Do you sometimes have pains in the joints or in the limbs?
Do you sometimes feel nauseated?
Do you have a noticeable hair loss?
Do you have headaches?
Do you feel run down although you sleep a lot?
Do you occasionally have a very dry mouth and eyes? Do you have conjunctivitis?
Do you sometimes have an increased temperature or an unexplainable fever?
Do you occasionally have a "butterfly" rash over the cheeks of the face or patchy redness on other parts of your body?
Are you sensitive to light and can no longer cope with direct sunlight?
Have you visited many doctors searching for advice and none of them had answers for you?
If you answer three or four of the above questions with "yes", it could be possible that you have a disease that is known as "Lupus erythematosus". Further symptoms or conditions that can also occur are for example heart, lung or kidney abnormalities, diarrhoea, problems to concentrate, convulsions and psychic disorders.
What is Lupus?
Lupus erythematosus (LE) is an autoimmune disease, which belongs to the inflammatory rheumatic conditions and thereby to the connective tissue diseases. There are two main forms of lupus: Cutaneus lupus erythematosus and systemic lupus erythematosus (SLE).
Cutaneus lupus erythematosus
Some of the patients only have an involvement of the skin. There are different sub forms of cutaneus lupus. The most frequent form ist the discoid lupus erythematosus (DLE). It is characterised by discoid shaped, squamous red rashes on the face or other parts of the body. In approximately five to ten percent of these patients, the cutaneus lupus develops into a systemic lupus erythematosus.
Systemic lupus erythematosus (SLE)
This is the most frequent form of lupus. There can be an involvement of the skin, the joints and internal organs. The typical skin rash on the cheeks (often in a butterfly shape) occurs only in less than half of the SLE-patients.
Most common symptoms of people with systemic lupus erythematosus:
(The above list is taken from: Hettenkofer, Hans-Jürgen [Ed.]: Rheumatologie. Stuttgart: Thieme Verlag, 1998, p. 91)
Factors that can trigger lupus erythematosus
Eighty percent of the patients with systemic lupus erythematosus are young women between 15 and 45. Lupus has in fact something to do with the female hormones (estrogenes). Quite often Lupus is triggered off during or after a pregnancy. The intake of the "pill" (contraceptives containing estrogen) can stimulate the disease. Also noticeable problems and irregularities (such as joint pains) correlated to the menstrual periods could be an indication of lupus.
Sensitiveness to sunlight is also typical of lupus. Sunlight can stimulate and worsen the disease progression. This is the reason, why lupus sometimes breaks out after sunbathing or a holiday in a sunny climate. Like other autoimmune diseases, physical and psychical stress influences the disease and its progression in a negative manner. Certain foods and medication can intensify disease activitiy.
Up to date, the cause of the disease is unknown. It is thought that similar to other autoimmune diseases, a genetic disposition together with other factors trigger the disease off. Certain prescribed drugs can induce symptoms similar to those of systemic lupus erythematosus (drug-induced lupus erythematosus). Usually the symptoms fade when the drug use is discontinued.
Often it takes a long time until the diagnosis lupus erythematosus is found. The reason is that patients have a wide variety of symptoms and different combinations of involvement. According to the 2012 published criteria of the American Rheumatism Association, four of seventeen findings must be given to consider the possibility of systemic lupus erythematosus (so called "SLICC-criteria"). A prooved involvement of internal organs is no essential criteria for the diagnosis "SLE". It is not possible to make the diagnosis of SLE in early phases of the disease by using the SLICC-Criteria. It is more difficult to make the diagnosis "Lupus" in cases of chronic progressive course of disease than in cases of intermittend disease progression (see below).
Due to the fact that lupus is a relatively seldom disease, it remains unknown to many doctors. In Germany, there are only approximately 30 - 40.000 people who suffer from SLE. Doctors can therefore not readily recognise the disease. Many young women are considered to be a hypochondriac.
The doctor for the diagnosis and treatment of lupus is a specialist for internal diseases, who has also specialised in rheumatology. It is possible to identify the disease through particular blood tests, first of all the antinuclear antibody (ANA) test and further antibody tests as for example the DNA antibody test.
Quite often, when the symptoms and blood tests show no clear indication for an exact diagnosis, the diagnosis that is then first given is "undifferentiated connective tissue disease". The treatment is the same. There are also a variety of mixed disease forms between LE and other connective tissue diseases such as Sjogren's syndrome, Scleroderma and the antiphospholipid antibody syndrome.
Course of disease
The progression of the illness in approximately two thirds of the lupus patients is intermittent. About half of these patients have in between the periods of disease activity (flares) little to no ailment (remission) whereas the other half experience continuing activity in between the major periods of disease activity (partial remission).
Approximately a third of the patients have a chronic progressive course of disease, i.e. the disease progressions continually developing slowly, without periods of major activity and non-activity.
To date there are no blood tests available to measure the disease activity. Neither the blood sedimentation nor the amount (titre) of the antinuclear antibody (ANA) test directly reflects the disease activity. The C-reactive protein (CRP) is in fact during a major activity period only slightly raised. It seems that the raise of the DNA antibody titre and a reduction of the complement indicates an increased disease activity. In order to judge the disease activity, it is necessary to review the patients symptoms and problems, the clinical results together with the results of labour tests (e.g. blood tests, tissue biopsies), whereas the clinical results carry the heavier weight.
As there is no definite indicator for the lupus erythematosus disease activity and the fact that in the early phases of the disease, organ involvement is often overseen, doctors tend to underestimate the seriousness of the disease.
The treatment of lupus erythematosus is carried out in a stepwise approach:
Painkillers as Non-steroidal Anti-inflammatory Drugs (NSAID)
Antimalarials (e.g. chloroquine)
Immunosuppressives (e.g. azathioprine, cyclosporine, mycophenolate mofetil), Belimumab
Cytotoxic drugs (e.g. methotrexate, cyclophosphamide)
This list starts with the weaker and ends with the stronger medications. The purpose of the use of these medications is to decrease inflammation and the level of autoimmune activity in the body. The medications intervene in the various locations of the disease process, however a causal treatment that brings permanent cure is to date not available.
The choice of medication depends on the seriousness of the clinical symptoms. Antimalarials have been found to be particularly effective in working against lupus erythematosus skin and joint problems. Immunosuppressives and cytotoxic drugs are used for treating severe cases. Patients with intermittent disease progression react generally better to the treatment of the known medication than the patients with a chronic progressive course of disease.
Presently, medical research in the university clinics concentrates on the development of new treatment methods (e.g. treatment with high-dose cyclophosphamide, stem cell transplantation) for the most severe intermittent forms of systemic lupus erythematosus which represent approximately five percent of all SLE-patients. In the last decade, there were no new medications developed for the most SLE-patients. The reason for this is that in view of the low numbers of LE-patients the development of new treatments /medicaments is not profitable for the pharmaceutical industry.
The earlier the treatment starts, the better the course of the disease can be influenced. Today, lupus patients can expect a normal life expectancy if the disease is recognised and treated early after its outbreak. The quality of life may be reduced in one way or the other.
Many thanks to an australian scleroderma patient for the English translation!