of peptic ulcer disease and gastrointestinal bleeding. Lupus can also cause serious complications, such as intestinal infarction or pancreatitis. The alarm symptoms may include, for example, abdominal pain, tarry stools, vomiting, diarrhea or yellowing of the skin;
7. Eyes – in the case of lupus, there is often a feeling of dry eyes or a feeling of a foreign body under the eyelids, related to the so-called dryness syndrome. In addition, some drugs (such as hydroxychloroquine) can cause visual disturbances, which makes regular ophthalmological monitoring in people taking these drugs.

Additionally, other diseases may develop in the course of lupus erythematosus.

1. Osteoporosis – lupus itself and the glucocorticosteroids used in its case significantly accelerate the development of osteoporosis. It is extremely important to start treatment early in order to reduce the risk of bone fractures;
2. Antiphospholipid syndrome – which is closely related to the presence of antiphospholipid antibodies, which appear quite often in lupus. These antibodies influence the tendency to form clots that block blood flow in the vessels;
3. Problems with procreation – women with lupus usually have no problems getting pregnant and carrying them on, but sometimes pregnancy is a threat, both for the mother and her unborn child. This is due to maternal kidney or heart disease, high lupus activity, the presence of an antiphospholipid syndrome, or some type of antibody. In order to avoid unnecessary risks, the pregnancy should be planned in such a way that it takes place during the silence of the disease.

The American Rheumatological Society speaks of sixteen criteria for the diagnosis of generalized systemic lupus erythematosus. Hence, two lupus patients may present completely different symptoms, and their disease may have a completely different intensity.

It often happens that the symptoms of lupus resemble another disease, so Dr. House goes even further in his judgment with the now famous statement: “It’s never lupus.” However, the disease does exist, and young patients often see a doctor because of progressive weakness, fevers, joint pains, worried about a lack of appetite, weight loss or hair loss..

A very characteristic feature of the disease that can speed up diagnosis is facial erythema, often intensified by sunlight, covering the skin of the cheeks and the bridge of the nose, with its butterfly shape.

It often happens that before a patient goes to an immunologist or rheumatologist, who will make a final diagnosis and start appropriate treatment, he has already had a long way to go through many specialists. And so – if skin lesions are dominant – lupus is often suspected by dermatologists who treat forms confined to the skin.

It also happens that initially, if they dominate general symptoms (weakness, weight loss, fever)accompanied by serious disturbances in the blood composition and generalized enlargement of the lymph nodes, the patient goes to hematologists who, after excluding diseases such as leukemia and lymphomas, suspect lupus.

Often, when fever is accompanied by joint pain, lupus is suspected by infectious disease physicians who fail to find any infectious agent responsible for the observed ailments. In some cases, there is no time to wonder, because the onset of the disease is very acute and there is a violent life-threatening damage to internal organs.

The appearance of symptoms of lupus erythematosus is an urgent indication for a doctor’s appointment. Patients suffering from this type of disease should remain under constant medical care.

When should you see a doctor? When you have experienced any of the symptoms listed below:

1. abdominal pain, vomiting, dark-colored stool (or with blood), yellowing of the skin – indicate the need for diagnostics of abdominal diseases;
2. cough, chest pain, shortness of breath, decreased exercise tolerance – indicate the need for diagnostics for lung and heart diseases;
3. any signs of infection (fever, cough, sore throat, burning sensation when urinating, weakness, fracture). Drugs for lupus and lupus itself deteriorate the body’s ability to fight germs, making infections requiring a quick and effective response;
4. any neurological symptoms (e.g. abnormal sensation, weakness or weakness in limbs, severe headache, visual disturbances). They may indicate a stroke or other neurological disease;
5. the appearance of new symptoms or those that are already starting to worsen is a sign that the disease is getting worse and will require more intensive treatment.

Find out more: How to fight autoimmune diseases?

Systemic lupus erythematosus – diagnosis

The diagnosis of the development of systemic lupus erythematosus is usually quite difficult. At the very beginning, the diagnosis is usually related to connective tissue disease, and over time, with the appearance of other symptoms, lupus is diagnosed. To recognize this disease and assess its activity, doctors use the following tests:

1. blood count,
2. syphilis (despite the absence of syphilis, this reaction is often positive in people with lupus),
3. lupus band test – a test characterized by examining a section of the skin under a microscope (especially useful when the picture of the disease is unclear),
4. capillaroscopy – this test is performed when the patient has Raynaud’s phenomenon; it consists in analyzing the nail shafts under a microscope,
5. ESR, CRP, the level of complement components (C3c, C4), proteinogram, i.e. indicators of inflammation,
6. coagulation indicators,
7. sometimes imaging tests (ultrasound, MRI, X-ray), rheumatological or virological examination,
8. tests to assess kidney function, creatinine, GFR, urinalysis;
9. AST, ALT (tests to assess liver function);
10. antinuclear antibodies;
11. antiphospholipid antibodies.

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Systemic lupus erythematosus – treatment

Since systemic lupus erythematosus is an autoimmune disease whose causes remain unknown, only the symptoms can be treated. The treatment of choice is the use of drugs aimed at suppressing the abnormal activity of the immune system, the so-called immunosuppressive drugs.

Since lupus varies in its course and severity in every patient, treatment is selected individually and should be carried out by a specialist doctor – an immunologist or rheumatologist. Treatment is often chronic, and the disease itself can never be completely cured, although it is possible to achieve – sometimes many years – remissions and periods when the use of drugs is not necessary.

The first-line drugs are steroid drugswhich, however, are not ideal drugs due to their numerous side effects (risk of diabetes, hypertension, steroid osteoporosis, weight gain due to water retention and increased appetite, face rounding, stretch marks). Depending on the stage of the disease and the occurrence of organ damage, other immunosuppressive drugs are also added to the treatment.

In recent years, research has also been conducted on new drugs, the so-called biological medicines that are intended to target specific cells of the immune system that are not working properly in the disease. However, the prognosis for lupus has improved significantly since the introduction of modern treatments. Until relatively recently, before 1955, five-year survival after diagnosis was achieved in 50% of patients, while the current 10-year survival in patients diagnosed with systemic lupus is 90%.

General principles of treatment recommended for patients with lupus

1. Avoiding stress.
2. Lots of rest.
3. Avoiding UV radiation and exposure to the sun during its peak activity.
4. Wearing appropriate clothing and headgear, and using UV-blocking creams and lotions.
5. Before a planned pregnancy, consultation with a doctor (possible discontinuation of drugs that may harm the fetus and performing appropriate tests). Pregnancy should occur during the disease remission period.
6. Physical activity.
7. Eating healthy, maintaining a healthy body weight, quitting cigarettes.
8. Prevention of osteoporosis.
9. Avoiding contamination.
10. Observance of hygiene and preventive vaccinations, e.g. against influenza (vaccinations should be performed in the period of low lupus activity). 
11. Mental help / support – the diagnosis of systemic lupus is a shock and shock for many people, therefore the help and support of relatives / qualified people is very important. For example, behavioral therapy is very effective. 

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Systemic lupus erythematosus – post-treatment management

Systemic lupus erythematosus requires constant rheumatological control. At the beginning and during an exacerbation, the patient needs frequent visits to the doctor to be able to take the amount of drugs and achieve remission of the disease. During the normal period of illness, visits are usually every 6 or 12 months.

Various scales such as BILAG, ECLAM, SLEDAI or SLICC are used to assess the progression and activity of the disease. Laboratory tests are performed to assess the functioning of internal organs and the activity of inflammation. Some of the drugs used in treatment require additional control, such as eye control with antimalarial drugs.

In addition, regular gynecological check-ups are recommended in the case of systemic lupus erythematosus. From time to time, tests such as chest X-ray, abdominal ultrasound or mammography are also carried out. Due to the accelerated development of atherosclerosis, it is necessary to assess the cardiovascular risk, e.g. regular monitoring of blood pressure, tests for diabetes mellitus. You should also not forget to treat osteoporosis in a timely manner, which will reduce the risk of bone fractures.

Are you unable to make a normal visit? Check how to prepare for an e-visit at the gynecologist?

Systemic lupus erythematosus – prevention

At the moment, we do not know the steps to prevent the development of systemic lupus erythematosus. It is said that it is good to avoid excessive exposure to the sun’s rays and follow the generally accepted rules of the so-called healthy lifestyle. Drugs that can induce drug-induced lupus should also be avoided if possible.

Systemic lupus erythematosus – inheritance

Most people assume that the onset of systemic lupus erythematosus is due to a combination of many factors. One of these factors is genetic predisposition, although they are not sufficient for the disease to manifest itself.

This can be seen very well with twins. In identical twins, the chance of developing the disease is 25 percent, and in fraternal twins it is 2 to 5 percent. It is also a fact that systemic lupus erythematosus can also occur in people whose family has never had any history of it.

See also: Certain X-linked diseases

Lupus erythematosus and pregnancy

As lupus is primarily a disease of young women, they often ask if they will be able to get pregnant despite the disease. Certainly women with lupus can have children, but when to get pregnant the timing should be very carefully chosen. Hence, constant care of a specialist doctor, who should be informed about the planned maternity, is of great importance. He will assess whether a given moment is safe for pregnancy, or whether the damage to internal organs caused by the current course of the disease does not pose a threat to the life and health of the future mother and her child, will perform the necessary specialist examinations and determine what treatment will be the safest during this period.

However, it should be remembered that the disease itself and the drugs used in its treatment are associated with an increased rate of gynecological failure. During pregnancy, the disease may exacerbate, which is a dangerous situation for both the mother and her child, therefore both of them need specialist care during this time.

When is systemic lupus erythematosus suspected?

What Indicates Lupus? Answer yourself 10 questions:

1. Have you ever had joint pain for more than 3 months?
2. Do your fingers go numb, turn pale, or start to ache when exposed to cold?
3. Have you ever had mouth sores that lasted more than 2 weeks?
4. Have you ever been diagnosed with blood disorders (anemia, low white blood cell or platelet counts)?
5. Have you ever had a raised rash on your face that lasted more than a month?
6. Does skin rash develop after sun exposure (without sunburn)?
7. Have you ever had pain with deep breathing (pleural pain) for more than a few days?
8. Have you ever been found to have protein in your urine?
9. Have you ever experienced sudden hair loss?
10. Have you ever had a seizure or seizure?

If your answer to at least three of the above questions is positive, it is worth asking an immunologist or rheumatologist and considering the need for specialized tests – including checking for the presence of antinuclear antibodies.

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