Lupus – causes, symptoms, diagnosis, treatment, possible complications

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Lupus is an autoimmune inflammatory disease often accompanied by characteristic erythematous skin lesions. For this reason, the disease is referred to as lupus erythematosus, but this systemic connective tissue disease often affects joints, kidneys, lungs, heart and other organs or organ systems. The exact causes of lupus are still unknown, and there is no specific method that can cure it completely. So how is lupus diagnosed?

Lupus – what is this disease?

Lupus belongs to the group of autoimmune diseases, which means that the immune system plays an important role in its development. The immune system is a complex network of organs, tissues and cells. It protects the body by fighting foreign bodies such as viruses, bacteria, fungi and toxins. It also removes dead or defective cells. The immune system protects the body by producing Y-shaped proteins called antibodies. These antibodies either neutralize the threat or send a signal to other cells to remove it. Specialized white blood cells, called B lymphocytes, make these antibodies.

When a person has an autoimmune disease such as lupus, the immune system cannot distinguish between unwanted substances or antigens from healthy tissue. As a result, the immune system targets antibodies against both healthy tissue and antigens. This causes swelling, pain and tissue damage.

Antinuclear antibodies (ANA) are commonly found in people with autoimmune diseases such as lupus. They work by targeting the nucleus of the body’s own cells. The nucleus is the part of the cell that contains genetic material. Resulting from such a reaction of the body the disease process may involve various organs and their entire systems. People with lupus can therefore complain of a wide variety of symptoms, ranging from skin lesions to fever and inflammation to acute changes that can lead to organ failure.

Lupus is chronic and requires long-term treatment. In the course of the disease, periods of exacerbation and remission (withdrawal of symptoms of the disease) are often observed, but there are also very severe cases. The prognosis of lupus patients depends on the individual characteristics of the case, timely diagnosis of the disease and implementation of appropriate treatment.

See also: Immunology – types, immunological tests, diagnostics

Where does lupus come from?

The exact causes of lupus are not fully understood. It is not clear why the immune system begins to attack tissues and organs in some people. It is suspected that the predisposition to developing lupus is genetically determined, and the development of the disease is additionally influenced by factors such as hormones, certain medications and environmental factors (e.g. viral infections or solar radiation).

One possible theory of lupus formation involves cell death, a natural process that occurs when the body renews its cells. Research suggests that due to genetic factors, the body of people with lupus may not be able to remove the dead cells properly. The dead cells that remain can lead to the production of autoantibodies, such as ANA, that attack the body, causing symptoms of lupus.

Lupus – risk factors

Lupus can develop in response to several factors. They can be hormonal, genetic, environmental, or a combination of these factors.

Lupus and hormones

Hormones are chemicals produced by the body. They control and regulate the activity of certain cells and organs. Hormonal activity can explain risk factors such as gender and age.

Women between the ages of 15 and 44 are estimated to be nine times more likely to develop lupus than men. Symptoms and diagnosis often appear between the ages of 15 and 45 in childbearing age. However, 20% of cases appear after the age of 50.

Some studies, such as those published in the journal PeerJ in 2019, suggest that exposure to estrogen, a sex hormone that women produce more than men, may increase the risk of autoimmune diseases such as lupus. This theory is also supported by the fact that many women experience more lupus symptoms before menstruation and during pregnancy, when estrogen levels are higher.

While more research is required, the presence of certain sex hormones may explain why lupus is so common in adult women.

Lupus and genetic factors

While more research is needed, scientists have identified certain genes that play a role in the immune system response that may contribute to the development of lupus. Evidence suggests that a person is more likely to develop lupus if a family member has it, further supporting the possibility that genetics may be a risk factor.

Twin studies also show that if one identical twin has lupus, the other has a 24% chance of developing the disease. Of course, lupus can occur in people with no family history of the disease, but other autoimmune conditions can run in the family.

Lupus risk factors include a combination of genetic and environmental factors. Lupus, which is more common in some demographic groups, may be due in part to genetics. However, differences in some outcomes, such as disease progression and mortality, may be due to social factors such as healthcare inequalities.

Lupus and environmental factors

Environmental factors such as chemicals or viruses can contribute to triggering lupus in people who are already genetically susceptible.

Possible environmental factors can have many aspects.

1. Smoking: Exposure to the toxic components of cigarette smoke can lead to genetic mutations and the activation of genes associated with SLE.
2. Exposure to sunlight: Radiation from direct sunlight can worsen symptoms of pre-existing SLE. However, more research is needed to see if it may be a risk factor for developing SLE. UV radiation can lead to cell damage and genetic mutations that may be involved in the development of SLE.
3. Infections: Infections such as the Epstein-Barr virus, also known as glandular fever (mononucleosis), are much more common in people with SLE. This suggests that infection may play a role in the development of the condition in susceptible individuals.
4. Pollution: Like cigarette smoke, air pollution can be a risk factor for the development of SLE.

When is lupus revealed?

Lupus erythematosus can affect people of all ages, but is most often detected in women between the ages of 16 and 55. When the disease manifests itself in people aged 14–18, it is referred to as juvenile systemic lupus. Lupus cases are also observed in younger children, even before the age of 10. Lupus erythematosus diagnosed in newborns and infants is quite a rare disease, and usually of a mild course.

Juvenile lupus is extremely aggressive – in its case, symptoms intensify and complications within the organs develop rapidly. Intensive immunosuppressive treatment is often necessary, and negative side effects are the consequence of long-term therapy of this type.

Lupus erythematosus – what forms does it occur in?

People suffering from lupus face various forms of the disease, because it can be limited to the skin (e.g. discoid lupus), but also affect organs and tissues – then we are talking about systemic or visceral lupus erythematosus. If skin lesions suggestive of lupus are diagnosed, diagnosis for organ lesions may be indicated. Observation and further research is necessary because lupus comes in many forms and causes diagnostic problems.

This condition may present as systemic lupus erythematosus (SLE) (SLE), the most common form of lupus, accounting for 70% of lupus cases with immune disorders, and research indicates the presence of ANA antinuclear antibodies. A sick person may suffer from various symptoms – both erythematous skin lesions and coexisting changes in organs.

In the initial phase, systemic lupus erythematosus is manifested in many cases by changes in the areas of the skin most often exposed to sunlight. The condition usually goes through cycles of exacerbation and remission. During periods of remission, a person with lupus may not have any symptoms. During an exacerbation, the disease is active and the person’s symptoms return or new symptoms may appear. Some people with SLE may have flare-ups on a regular basis. Others may only experience them every few years. Still others may experience prolonged disease activity.

As for the skin lesions accompanying SLE, they most often appear in the following forms:

1. acute cutaneous lupus – it is very common in people with lupus (up to 70% of cases), butterfly-shaped erythema on the face (nose and cheeks) is typical, but changes (also maculopapular, epidermal necrolysis or blisters) can also affect the forearms, neck, cleavage or face and become tougher as a result of sun exposure;
2. subacute cutaneous lupus – appears in about 20 percent in patients, it has the form of exfoliative papular lesions and red rims with a light interior; it appears on the torso, limbs, décolleté or neck, but rather not on the face;
3. lupus in the form of deep subcutaneous – these are inflammatory bumps that appear under the skin (mainly on the face and limbs) and leave dimples after they resolve; usually lesions appear in parallel with acute or chronic lupus;
4. discoid lupus erythematosus – Chronic lupus, usually confined to the skin, is in the form of discrete discs on the neck and face that leave behind scars. Occasionally, the lesions also appear on other parts of the body, such as inside the ears. Lesions tend to be reddened and can become thick and scaly. If the lesions heal on the scalp, hair may not grow back in that area. This form does not affect internal organs, but according to the Lupus Foundation of America, about 10% of people with discoid lupus later develop systemic lupus erythematosus. However, it is possible that these people already had TRK and that skin symptoms led to the diagnosis of SLE.

Specific types of lupus are those that occur in certain age groups and appear as a result of the use of certain medications:

1. Neonatal lupus erythematosus (NLE). It develops in newborns and infants whose mothers have anti-Ro / SSA, La / SSB or U1 (U1-RNP) autoantibodies. Antibodies that can cause neonatal lupus erythematosus are associated with lupus and Sjögren’s syndrome. The biological parent may have any of these conditions or not have any symptoms, but can still pass on these antibodies. When this occurs, the likelihood of an infant developing lupus is 2%. It is a rare autoimmune disease that is usually mild and goes away on its own after a few months. This mainly applies to skin lesions that are visible immediately after birth or become apparent within a few weeks. However, if NLE affects certain systems or organs, such as the heart, the consequences of the disease can be serious. After birth, infants with NLE may develop a skin rash, liver problems, and low blood cell counts. We are talking about a situation where infants have a congenital heart block in which the heart cannot regulate the normal and rhythmic pumping of blood. This is a more serious complication and the infant may need a pacemaker. Most infants born to biological parents with SLE are perfectly healthy. However, an expectant parent with SLE may want to talk to a doctor about how her condition might affect the pregnancy.
2. Juvenile systemic lupus (otherwise: juvenile visceral lupus). This type of lupus is usually diagnosed when it appears between the ages of 14 and 18. The disease is more severe than the adult forms of lupus – symptoms are more rapid, complications develop rapidly, and treatment is long and burdensome for the body. Juvenile lupus most often affects young women, but it also occurs in teenage men and is even more severe in their case.
3. Drug-induced lupus. This form of lupus can develop as a result of some medical treatments, and most commonly affects people aged 50–70 years. The symptoms of this form of the disease resolve when the drug causing them is stopped (usually within 6 months of stopping treatment). Medicinal agents that can cause drug-induced lupus include procainamide (a drug for heart arrhythmia), hydralazine (a drug for high blood pressure), methyldopa (an antihypertensive drug), diltiazem (a drug used to treat cardiovascular diseases), antimalarial drugs, anti-tuberculosis drugs and some antipsychotics . The symptoms are similar to those in SLE, but are often less severe. Symptoms of this form of lupus usually develop after months to years of continued treatment with these drugs. About 5% of those taking hydralazine and 20% of those taking procainamide for long periods develop the drug-induced form. Although other drugs can trigger TP, the likelihood is much less.

See more: Skin lesions in internal diseases – diseases, photos

Lupus – symptoms of the disease

The symptoms of lupus patients usually concern skin lesions of a different nature, and a wide variety of systemic symptoms. The appearance of the lesions and the nature of other symptoms may vary depending on the form of the disease.

One of the most characteristic symptoms of lupus is the butterfly-shaped erythema in the acute form on the face. Other erythematous changes are also observed, including those located around the nails or disc erythema. Additionally, rashes (e.g. macular and macular), hives, purpura, blisters and mouth ulcers are possible. In the course of lupus, there are general symptoms such as fatigue, fever, nausea, vomiting, weight loss, anemia or enlargement of the lymph nodes.

Other signs of the disease may be associated with specific systems – one organ may be affected in the affected person (e.g. in the initial stage of the disease), but there is also the likelihood of multiple organ changes. Examples of symptoms of systemic lupus erythematosus that are assessed by a physician diagnosing or treating lupus include:

1. arthritis and progressive joint pain;
2. muscle inflammation;
3. edema, including angioedema;
4. respiratory symptoms – shortness of breath, pleurisy, pneumonia;
5. kidney problems – proteinuria, hematuria, high creatinine, urine rolls;
6. anemia, leukopenia, lymphopenia, thrombocytopenia;
7. heart abnormalities including arrhythmia and inflammation of the heart muscle or pericardium;
8. circulatory disorders – vasculitis, cyanosis, thrombosis without strokes;
9. neuropsychiatric symptoms – seizures, psychosis, peripheral or cranial neuropathies, migraines, persistent (acute) headaches, stroke, organic brain disease;
10. gastrointestinal symptoms – abdominal pain, intestinal disorders (ischemia, vasculitis), pancreatitis or sterile peritonitis;
11. eye problems – retinal or choroidal effusion or haemorrhage, inflammation of the optic nerve;
12. hypocomplementemic;
13. increased ESR

Lupus symptoms in women

Lupus may present differently from person to person. Evidence suggests lupus may present differently in men and women. Women tend to have less severe symptoms than men.

Symptoms that are more common in women include:

1. hair loss;
2. sensitivity to sunlight;
3. mouth ulcers;
4. arthritis;
5. erythema on the face in the form of a butterfly.

Lupus symptoms in men

There is a common misconception that only women can have lupus. Although less common, men can also develop lupus. What’s more, research suggests that men with lupus tend to have more severe symptoms.

Symptoms that are more common in men include:

1. cardiovascular complications;
2. low blood cell count;
3. weight loss;
4. kidney complications;
5. pain in the chest.

Lupus and its impact on individual organ systems

Lupus can also affect how certain organs work, such as the nervous system, kidneys and lungs.

1. Kidneys: Lupus can cause a kidney disease called lupus nephritis, in which inflammation prevents the kidneys from working properly. Symptoms may include swelling in the legs, feet and face, frequent urination, and high blood pressure.
2. Lungs: Some people may experience inflammation of the lining of the lungs, known as pleurisy, which causes chest pain when deep breathing. Some people can also develop pneumonia.
3. Central nervous system: Lupus can sometimes affect the brain or central nervous system. Symptoms can include brain fog (confusion and trouble concentrating), headaches, seizures and strokes. Lupus encephalitis may also occur, causing acute confusion, cognitive impairment, mood changes, seizures, lethargy and coma.
4. Blood vessels: lupus can lead to inflammation of the blood vessels.
5. Blood: Lupus can cause anemia, leukopenia (reduced number of white blood cells), or thrombocytopenia (reduction in the number of platelets that help blood to clot).
6. Heart: If inflammation affects the heart, it can cause myocarditis and endocarditis. It can also affect the membrane that surrounds the heart, causing pericarditis. This can cause symptoms including chest pain. Endocarditis can damage the heart valves, causing the valve surface to thicken and holes to form. This could eventually lead to a heart murmur.

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Lupus – complications

There are various complications that can result from lupus.

Lupus complications – infection

Infection is more likely when a person has lupus because both the disease and its treatment weaken the immune system. Common infections include:

1. urinary tract infections;
2. respiratory infections
3. yeast infections;
4. salmonellę;
5. herpes;
6. shingles.

See also: The first symptoms of a respiratory infection that must not be ignored

Lupus complications – bone necrosis

This happens when there is low blood flow to the bones, which causes tiny cracks to form. Eventually, it can lead to the bones collapsing. People with lupus may be at increased risk due to the condition itself and medications, such as corticosteroids, which are used to treat lupus.

Lupus complications and pregnancy

People with lupus have a higher risk of pregnancy loss, premature birth, and pre-eclampsia, a dangerous condition that includes high blood pressure. To reduce the risk of these complications, doctors often recommend delaying pregnancy until lupus is under control for at least 6 months.

Lupus – diagnosis

Diagnosing lupus can be difficult because of the wide variety of symptoms that may resemble those of other medical conditions.

In the beginning, the doctor asks about symptoms, performs a physical examination, and will focus on personal and family medical history. In addition, your doctor may order some blood tests and other laboratory tests.

Biomarkery

Biomarkers are antibodies, proteins, genetics, and other factors that can show your doctor what is happening in your body or how your body responds to treatment. They are useful because they can indicate whether a person has a certain condition, even when there are no symptoms. Lupus affects people in different ways. This makes it difficult to find reliable biomarkers. However, a combination of blood tests and other tests can help your doctor confirm the diagnosis.

Blood tests

Blood tests can show if certain biomarkers are present, and biomarkers can provide information about what autoimmune disease a person has.

1. Antinuclear antibody: About 95% of people with lupus will be ANA positive. It should be added, however, that there are cases of people who have tested positive in the ANA test and have not had lupus.
2. Antiphospholipid antibodies (APL): APL is a type of anti-phospholipid antibody. APL is present in up to 50% of people with lupus. People without lupus can also have APL.
3. Anti-dsDNA Test: About 30% of people with lupus test positive for these antibodies. The result is more likely in an exacerbation. It occurs in less than 1% of people without lupus, and is rare in people with other rheumatic diseases. Therefore, it is a helpful test to confirm a diagnosis of lupus.
4. Anti-Smith antibody: About 20% of people with lupus have an antibody to Sm, a ribonucleoprotein that is present in the nucleus of the cell.
5. Anti-U1RNP antibody: About 25-30% of people with lupus and less than 1% of people without lupus have anti-U1RNP antibodies. This antibody may be present in other autoimmune conditions.
6. Anti-Ro / SSA and anti-La / SSB antibodies: 30-40% of people with lupus have anti-Ro / SSA and anti-La / SSB antibodies. These antibodies are not specific to lupus, but may indicate certain symptoms, such as extreme sun sensitivity.
7. Antihistone antibodies: Histone antibodies are proteins that play a role in the structure of DNA. People with drug-induced lupus usually have it, and people with systemic lupus erythematosus may have it. However, these antibodies do not necessarily confirm a diagnosis of lupus.
8. The complement system: This test measures the levels of proteins your body uses when inflammation occurs. If a person has a low level, this suggests that there is inflammation in the body.
9. Non-specific tests: Rheumatologists may choose to run tests that look for markers of inflammation, including C-reactive protein and erythrocyte sedimentation rate.

In addition to blood tests, further tests may include urine tests, diagnostic imaging, and a biopsy.

1. Urine tests: Urine tests can help doctors diagnose and monitor the effects of lupus on the kidneys. The presence of protein, red blood cells, white blood cells, and rollers can help show how well your kidneys are working. For some studies, only one sample is needed. For others, it may be necessary to collect samples within 24 hours.
2. Tissue biopsies: Your doctor may also request a biopsy, usually of the skin or kidneys, to check for damage or inflammation.
3. Imaging tests: X-rays and other imaging tests can help doctors see organs affected by lupus.

Lupus – treatment

Lupus lesions can usually be effectively treated, although scarring is inevitable with disc lesions. In the case of remaining lesions, spots or papules, local treatment and avoiding UV radiation gives a chance to effectively deal with the spots and lesions. Topical treatment with steroids is used, sometimes (with stronger lesions) also with oral steroids or antimalarial drugs. Sulfones are also administered (for blisters and urticaria), and in the second and second rounds, retinoids, azathioprine, thalidomide or cyclosporine.

On the other hand, controlling the inflammatory process in the body, fighting relapses and preventing complications of the disease turns out to be a big challenge in the treatment of lupus. Treatment is selected depending on the symptoms and their severity. In mild cases, it is used, inter alia, NSAIDs or antimalarial chloroquine and hydroxychloroquine. Severe cases of lupus is treated with glucocorticoids and immunosuppressants (high-dose corticosteroids such as prednisone and drugs that suppress the immune system such as mycophenolate and methotrexate). This treatment aims to target a specific part of the immune system to control inflammation.

Your doctor may also prescribe medications to treat complications that often occur in people with lupus, such as medications for seizures, antibiotics for infections, and vitamin D to help improve kidney function.

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Lupus – home remedies

Because inadequately treated lupus can lead to significant organ damage, doctors often recommend home remedies and lifestyle changes in conjunction with certain medications.

One possible way a person can manage lupus symptoms at home is through diet. While research into the effects of diet on lupus is limited, some evidence suggests that it may play a role in treating the disease.

Patients should aim for a balanced and varied diet that includes fruits and vegetables, whole grains, and a moderate amount of protein. A given lupus can vary greatly from person to person, so a person with lupus should talk to a doctor about what diet may be best for them.

Some changes a person may make to their diet to help manage their condition include:

1. omega-3 fatty acids;
2. reduction of cholesterol and saturated fat;
3. reducing the amount of sodium;
4. taking vitamins such as vitamin D and B vitamins

Further lifestyle changes a person may make to help manage their condition include:

Avoiding smoking: Smoking can damage many organs in the body and lead to worsening of lupus symptoms.

Avoiding sun exposure: sun exposure can be a problem for many people with lupus. Avoid going outside when the sun is “hot” most, wear protective clothing (long sleeves and a brimmed hat), and wear sunscreen.

Consuming less alcohol: Alcohol can make some medications less effective and affect the health of your liver. You should talk to your doctor about how alcohol may interact with your treatment and your current health status.

Performing moderate exercise: light exercise, such as walking, swimming, Pilates, and yoga, can help people to strengthen their body without risking their arthritis getting worse. Moderate exercise can support the health of the heart, lungs, bones, and joints, and aid the mental health of those who are coping with the stresses of the disease.

Caring for social relationships: Research suggests that 43% of people with lupus are concerned about social isolation, and 30% believe that the emotional impact of their disease is misunderstood. A person with lupus can benefit from having an understanding support system around them. It could be family, friends or the Internet community.

Coping with stress: Stress can trigger lupus flare-ups. Reducing stress and developing coping skills can help reduce or prevent flare-ups.

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Lupus – what is the prognosis?

In the past, people suffering from lupus had to take into account high mortality in the first few years of the disease – in the 50s, only 5% were able to survive 50 years from diagnosis. sick people. Currently, the prognosis among patients with systemic lupus erythematosus is much better, as 10% survive 85 years after the diagnosis of the disease. patients. The chance of surviving 20 years from the diagnosis of the disease has 75%. sick.

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Is it really lupus?

Diagnosing systemic lupus is not always easy. You may need to differentiate lupus from other diseases, especially dermatoses (skin conditions). During the diagnosis of lupus erythematosus, the following diseases are considered:

1. rosacea;
2. photosensitization reactions, e.g. multiforme light rashes;
3. dermatomyositis;
4. psoriasis;
5. pemphigus erythematosus;
6. cutaneous forms of granulomatous diseases (e.g. sarcoidosis, annular granuloma);
7. in newborns: seborrheic or atopic dermatitis, neonatal acne, erythema multiforme, dermatophytosis, congenital syphilis, congenital rubella, Langerhans cell histiocytosis, Bloom syndrome, Rothmund-Thomson syndrome.