SLE, a disease which takes its name from the Latin for wolf, sees the body's immune system attack its own vital organs, writes Nicola Wharrier
Systemic lupus erythematosus (SLE) is a chronic and presently incurable inflammatory condition caused by an autoimmune reaction.
Our immune system normally functions to protect the body and helps to fight invading infections, turning itself on when the infection is detected and shutting down when it resolves. However, in the case of SLE this process fails and for reasons unknown the body's immune system attacks organs in the body.
Patients with SLE produce abnormal antibodies in their blood, and these antibodies target tissues in their own body rather than foreign infectious agents.
The name systemic lupus implies that almost any organ or system within the body can be affected including skin, heart, lungs, kidneys, joints and the nervous system.
When only the skin is involved, the condition is called discoid lupus. A small percentage of sufferers have drug-induced lupus. Drug induced lupus is usually less severe than SLE and disappears when the patient stops taking the drug in question.
The exact cause of these conditions is unknown. This, along with the many different symptoms, which are individually non-specific, makes diagnosis and treatment difficult.
SLE may be triggered by various means and can appear in a bewildering number of ways, even to the extent of mimicking diseases such as rheumatoid arthritis and multiple sclerosis. Some factors which may trigger the autoimmune system into overdrive include viruses, infection, certain medications, sunlight or other environmental factors. Hormone changes may also play a role in SLE, which may explain why this condition is more common in women.
In 1954 survival for those with SLE after four years was only 50%; today it is more than 97%. Better awareness of the condition and earlier diagnosis with appropriate treatment has seen an improvement in the mortality rates, with treatment aimed at managing and suppressing the symptoms when the disease is active.
Many patients have symptoms many years before being diagnosed. These can include finger swelling, joint pain, Raynaud's disease symptoms and most commonly overwhelming fatigue. Frequent throat, gum or respiratory infections are common.
As these symptoms are individually not diagnostic, a formal diagnosis of SLE is often not made until the disease has progressed to the point where there is damage to the internal organs.
It has already been acknowledged that diagnosis is difficult and in the early stages it is often hard to differentiate from other connective tissue disorders such as rheumatoid arthritis.
Once symptoms have become highly suggestive of SLE, of 11 criteria laid down by the American College of Rheumatology to make a definite diagnosis four must be present. In addition, the antinuclear antibody test is almost always positive in SLE.
There is no cure for SLE but careful monitoring of the disease, and a treatment programme with medication adjusted as appropriate, enables the condition to be controlled with most patients living to the same age as they would have done withouth contracting SLE.
In mild disease a non-steroidal anti-inflammatory (diclofenac) can be used to control the symptoms of joint pain. Anti-malarial medication such as plaquenil reduces SLE activity and can help ease joint and skin complaints.
In more aggressive disease, steroids such as prednisolone are used in an attempt to reduce the inflammation that could lead to scarring and thickening of the organ surface, called fibrosis.
Unfortunately there are long-term risks in taking aggressive forms of medication, and by suppressing the immune system there is a risk that infections and cancers may occur. The exact treatment will depend on the individual's symptoms.
Older textbooks give a bleak picture for SLE suffers and should be ignored. Mild forms of the disease are now recognised more often and treated.
This is due to antibody tests being carried out on patients who would, in the past, have been labelled as having "aches and pains" without any clear diagnosis. Modern treatments are now more effective. Therefore, the outlook is more favourable than in the past.
For a few, SLE is severe and sometimes life threatening. Severe inflammation of the kidneys leading to kidney failure is uncommon, but is the main cause of mortality.
Severe brain or heart involvement is also rare but can be serious. However, modern immunosuppressive treatments have improved the outlook even for people with severe disease.
SLE is one of the more complex conditions that underwriters assess due to the relapsing and remitting nature and the many symptoms that vary between individuals.
Each case is assessed on its own merits, taking into account years since diagnosis, activity of the disease, medication and response to therapy, severity of the condition and major organ involvement.
Terms will vary widely and are dictated by factors ranging from ordinary rates in the best scenario to declinature where the disease is at its most aggressive and severe.
Nicola Wharrier is a life and disability underwriter at Scottish Equitable Protect
As with most relapsing remitting conditions, the longer the known history of the disease the better the assessment of the future prognosis of the condition. Most affected proposers are closely followed up by their GP and specialists. Copies of any hospital reports provide a useful history of the course of the condition and this allows the underwriter to quantify the risk.
Any application would only be considered 12 months after the initial diagnosis. Any proposal forms received before this would be delayed. Following the 12-month postponement period the ratings reduce as each year passes subject to the patient taking no more than 7.5mg of steroids a day and the major organs, such as the kidneys and central nervous system, not being involved.
Life Cover: Ratings are very high and start at +250% in the year following diagnosis. These ratings reduce accordingly as mentioned above. If, after five years, the proposer has been off treatment for two to four years and the condition is in remission the ratings can be reduced further.
Critical illness (CI): The risk of major organ failure as well as the general unpredictable and widespread nature of the condition present a considerable extra risk for CI and it should be noted that terms are only available for best cases.
Disability benefits: The extra morbidity risk associated with the condition is considerable, due to the extensive nature of SLE and its many symptoms, particularly joint involvement and the associated toxicity of taking long-term medication.
Income protection, waiver of premium and total and permanent disability are unlikely to be offered until five years after treatment was received and symptoms seen.
All about SLE
• More people have SLE than Aids, sickle-cell anaemia, cerebral palsy, multiple sclerosis and cystic fibrosis combined.
• SLE affects 10,000 people in the UK.
• The disease can occur at any age but is most common in those aged between 20 and 40.
• It is more common in non-Caucasians.
• There may be a hereditary factor involved.
• 90% of lupus patients are women.
• Lupus is the Latin word for wolf.
• Lupus vulgaris is a severe facial rash, now rarely seen, which is said to resemble a wolf's bite.
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