Lupus is an autoimmune disorder which varies in severity from mild to life threatening and can therefore be of little or great significance for life and disability cover.
There are several types of lupus disorders but the most common is systemic lupus erythematosus (SLE), accounting for about 70% of all cases.
The immune system is an army of white blood cells which fights off infection by recognising foreign bodies such as viruses and bacteria (antigens) and destroying them. Occasionally the immune system gets it wrong and tries to destroy healthy tissue - the body effectively tries to destroy itself. It is not known why the immune system cannot tell the difference between antigens and healthy tissue, but it gives rise to many well known conditions such as type 1 diabetes, multiple sclerosis, rheumatoid arthritis, some thyroid disorders and coeliac disease, amongst others.
Autoimmune disorders fall into two general types: "localised" where only a single organ or tissue is directly damaged by the autoimmune process, and "systemic"; damaging many organs. As the name states, SLE is a systemic disorder. The tissues most commonly affected are the skin, joints and muscles, but the blood, heart, lungs, kidneys and brain may also be affected.
"Lupus" is derived from the Latin for wolf, but how the name of Lupus came to be associated with this disease is obscure. It is thought maybe that the distinctive butterfly shaped rash over the cheeks and nose resembles a wolf's markings, but there are other tentative explanations such as the rash associated with SLE resembles that of a wolf bite, or a rash that wolves themselves suffer. These explanations seem rather unlikely, but the name Lupus has stuck.
Erythematosus refers to redness and rashes of the skin, characteristic of the condition.
SLE is a connective tissue disorder. The function of connective tissue is to support, anchor, connect (and separate) all organs and body cavities. Connective Tissue Proper includes areolar tissue, adipose tissue (fat), and dense regular tissue, among others. Areolar connective tissue is the most widespread connective tissue of the body. It is used to attach the skin to the underlying tissue, it fills the spaces between various organs and holds them in place as well as cushions and protects them, and surrounds and supports the blood vessels. Specialized Connective Tissue includes cartilage, bone, and blood. Given that connective tissue is the most widespread and abundant type of tissue in the human body it is easy to see that an autoimmune disorder affecting it could have devastating consequences.
The Lupus Foundation of America estimates that five million people worldwide have a form of Lupus, and SLE affects about 3 in 10,000 people in the UK. Anyone can develop lupus, including children, but certain people are more likely to develop the disease. Nine out of ten people who have lupus are women, and the disease usually strikes during the childbearing years. It is also more common in those of non-European descent.
A person's risk of developing lupus appears to be determined by genetic (hereditary) factors. However, the onset of the disease may be triggered by environmental factors such as infection, sunlight, hormonal changes or stress.
Common presenting symptoms are skin rash, low grade fever, fatigue and painful joints. Unfortunately these symptoms are common in many other conditions, making diagnosis difficult. A Lupus Foundation of America survey of its members suggests that more than half of those afflicted with lupus suffered for at least four years, and saw three or more doctors before obtaining a correct diagnosis of lupus. There is no one definitive test to confirm the diagnosis although the presence of particular antibodies together with typical symptoms is an indication for a diagnosis of SLE. A number of tests will also be carried out to eliminate alternative diagnoses.
SLE is chronic: there is no cure. There are usually periods of remission where the symptoms are minimal, with relapses lasting a few weeks or longer. Symptoms are classified as mild, moderate or severe:
MILD - joint and/or skin symptoms and tiredness. This is unpleasant but not serious, and symptoms are usually eased with treatment.
MODERATE - usually includes inflammation of other parts of the body such as the pleura, which is the connective tissue surrounding the lungs resulting in pleurisy, or the endocardium surrounding the heart resulting in endocarditis.. There may also be kidney inflammation.
SEVERE - The actual heart and lung tissues are affected, and the kidneys more extensively involved. Brain tissue can also be inflamed. Inflammation of any of these organs can lead to severe complications and even death.
Once SLE has been diagnosed the patient will be regularly followed up, usually by a rheumatologist who will regularly check for the development of any of these serious complications, as well as checking on the level of activity of the disease.
Treatment options include the following:
- Non steroidal anti-inflammatory drugs (NSAIDs) eg ibuprofen, diclofenac, naproxen - prescribed to ease pain from joint and muscle inflammation
- Hydroxychloroquine - improves the skin problems of rashes and ulceration - can cause eye problems
- Steroids - usually only given in more severe cases to reduce inflammation. The mortality risk for SLE is fivefold when compared to the normal population in the late stages, which can be attributed to cardiovascular diseases acquired from corticosteroid therapy, the leading cause of death for people with SLE.
- Immunosuppressants eg azathioprine, cyclosporine, methotrexate - these drugs suppress the immune system and are used in many autoimmune disorders as well as to prevent rejection following organ transplant, but they can have serious side effects and require constant monitoring. By depressing the immune system they are also increasing the patient's risk to infection. Infections themselves can trigger a relapse of SLE.
Prognosis
In the 1950s, a diagnosis of SLE had a poor outlook because only those with the most severe disease were recognised, and most people diagnosed with SLE lived fewer than five years. Today's doctors are more aware of the condition and although diagnosis may be elusive, it is generally diagnosed earlier and can now be treated more effectively. Advances in diagnosis and treatment have improved survival to the point where over 90% now survive for more than ten years, and many can live relatively asymptomatically. Prognosis is normally worse for men and children than for women; however, if symptoms are present after age 60, the disease tends to run a more benign course. Early mortality, within 5 years, is due to organ failure or overwhelming infections, both of which can be modified by early diagnosis and treatment.
The Lupus Foundation of America estimates that 10 - 15% of lupus sufferers will die prematurely as a result of complications of their condition. Two out of three lupus patients reported a complete or partial loss of their income because they are unable to work full time due to complications of lupus. This is due not only to the symptoms of the many complications, but also due to a high rate of depression. A significant number of SLE patients suffer with depression, either as a result of direct brain damage, a response to the burden of a chronic disease, or the social consequences of a chronic disease. Other complications of particular relevance to critical illness cover include renal failure, heart attacks secondary to advanced atherosclerosis, heart valve problems, chronic lung disease, myeloid malignancies and others. This has clear implications for life, serious illness and disability cover.
UNDERWRITING IMPLICATIONS
There will be two scenarios presented to the underwriter, cases where a diagnosis of SLE has been confirmed, and cases where SLE is suspected.
The information required is:
- Diagnosis, if known
- Duration and course of disease
- Whether currently active or in remission
- Current symptoms
- Results of any tests including regular antibody tests for disease activity
- Organ involvement
- Treatment and response to treatment
It may be sufficient to use telemedical interviewing to establish whether the disease falls into the mild category. Anything more severe will require a report from the GP for full medical details including test results.
The risk will then be classified into mild, moderate, severe or "disease suspected", based on activity of disease, symptoms and treatment.
Severe cases will warrant declinature for all benefits.
For life cover a recent diagnosis (within a year) will result in postponement. After this, the rating will depend on the age of the client and the severity of the condition. Younger clients will have higher ratings and of course the more severe the disease the higher the rating. Ratings range from approximately +50 to +250.
For critical illness the postponement period following diagnosis will be longer, typically two years, and the ratings higher with moderate and severe cases being declined. Individual ratings will depend on the range of conditions covered by the provider.
Due to the relapsing nature of the condition and the uncertain prognosis, as well as the many complications which affect the sufferer's ability to work, the majority of IP applications will be declined.
Anne Llewllyn is underwriting training and development manager at PruProtect
1. Lupus Foundation of America "Statistics on Lupus" http://www.lupus.org
2. Alliance for Lupus Research http://www.lupusresearch.org/
3. Vasudevan AR and Ginzler EM (August 4, 2009). "Established and novel treatments for lupus". The Journal of Musculoskeletal Medicine 26 (8).
4. Stoll T et al (January 16, 2001) "Prediction of depression in systemic lupus erythematosus patients using SF-36 Mental Health scores" Oxford Journal of Rheumatology" 40 (695-698)
5. Underwriting implications from Pacific Life Re
