The term ‘lupus’ comes from the Latin word for wolf – the facial rashes associated with the condition were once thought to resemble a wolf’s bite.
The common term ‘lupus’ (lupus erythematosus) actually covers a family of conditions classified as autoimmune diseases. In autoimmune diseases the body’s own self-defence mechanisms (antibodies) become ‘hyperactive’ and attack normal healthy tissues within the body.
Autoimmune diseases can affect many parts of the body, including joints, blood cells, the heart, and other major organs, including the skin.
Among the lupus family, systemic lupus erythematosus is the most common and severe form of the condition.
Classification
lupus can manifest itself as both a ‘systemic’ disease (i.e. one that affects multiple organs of the body within one patient) or in a ‘cutaneous’ form (affecting only those organs of the integumentary system, including hair, skin and nails).
Although there are numerous sub-categories of lupus, the disease can be broken down into four main ‘types’:
● systemic
● discoid
● drug-induced
● neonatal
Other forms of lupus include:
● acute cutaneous lupus erythematosus
● subacute cutaneous lupus erythematosus
● chronic cutaneous lupus erythematosus
● discoid lupus erythematosus
● childhood discoid lupus erythematosus
● generalized discoid lupus erythematosus
● localized discoid lupus erythematosus
● chilblain lupus erythematosus (Hutchinson)
● lupus erythematosus-lichen planus overlap syndrome
● lupus erythematosus panniculitis (lupus erythematosus profundus)
● tumid lupus erythematosus
● verrucous lupus erythematosus (hypertrophic lupus erythematosus)
● complement deficiency syndromes
● drug-induced lupus erythematosus
● neonatal lupus erythematosus
● systemic lupus erythematosus
Signs and Symptoms
The symptoms of lupus can vary from patient to patient. They may last for some time, or come and go over a period of time. Joint pain and swelling are among the most common symptoms and are experienced by almost all sufferers. Commonly, arthritis will affect the joints, including fingers, hands, wrists and knees.
Other common symptoms of lupus include:
● chest pain when taking a deep breath
● fatigue
● fever with no other cause
● general discomfort, uneasiness, or ill-feeling (malaise)
● hair loss
● mouth sores
● sensitivity to sunlight
● skin rash – a “butterfly” rash in about half people with SLE
● swollen lymph nodes
Another common symptom of lupus is photosensitivity (sensitivity to light) although at this time the exact causal relationship between lupus and photosensitivity has yet to be established and research is continuing in this area. Suspected causes of photosensitivity in sufferers of lupus include:
● change in autoantibody location
● cytotoxicity
● inducing apoptosis with autoantigens in apoptotic blebs
● upregulation of adhesion molecules and cytokines
● inducing nitric oxide synthase expression
● ultraviolet-generated antigenic DNA.
● tumor necrosis factor alpha also seems to play a role in the development of photosensitivity.
Genetics
It is widely believed by experts that lupus can be influenced by multiple genes. Lupus is commonly influenced by ‘gene plymorphisms’ and to date 30 of these have been linked to the disorder. However, research is still very active in this area, and little is known about some of these connections at the moment.
Lupus can occur at any age, but most commonly it develops between the ages of 15 and 44. Those who develop lupus at a younger age are more likely to suffer alopecia (hair loss) skin rash and mucocutaneous manifestations … however, patients who develop the disease later in life experience a far higher mortality rate. The mortality rate among those who develop ‘late-onset’ lupus can be as high as 50%.
Ethnicity also seems to play a role both in the likelihood of contracting lupus and the prognosis of patients. Caucasians are far less likely to develop lupus, and if they do are likely to suffer a milder variant, whereas Asian, African and Native Americans are more likely to develop more serious strains.
Survival rates after five years among Caucasians are typically around 94-96%. The only ethnic group with a higher documented survival rate are Koreans, with an average of 98%.
Treatments
As lupus is categorised as an autoimmune disease, most treatments involve the use of immunosuppressive drugs such as hydroxychloroquine and corticosteroids. In 2011, the first new drug in 50 years for the treatment of lupus, belimumab, was approved in the USA by the FDA.
In addition, patients can do a lot to manage their own condition. Rest is essential, and avoiding direct sunlight can help to reduce symptoms. It is important to make family and friends aware of the condition and its unpredictable nature – the nature of lupus can make patients susceptible to stress and depression.
Facts and Figures
● Forms of lupus affect around 5 million people worldwide
● Systemic lupus erythematosus account for around 70% of cases
● Around 5% of children born to people with lupus will develop the illness
● The ratio of women to men with SLE is around 7:1
● Around 0.041% of the UK population has some form of lupus
Conclusion
Whilst lupus is categorised as an ‘incurable’ condition, through a combination of medication and lifestyle it is possible to manage the symptoms of lupus. Likewise, as treatments advance and research continues, most lupus patients can now expect to achieve a normal lifespan.
