Systemic lupus erythematosus
(Lupus erythematodes disseminatus)
Systemic lupus erythematosus (SLE)
Systemic lupus
Systemic lupus is a chronic inflammatory multisystem disease, which means that it affects several organs and organ systems. The autoimmune nature of the disease is accompanied by a range of signs and symptoms. Systemic lupus erythematosus mainly occurs in young women of childbearing age. The leading symptoms of systemic lupus erythematosus are arthralgia and arthritis (painful and swollen joints); the skin is most often affected on the face, as a butterfly-shaped rash across the cheeks, but other areas of skin can be affected too. The serous membranes around the lungs and heart may be involved (pleuritis or pericarditis), as may the kidneys (relevant to prognosis and diagnosis) and the central nervous system, and there may be cytopenia in the blood count (a reduction in some of the cellular components of the blood). The laboratory tests your specialist rheumatologist will order are very complex, both for diagnosing and monitoring systemic lupus erythematosus and for its complications.
Diagnosis is based on the medical history and on clinical and laboratory (serological) tests. The criteria are used for large clinical studies, while in practice they vary considerably. Treatment of the more severe forms of systemic lupus erythematosus includes corticosteroids, often hydroxychloroquine, and sometimes immunosuppressants.
Women (usually of childbearing age) account for 70–90% of all cases. Difficulty conceiving is very common. SLE occurs throughout the world but is somewhat more common in Black people. Systemic lupus can occur at any age, even in infancy. Earlier recognition of milder forms, together with better diagnosis and treatment in specialist rheumatology centres, has led to a worldwide rise in the prevalence of systemic lupus. The cause of systemic lupus erythematosus is unknown, but unknown triggers clearly play a role: genetically predisposed people with certain HLA types are exposed to environmental triggers that set off autoimmune reactions in the body. A number of medicines can cause what is known as drug-induced lupus (for example, hydralazine and procainamide cause a lupus-like syndrome). There is also localised cutaneous lupus, which has its own characteristics.
Signs, symptoms and a medical history suggestive of systemic lupus erythematosus.
SLE CRITERIA*
For clinical research reports, at least 4 of the following SLE criteria must be met:
Malar rash – a rash around the nose; the nasolabial fold is usually spared
Discoid rash
Photosensitivity – sensitivity to sunlight
Oral ulcers – aphthae
Arthritis – painful and swollen joints
Serositis – pleuritis and pericarditis
Nephropathy
Leukopenia (<4000/μl),
lymphopenia (<1500/μl),
haemolytic anaemia or thrombocytopenia (<100,000/μl)
Neurological deficits
Positive anti-DNA or anti-Sm antibodies; positive antiphospholipid antibodies
ANA / ENA
* These 11 criteria were set out by the American College of Rheumatology and are often used as an aid to diagnosis. Although at least 4 criteria are not always
required for a diagnosis of SLE, the list is useful for an overview of the symptoms.
The criteria do not include very important signs and symptoms such as fever and malaise, which can last for months or years. Joint pain with or without swelling is common in systemic lupus erythematosus. Also common are headaches, miscarriages, alopecia (hair loss), petechiae (small haemorrhages), vasculitis, heart valve involvement (Libman–Sacks endocarditis), rapidly progressive atherosclerosis and AV block, generalised lymphadenopathy, splenomegaly (an enlarged spleen), cognitive impairment, personality changes, ischaemic strokes, subarachnoid haemorrhage, seizures, psychoses, organic brain syndrome, aseptic meningitis, peripheral neuropathies, transverse myelitis and cerebellar dysfunction, among others. Differential diagnosis and overlap with other diseases are common. Systemic lupus erythematosus (SLE) can mimic many diseases and is a great challenge for any doctor. At Faktor Zdravlje Polyclinic we follow the latest developments in the diagnosis and treatment of systemic lupus erythematosus (SLE).
Facts about lupus:
– a chronic inflammatory rheumatic disease that can affect various organs and organ systems, especially the skin, joints, blood and kidneys
– systemic lupus erythematosus often starts and worsens suddenly (flares), while some patients have constant lupus activity.
– lupus can range from mild to fatal and should always be treated by a doctor.
– cooperation between patient and doctor, in other words good medical care, allows most patients to have a good quality of life.
– lupus is not contagious, not even through sexual contact. You cannot ‘catch’ lupus from someone or ‘give’ it to anyone.
– systemic lupus is not ‘like cancer’ and is not related to it.
– systemic lupus is not ‘like HIV/AIDS’, in which the immune system is underactive; in lupus it is overactive.
– more than 5 million people worldwide have some form of lupus
– systemic lupus erythematosus affects women of childbearing age (the years when a woman can conceive)
– men, children and adolescents can have lupus
– people of all ethnicities and both sexes can develop lupus, but it is 2–3 times more common in Black people.
At Faktor Zdravlje Polyclinic, our rheumatologists will give you expert, evidence-based advice.
