What is Lupus?

This site is intended for healthcare professionals as a useful source of information on the diagnosis, treatment and support of patients with lupus and related connective tissue diseases.
Systemic lupus erythematosus, SLE or lupus is an autoimmune disease of unknown aetiology. Lupus is a long term health condition with no known cure that can be life-threatening. It is the most commonly presenting Connective Tissue Disease (CTD) and can present in a variety of forms, some of which may be life threatening. It is ten times more common in women than men and is classified as follows:-

• SLE, a diffuse multi-system disease affecting the skin, joints and internal organs
• Discoid lupus erythematosus (DLE), primarily affecting the skin
• Drug associated lupus-like syndromes

In lupus the immune system produces too many antibodies (or blood proteins). Instead of defending the body from infection, these antibodies attack the patient’s own healthy tissue and organs. This can directly or indirectly cause problems in almost any part of the body by inflaming and occasionally damaging the tissue. The antibodies are often directed against components of the nucleus such as double stranded deoxyribonucleic acid (anti-dsDNA). These proteins attach themselves to the skin, causing rashes, or stick to the walls of blood vessels or can deposit in the kidney, brain, lungs, heart, joints, or in fact in any connective tissue. In most cases, when the inflammation settles down, the tissues or affected organs recover leaving no permanent damage.

The name lupus is derived from the Latin for ‘wolf’ describing the pattern of the symptomatic pink facial rash that was thought to resemble a wolf’s mask or a wolf bite. Lupus commonly affects women in their child bearing years, although men, children and new-born babies can develop the disease. It is thought that currently up to 50,000 people in the UK have lupus. Although it is difficult to estimate the incidence in such a rare disease, current figures suggest 1-8 cases per 100,000 people per year in North America and Europe. Lupus is more commonly seen in those of Afro-Caribbean, Asian and Hispanic descent.

Lupus is known as the greatest mimic and is difficult to diagnose as often signs and symptoms can be non-specific with misdiagnoses made, such as rheumatoid arthritis and multiple sclerosis. Lupus is now thought to be more common worldwide than leukaemia, multiple sclerosis, and many other well-known diseases.

There is no one single cause that leads to someone developing lupus, although research has indicated a number of factors, including genetics, hormones and certain infections including viruses. There is a 3-10% chance of family members developing lupus or other associated connective tissue diseases such as mixed connective tissue disease or Sjögren’s syndrome. One of the strongest host factors is the female gender, where women present commonly during pubescence, during child-bearing years and during the menopause. It can be triggered by exposure to strong sun, stress, certain drugs and hormonal changes. It is important to note that in most cases lupus is treatable by medication and patients can, after a flare, live normal and rewarding lives.