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Systemic lupus erythematosus
Systemic lupus erythematosus
Systemic lupus erythematosus is a chronic autoimmune disease known as systemic because it can affect several organs, including the skin, kidneys, joints, lungs and nervous system.
- Clinical manifestations vary greatly from one person to another, but also in the same person over time.
- Lupus mainly affects women (9 women for every 1 man) and young people (average age between 15 and 45 years). It is a rare disease. It is estimated to affect approximately 5,000 people in Belgium.
- Lupus is characterised by the presence of autoantibodies in the blood. These are produced inappropriately by the immune system, which, in addition to defending the body against microbes, targets the body’s own cells. Certain types of white blood cells (lymphocytes) become abnormally activated and cause inflammation and lesions in various organs. The onset of this immune dysregulation is probably linked to several factors (hormonal factors, smoking, viruses, medicines, etc.) in individuals with a genetic susceptibility to developing the disease.
What are the complications of lupus?
- In its cutaneous form, lupus causes a rash on the face, often on the cheeks and nose (“malar rash” or “wolf mask”, hence the name lupus). Red patches may appear on areas exposed to the sun (photosensitivity). They may be accompanied by mouth ulcers and/or more or less significant hair loss. This form affects up to 80% of patients during the course of their disease.
- Joint pain, often affecting the small joints of the hands and wrists, may occur, sometimes associated with swollen joints. This condition is often symmetrical. Inflammation does not generally lead to joint destruction. Joint involvement is present in 75 to 90% of patients.
- Raynaud’s phenomenon affects approximately 30% of patients with lupus. It involves a change in the colour of the fingertips and toes caused by cold or stress, sometimes accompanied by severe pain. This symptom is not specific to lupus and may occur in the general population, particularly among young women, without any immune disease.
- Some autoantibodies may predispose patients to thrombosis (the formation of blood clots in the blood vessels), which can cause phlebitis, pulmonary embolism, myocardial infarction or a cerebrovascular accident. This is referred to as antiphospholipid syndrome.
- Kidney involvement can vary in severity and may sometimes progress to chronic disease. It may develop insidiously, without specific symptoms. It is then detected only through blood tests and testing for proteins in the urine. It may sometimes manifest as fluid retention or high blood pressure. In severe cases, this condition can lead to kidney failure.
- Lupus can cause inflammation of the membranes surrounding the heart (pericardium) and lungs (pleura). The patient may then experience oppressive, recurrent chest pain, coughing or shortness of breath.
- Blood abnormalities are observed in nearly 85% of patients with lupus. They fluctuate during the course of the disease and vary widely. They may include anaemia (a decrease in red blood cells), leukopenia (a decrease in white blood cells) or thrombocytopenia (a decrease in platelets).
- Neurological manifestations also vary in presentation. Headaches are fairly common (30 to 60% of patients with lupus). More rarely, patients may develop epileptic seizures, concentration or memory problems, confusion, mood disorders, depression or drowsiness.
How is lupus diagnosed?
The diagnosis of lupus is established by taking into account the various symptoms presented by the patient and the results of additional examinations.
These examinations include blood tests (to identify antinuclear antibodies, and more specifically anti-Sm or anti-dsDNA antibodies, an increased total antibody level, and reduced levels of certain proteins known as complement factors), imaging examinations to look for complications (heart ultrasound, joint X-rays, etc.), and, if necessary, a skin biopsy.
What treatment is available?
Given the diversity of clinical manifestations and their combinations, on the one hand, and the parallel and simultaneous involvement of different organs, on the other, the management of patients with lupus must be multidisciplinary. It involves the various specialists for each affected organ, in order to determine the severity of each manifestation and define the best treatment, taking into account all the lesions present in a particular patient.
As each patient with lupus has specific clinical manifestations of varying severity, the proposed treatment will be adapted to each patient and may change over the course of the disease.
No treatment can cure lupus. The aim of treatment is to reduce inflammation, prevent long-term complications, improve the patient’s quality of life and, if possible, bring the disease into remission (that is, normalise a series of abnormalities in the blood tests and eliminate certain symptoms indicating inflammation).
The immune origin of the disease means that medicines are used to regulate or reduce immune activity (immunomodulators and immunosuppressants), including cortisone (sometimes at high doses) and hydroxychloroquine (or Plaquenil, which has a preventive effect against relapses). Other medicines may sometimes be prescribed to help limit the use of cortisone, including azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate, etc.
New medicines have recently begun to be used, such as rituximab and belimumab. They reduce the number of cells that produce antibodies.
Other treatments are currently being evaluated for lupus as part of clinical research protocols.
