Systemic lupus erythematosus

Proven content

Systemic lupus erythematosus is a disease linking British singer Seal, three-time gold medalist Shannon Boxx, and host of the American version of Nick Cannon’s Got Talent. However, lupus does not stop them from being professionally active. Can the sick in Poland also live actively?

– I went to various specialists for three years before the doctors managed to make the correct diagnosis – says thirty-year-old Marta Kotarba Kańczugowska, suffering from systemic lupus erythematosus. Meanwhile, specialists emphasize that the earlier the treatment is started, the greater the chance of controlling the disease.

Shortly after the birth of my daughter, Marta developed a fever, enlarged lymph nodes and a reddened face. She was hospitalized in the infectious diseases ward. Doctors suspected it was a viral or bacterial infection. After returning from the hospital, she did not feel well. She was still tired. Therefore, she decided to look for the causes of these ailments. – Since I suffer from myasthenia gravis, the doctors I have visited have associated all my problems with this disease. It never occurred to them that I might have another disease – she says. She was diagnosed with a rheumatologist, dermatologist, nephrologist, hematologist. She was in the hospital five times. Finally, after three years, when she was admitted to the nephrology department due to kidney problems, the correct diagnosis was made – damage to the renal parenchyma caused by systemic lupus erythematosus. Time wasted on improper treatment is impossible to make up for, because specialists emphasize that treating the disease makes sense when it has not yet caused permanent changes.

Systemic lupus erythematosus – symptoms

The Latin name for systemic lupus erythematosus (abbreviated as SLE) is lupus erythematosus systemicus. The name “wolf” probably comes from the fact that in the old days the healers of this disease, the changes on the patient’s body resembled a wolf’s bite. Systemic lupus erythematosus is a chronic, multiorgan rheumatic disease with an immune background. Most often it starts skin lesions in the form of erythema on the face in the shape of a butterfly, joint ailments and anemia. The patient’s immune system begins to act against his own body. The disease is life-threatening when important organs such as kidneys, lungs, skin, nervous system, marrow, liver, heart are affected and damaged. In Poland, it is estimated that 20 people suffer from it. Systemic lupus erythematosus affects mainly women, and the mean age of onset of symptoms is 000 years. Reasons for occurrence lupus erythematosus systemicus are not known. There are periods of exacerbation and long periods of remission in the course of SLE.

Symptoms for individual organs and systems

Joints – Lupus most commonly affects the finger, knee, foot and wrist joints. Sometimes the pain is also accompanied by swelling or exudation. This pain travels from one joint to another. If pain persists for a long period of time, consult your doctor.

Muscles – muscle pain is a very common symptom of lupus. Muscle wasting can often occur.

The skin and mucous membranes – Lupus causes a butterfly-shaped erythema on the face. Most often it becomes active after long exposure to the sun. It is a slight reddening of the nose and cheeks, it can also appear on the neck, cleavage, forehead or around the eyes. This erythema disappears as soon as lupus activity is reduced. The sun can exacerbate the symptoms of the disease (attacks internal organs). In addition to erythema, there may be erosions in the mouth and nose that must be assessed by a doctor. Cutaneous lupus may include urticaria, thickening of the subcutaneous tissue, erythema around the nails, Raynaud’s phenomenon, erythema of the hand or reticular cyanosis. Excessive hair loss and thinning often occur in lupus.

Kidneys – lupus causes nephritis in 1/3 of patients, and consequently leads to failure. This problem largely affects young people and manifests itself in the first 2 years of the disease. Kidney failure is one of the most important causes of mortality in people with lupus.

Heart – lupus causes endocarditis accompanied by a thickening of the valves. Various pathogens often find their place on such changed heart valves. Patients with this problem should take antibiotics to avoid infective endocarditis. Lupus can cause heart attacks and coronary artery disease, often caused by the development of atherosclerosis.

Blood vessels – atherosclerosis: a condition that develops very rapidly in lupus due to its inflammation. Vasculitis: a rare inflammation of the veins or artery walls.

Lungs – we rarely deal with lupus pneumonia. If it does, it is accompanied by shortness of breath, coughing and high temperature. Pulmonary fibrosis may occur, and this should be considered if a dry cough and shortness of breath occur immediately after exercise.

The nervous system – there may be some neurological and psychological symptoms. Problems with memory, planning, reasoning, frequent mood disorders, irritation or depression – these are common symptoms. We often deal with paresis, convulsions or sensory disturbances.

Digestive system – heartburn and abdominal pain can accompany lupus. The preparations used also contribute to the formation of ulcers or bleeding into the gastrointestinal tract. It happens that lupus leads to unpleasant complications in the form of inflammation of the pancreas or peritoneum. When should you be concerned? Report to a doctor immediately if you develop: vomiting, diarrhea, tarry stools, yellowing of the skin, severe abdominal pain.

Serum membranes – pleurisy and pericarditis may develop, which is usually mild.

Eyes Dry eye, sensation of a foreign body under the eyelid. There may be visual disturbances due to certain medications.

Procreation – women with lupus have the chance to get pregnant normally and give birth to a healthy baby. It happens, however, that women who have sick kidneys or heart, highly developed lupus, have a greater predisposition that the child and mother will be at risk. Therefore, it is recommended to plan pregnancy while the disease is alleviating.

Osteoporosis– lupus accelerates the development of osteoporosis.

Antiphospholipid syndrome – it is closely related to antiphospholipid antibodies which increase the risk of blood clots blocking blood flow in the vessels. This syndrome may also develop gynecological complications in the form of recurrent miscarriages. Drugs that reduce blood clotting should be used to prevent them.

Lupus – newborns

Neonatal lupus is caused by maternal anti-Ro (SSA) or anti-La (SSB) antibodies. They cross the placenta, which in turn causes symptoms of the disease in the child. This situation occurs in approximately 3% of all pregnancies. A complication of neonatal lupus may be congenital heart block, therefore the fetal heart should be frequently examined from the 16th week of pregnancy. In addition, newborns may develop jaundice and skin changes.

Lupus – causes

The cause of lupus is not really known. It is said that it is mainly genetic, but has a chance to develop faster when some additional factors are applied:

1. infection: any microorganisms, human retroviruses, certain bacteria or the Espteina-Barr virus;
2. hormones: lupus is more common in women and is associated with estrogens and prolactin, which increase its risk. Lupus can worsen in women who are taking hormone replacement therapy or taking oral contraceptives;
3. certain preparations: drug-induced lupus – some drugs can influence its formation. The list of drugs that can cause this type of lupus is very long, but includes interferon, procainamide, methyldopa, and isoniazid, among others. This type of lupus is quite mild, spares internal organs, and resolves after drug discontinuation.
4. exposure to sunlight – ultraviolet radiation causes damage to epidermal cells, fragments of which are removed by macrophages. In healthy people, this process runs smoothly, while in lupus the removal of dead cells is disturbed, and the remaining fragments become a “target” for autoantibodies; in this way, the cascade of inflammation is triggered and characteristic skin lesions are formed, as well as the involvement of internal organs
5. others: certain chemicals, such as organic solvents, silicon compounds, aromatic amines, as well as a high-fat diet and smoking, can stimulate the development of autoimmune diseases; unsaturated fatty acids and vitamin D have potential protective effects.

Lupus – diagnosis

– The diagnosis of systemic lupus erythematosus due to the heterogeneous clinical picture and rarity is very difficult and requires a number of specialist tests. Early diagnosis is also not conducive to the lack of sufficient knowledge about the disease among primary care physicians who are first treated by patients. Patients with symptoms characteristic of lupus, depending on the type of prevailing symptoms, are referred to a dermatologist, nephrologist or hematologist, instead of a rheumatologist who is a specialist in the treatment of this disease, which further delays the implementation of effective treatment – says Prof. Piotr Wiland, President of the Board of the Polish Society of Rheumatology.

The diagnosis of systemic lupus erythematosus is based on a careful history, laboratory tests, especially the titers of antinuclear antibodies, and various additional tests, e.g. chest radiographs, ultrasound of the heart, blood and urine tests, and imaging of the central nervous system. The mere presence of antinuclear antibodies in the absence of clinical symptoms does not warrant the diagnosis of systemic lupus erythematosus.

The following tests are used:

1. antinuclear antibodies: basic test;
2. antiphospholipid antibodies: important in women planning a pregnancy;
3. syphilitic reaction,
4. inflammatory markers, e.g. ESR, CRP, proteinogram, complement levels (C3c, C4);
5. blood count;
6. coagulation indicators;
7. assessment of kidney function – creatinine, GFR index, urinalysis;
8. liver function assessment, e.g. AST, ALT;
9. lupus band test – examination of a skin section under a microscope;
10. capillaroscopy – for Raynaud’s symptoms. The nail folds are examined under the microscope – changes in the blood vessels present there may indicate the presence of a systemic disease.

Systemic lupus erythematosus – treatment

Marta suffering from lupus does not have one rheumatologist who would deal with her comprehensively. He visits various specialists. – The worst thing is that their opinions are often mutually exclusive. The dermatologist orders you to stop taking medications, and the rheumatologist says you should take them – he says. – In addition, registering with a specialist is not easy. Several months before the end of the year, many centers have exhausted contracts and are no longer enrolling patients. Therefore, if my symptoms worsen, I go to the hospital emergency room. Fortunately, I have a good family doctor who I can always consult – he adds. Meanwhile, it should be remembered that the risk of death from any cause is 2,4-3,4 times higher in the group of patients with systemic lupus erythematosus than in the general population. The most common causes of death are infections and severe organ changes. Therefore, as emphasized by prof. Maria Majdan, head of the Department and Clinic of Rheumatology and Systemic Diseases of the Connective Tissue, Medical University of Lublin, the treatment of SLE in a patient should be largely individualized and adapted to the very different disease conditions of a given person, tailored to the needs of a given patient. – Treatment of systemic lupus erythematosus aims to properly control inflammatory activity to prevent permanent organ damage. At the same time, it is important to minimize the side effects of drugs and the risk of secondary infections, explains Prof. Maidan. Unfortunately, in Poland there are no specialized centers dealing with the treatment of this disease, where the patient could benefit from the advice of a dermatologist, nephrologist and rheumatologist cooperating with each other.

Treatment includes:

1. avoiding exposure to the sun when it is at its peak activity;
2. avoiding stress,
3. rest;
4. healthy eating,
5. maintaining a proper body weight;
6. quitting smoking;
7. prophylaxis of osteoporosis;
8. following the rules of hygiene and vaccination to prevent infections;
9. women planning pregnancy should discontinue preparations that could harm their baby in advance;
10. psychologist: psychological support is very important because lupus is associated with anxiety and fear for the future. Most sick people struggle with this.

Treatment for symptoms

1. muscle and joint pain, general symptoms – non-steroidal anti-inflammatory drugs, severe symptoms: steroids, anti-malaria drugs or methotrexate;
2. skin changes – avoiding long exposure to the sun, using ointments and creams with glucocorticosteroid or tacrolimus. In some cases it is necessary to use systemic immunosuppressants (usually orally);
3. cardiovascular diseases – take medications that lower cholesterol, antidiabetic drugs, omega-3 fatty acids, aspirin or antihypertensive drugs;
4. internal organs – in this case, intensive immunosuppressive treatment and fighting infections are necessary. Control blood pressure, limit protein intake, and quit smoking. Persistent kidney damage requires dialysis and sometimes even a transplant;
5. new therapies – new solutions and drugs effective in the fight against lupus are still being searched for. The effectiveness of, among others, mycophenolate mofetil in the treatment of lupus nephritis and belimumab (a so-called biological agent), which may be beneficial especially in patients with moderately severe lupus;
6. osteoporosis – it is recommended to take large amounts of calcium and vitamin D. In patients with lupus treated with steroids, it is necessary to use drugs against osteoporosis, which reduce the risk of bone fractures.

In order to avoid lupus, you should first of all lead a healthy lifestyle, avoid drugs that can cause it and, importantly, do not stay in the sun for too long.

Prognosis in lupus

Advances in medicine have improved the treatment outcomes of lupus. Modern biological drugs appear. Unfortunately, they are poorly available in Poland. In the 50s, 5 years after diagnosis, less than half of patients with SLE survived. Today, 10 years after lupus diagnosis, 92% of patients are alive. Dr. Maria Rell-Bakalarska from the Rheuma Medicuds Specialist Center for Rheumatology and Osteoporosis in Warsaw emphasizes that good treatment gives the opportunity to lead an active life. Her patient was climbing in the mountains of Thailand. A great example is also the British singer and composer, three-time Grammy Award winner – Seal. He fell ill with SLE in his youth. His own immune system destroyed the skin on his face. However, this did not stop him in his career. Likewise, Nick Cannon, who is mostly known for Men in Black, has the disease, but with his wife Mariah Carey, he is raising the twins and hosting the American version of Got Talent. Also struggling with TRU Shannon Leigh Boxx, an American footballer, and her team won gold medals at the 2004 Summer Olympics in Athens, 2008 in Beijing and 2012 in London.

The end of lupus – and what’s next?

Lupus erythematosus is a chronic disease that requires constant monitoring by a rheumatologist. At the moment, there is no possibility of a complete cure. Sometimes symptoms go away on their own – but this is very rare. When the patient’s health is stabilized, he or she should consult a doctor every six months or every year. Your doctor will evaluate the activity and development of lupus on the basis of various scales (SLEDAI, ECLAM, BILAG, SLICC). Laboratory tests should also be performed to check, for example, the functioning of internal organs. Some medications used require additional control (e.g. ophthalmological control with antimalarial drugs). Women should check themselves at the gynecologist! Dear patients, remember to periodically perform abdominal ultrasound, chest X-ray and mammography. It is important to control blood pressure, diabetes and lipid disorders at least once a year. Do you want to lower the risk of bone fractures? Remember to treat osteoporosis early.