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World Systemic Lupus Erythematosus Day is set for 10 May and aims to raise awareness of the disease, its complications and treatment measures.
What is lupus erythematosus?
Lupus erythematosus is an autoimmune inflammatory disorder, which occurs when the immune system, through the production of autoantibodies, attacks the healthy cells and tissues of the body itself.
It is classified into 4 categories, based on the course of the disease, the body systems involved and the types of skin lesions:
- Systemic lupus erythematosus (SLE)
- Subacute cutaneous lupus erythematosus (SCLE)
- Discoid lupus erythematosus (DLE)
- Lupus panniculitis/deep lupus (lupus profundus)
What is Systemic Lupus Erythematosus (SLE)?
SLE, which we will discuss in this article, is the most common and serious form of lupus. It affects one per 100,000 people per year. It affects all ages, but is most common between the ages of 15 and 45. The ratio of women to men is about 9:1.
It is a chronic, systemic, inflammatory disease, with fluctuations in its activity, known as flares and remissions. It can affect people of any age, including neonates. It affects many different systems of the body, including the skin, joints, blood, heart, blood vessels, brain, lungs and kidneys. Although it is a chronic condition, this does not mean that the patient cannot lead a normal life. It may not be curable, but it is possible to control it with appropriate treatment.
What are the causes?
The aetiology of the disease is not known, nor can it be predicted who will get it. What is certain is that there is some disorder in the body's immune system, which, instead of protecting it, produces autoantibodies against it. These antibodies attack the tissues of the body, causing inflammation, damage and eventually the symptoms of the disease.
The causes of this disorder are multifactorial. They have to do with genetic and environmental factors. Environmental factors can be viral infections, exposure to sunlight, chemicals and metabolic disorders related to lifestyle and diet. Certain drugs can cause a reversible SLE-type syndrome, which almost always disappears with discontinuation of the responsible drug. Female hormones also play an important role in the development of the disease. That is why women are predominantly affected. Finally, studies show that mental stress from stressful events in life are linked to the onset or flare-up of the disease.
Some studies show that some people inherit the tendency to get lupus. However, there is no evidence that it is transmitted directly, for example from mother to daughter.
How does SLE manifest itself?
The symptoms experienced by each patient with SLE are different. Its course usually has flares and remissions.
Painful or swollen joints, fever and skin rashes accompanied by malaise are the usual manifestations of SLE. It is typical that about half of the patients have a reddening of the cheeks, while very few have the typical "butterfly" rash, which extends to the cheeks and the bridge of the nose.
Patients with SLE may have any of the following manifestations:
- Systemic (fatigue, headaches, fever, arthralgia, weight loss/gain)
- Musculoskeletal (arthralgia, myositis, myalgia, arthritis)
- Dermatological (mucocutaneous lesions, facial rash, photosensitivity, lupus discoides, mouth ulcers)
- Renal (kidney failure, kidney disease)
- Neuropsychiatric (encephalitis, convulsions, psychosis)
- Pulmonary (pleurisy, pleural effusions, pneumonitis, pulmonary hypertension)
- Gastrointestinal (nausea, indigestion, abdominal pain)
- Cardiac (pericarditis, myocarditis)
- Haematological (autoimmune haemolytic anaemia, cytopenias). Cytopenias lead to an increased tendency for infections, anaemia and easy bleeding. Other individuals have an increased tendency to form blood clots.
Other general symptoms of the disease may include:
- Swelling in the hands, feet and around the eyes
- Hair loss (alopecia)
- Swollen lymph nodes
- Orogenitis
- Raynaud's syndrome (Raynaud's) - A disorder of blood circulation in the hands and feet in which the fingertips become pale or blue.
How is the diagnosis made?
Sometimes it is difficult to diagnose the condition, especially in people with mild symptoms. A complete history, together with a physical examination and the results of special tests, are what finally establish the diagnosis. The tests performed are:
- Laboratory tests
- Blood tests
- Measurement of autoantibodies, mainly ANA, anti-dsDNA, anti-Sm, anti-RNP, anti-Ro(SSA), anti-La(SSB), and antiphospholipid antibodies.
- Urine analysis
- Imaging tests
- Chest X-ray
- Heart ultrasound
- Biopsy
Taking a tissue sample from the skin or kidney.
What is the treatment?
Each patient with systemic lupus erythematosus is a unique case from a therapeutic point of view. In determining treatment, careful discussion of the benefits and risks of treatment between the patient and the treating physician is required. The physician in charge must be in constant communication with physicians from various specialties so that the patient receives the best possible care for each problem.
The treatment programme for lupus includes medication, rest, caution in sun exposure, proper nutrition and psychological support. In more detail:
- Medication
- Non-steroidal anti-inflammatory drugs
- Antimalarial drugs
- Corticosteroids
- Immunosuppressants
- Biological factors
- Anticoagulants
- Medicines that involve damage to a specific organ
The goals of drug therapy are to control symptoms, reduce the intensity of inflammation and damage to organs that may be affected, suppress the immune system and reduce recurrences. Any side effects, which result both from the drugs themselves and from immune suppression, must be detected and treated in a timely manner.
- Protection from the sun
Sun exposure makes skin symptoms worse in some people. Therefore, patients should take certain precautions.
- Diet
A balanced diet is an important part of the treatment programme. A diet that is rich in nutrients and fish oil can have beneficial effects on the course of lupus, preventing inflammation from developing.
- Psychological support
There is no doubt that every chronic illness is accompanied by psychological and emotional burden. Therefore, it is very important that these people have support from mental health specialists.
What is the prognosis of the disease?
The outcome of SLE improves dramatically with early diagnosis and proper treatment. Many patients with childhood-onset SLE have a very good outcome. In general, however, the disease can be severe and life-threatening and can remain active throughout a person's life.
Pregnancy and Systemic lupus erythematosus
Although pregnancy in women with SLE is considered high-risk, recent research has shown that most women with mild to moderate SLE go into labour safely. Ideally, a woman with SLE should become pregnant if her disease is in complete remission, both clinically and laboratory. However, in some women, during pregnancy or in postpartum, an exacerbation of the disease may occur.
In general, fertility or the ability to conceive may be reduced during disease flares. In women with specific antibodies, miscarriages are more common. Therefore, they must receive appropriate treatment. However, the drugs the patient is taking must be monitored to ensure that they are not toxic to the foetus. Therefore, co-monitoring of the patient by the obstetrician-gynaecologist and the rheumatologist is absolutely necessary and beneficial for the patient.
References
https://rheumatism.org.cy/παθήσεις/συστηματικός-ερυθηματώδης-λύκος/
https://www.tosomasoumilaei.gr/ΣΥΣΤΗΜΑΤΙΚΟΣ-ΕΡΥΘΗΜΑΤΩΔΗΣ-ΛΥΚΟΣ
https://www.e-rheumatology.gr/scientific-articles/systimatikos-erythimatodis-lykos-0
http://www.elire.gr/info_det.php?di=12
https://iator.net/aftoanosa-nosimata/sustimatikos-eruthimatodis-lukos/
https://dralexiou.gr/συχνοτερεσ-δερματικεσ-παθησεισ/ερυθηματώδης-λύκος/
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