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Information for Medical Professionals

Has your patient got S.L.E?

GrAs practice nurses you would probably see many patients who tell you that they have a collection of symptoms, which their doctors have not been able to diagnose. As a clinical nurse specialist for lupus I probably have the same conversations that you do with similar patients.

Many of our patients express a feeling of relief when they finally receive their diagnosis of lupus. Many of the women that I speak to tell of the long agonising struggle to find someone to take their symptoms seriously. Some of them have been told that their symptoms are related to depression, the menopause and postnatal depression. When you read later in this article about the symptoms that they suffer from you will realise why they are so desperate for help.

I will try to explain what Lupus is and how we diagnose it with specific information about which symptoms you might notice first. I will also discuss the medication that many of our patients are prescribed. In this way you will see just how much you can help your patient to a diagnoses and hopefully an improved quality of life.

Why are patients referred from around Great Britain

Many doctors refer to us out of desperation as it is difficult for them to confirm the diagnosis. The number of patients who search for information themselves and ask for a referral to us is also increasing. This Unit is unique in that we specialise in SLE and Hughes Syndrome (anti-phospholipid Syndrome). We have tried to give G.P.s information and have held many study days specifically aimed at G.P.'s. This has certainly helped the patients and also the G.P's who will continue to help the patients in the community. We have an education programme for the nurses within the trust and eventually we would like to hold study days for practice nurses too.

When you are aware of the symptoms to look for it is easier to support your patients. You may not be correct in your diagnosis when a patient tells you how they are feeling but it is certainly worth thinking of lupus. You could take that chance and suggest a referral to a Lupus specialist. When your patient receives a diagnosis we can then start to help with the symptoms and you can support them too.

Where can you read about patients' experiences

Some of our patients also have Antiphospholipid Syndrome or Hughes Syndrome. This is a clotting disorder and its symptoms will be seen later in this article. These patients have also experienced a struggle at times for diagnoses. One of our patients Triona Holden has written an excellent book which records the patients experience whilst struggling to get a diagnosis. She describes well the journey she took to receiving her diagnosis of Lupus and Hughes Syndrome.

What is Systemic Lupus Erythematosus (SLE)?

It is a disorder of the immune system - an autoimmune disease. As the body harms itself it causes inflammation and damage to various tissues. Lupus can affect the whole body, which can include joints, skin, brain, blood vessels and probably the most serious damage would be to the kidney. Some patients have antiphospholipid Syndrome (Hughes Syndrome) too.

Who does it affect?

It is a disease that can affect people of any age. However it has been found that women outnumber men nine to one. It is often a disease of childbearing age (15-40) years and often it will appear for the first time in the early twenties.

It is thought that SLE affects 1 in 750 women. In some ethnic groups it is known to be higher and is probably up to 1 in 250. Hughes syndrome is also not rare it is known to be the cause of a quarter of all miscarriages and 1 in 5 strokes under the age of 40.

Lupus is particularly common in the countries like Thailand, Singapore, Malaysia and Hong Kong. In fact it is thought to be more common than rheumatoid arthritis on medical wards there. It is more common worldwide than leukaemia.

Aetiology

Despite the many years of research no cause is known at present however there is a small genetic tendency. No viral or infectious cause has ever been identified.

What are the symptoms?

If you look at figure one you will see the symptoms for SLE that patients experience. They do not all have every symptom and it does depend largely on the severity of the disease. In figure two I have shown you the symptoms of Hughes syndrome as I have mentioned earlier our patients sometimes do have the two diseases. You will notice that many of the symptoms are similar.

Figure 1: Signs and Symptoms of Systemic Lupus Erythematosus

· Extreme Fatigue
· Joint pain and swelling
· Inflammation of the tendons
· Hair loss
· Chest pain
· Depression
· Dryness of the eyes
· Migraines
· Blood and/or protein in the urine of patients with possible renal involvement
· Rashes - after exposure to the sun
· Anaemia
· Butterfly rash on the face
· Pale or purple fingers or toes from cold (Raynaud's Phenomenon)
· Unexplained fever
· Bruising (low platelets)
· Fits

Figure 2: Signs and Symptoms of Hughes Syndrome

· Deep vein thrombosis or pulmonary emboli
· Recurrent miscarriage
· Stroke - usually under age 40
· Memory loss, speech difficulties
· Headaches, migraines, fits
· Pins and needles, difficulty with balance (symptoms often associated with Multiple Sclerosis)
· Extreme fatigue
· Muscle pains and cramps
· Blotchy skin (livedo reticularis)
· Bruising (low platelets)

How is it diagnosed?

Lupus can normally be diagnosed by blood tests such as antinuclear antibody (ANA) and the DNA binding tests. Other tests could reveal anaemia and in some cases a low platelet count that could mean your patient is more likely to bleed. If the ESR is raised this would show us that inflammation is present. In Lupus the C-reactive protein can remain stable. Urine testing is always completed as blood and protein can lead us to decide to investigate renal function further.

Not all patients with lupus have positive ANA and DNA tests and in fact some other diseases show a positive ANA test. The DNA tests are more specific for lupus. Therefore the symptoms that are seen in the patient on examination and the history that they give us are vital clues.

Our patients would also be tested for Lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) to establish whether they have Hughes syndrome or not.

How is it treated?

We attempt to control the symptoms to improve the patients' quality of life but also to prevent permanent damage to organs. We are always concerned about the patients' renal function because we know that damage to the kidney can be irreparable.

The are four main drug groups which we use in the treatment of lupus.

· Aspirin - will usually be prescribed if a patient has Hughes syndrome in the first instance. One junior aspirin (75mg daily) is often sufficient.

· Antimalarials - we use mainly Hydroxychloroquine (Plaquenil) in the first instance. This is often used in conjunction with steroids but the aim is to have a small dose of steroids or to stop them completely. Some of our patients, with mild lupus, have found that this drug can help them if they have skin and/or joint involvement. Some patients seem to always need a small dose of prednisolone too.

We are now prescribing mepacrine (Atabrine) for some of our patients, which we have found, is extremely effective especially with resistant skin involvement.

· Steroids - prednisolone has been and is still vital in our efforts to dampen down or suppressing disease activity. We use it as a depo injection, Intravenous therapy and also orally depending on the needs of the patients. It is always our aim to reduce the dose to the smallest possible

· Immune Suppressants - these are usually used in patients when antimalarials and steroids do not work. This would be moderate to severe disease. We would use azathioprine (Imuran), methotrexate or mycophenolate (CellCept). However with these drugs there is a complication that they can suppress liver function and so regular blood tests are required.

Often when the kidneys or the brain are involved or in very resistance disease we can use intravenous cyclophosphamide (Endoxana), which we give every two weeks for six doses. Our patient would then return to oral immune suppressants.


Why the patients need your continued support?

In my role as clinical nurse specialist I know that much of my time is spent listening to the patients. This might be in the nurse led clinic or perhaps during a consultation by phone (we have a nurse led advice line). Our aim is to work in partnership with our patients and so that is why we try to educate them as much as possible from time of diagnosis. In this way they can recognise what is happening to their bodies earlier and have the confidence to contact either us or their surgery for help.

However with 2,500 patients and growing numbers this is not something I can do alone. Patients need more than just clinical nurse specialists. They need their local practice nurse to have an understanding too.

Although we have treatments we do not find that we can cure the extreme tiredness that patients suffer from and this can lead to depression. It is important that they can have contact with someone who can recognise this and ensure that new symptoms are recognised. They need to know that when they go to a G.P's surgery they will be listened to and to know that you have an understanding of their disease and its effects will help them.

You can help them recognise a flare (this could be increased fatigue, rash, joint paint, fever, dizziness) and ensure that they receive the care that they need. Not all of their health problems are lupus related and so it is important to be clear about their symptoms so that some other problem is not missed. You could monitor urine when they visit you, as the visits to us are so infrequent at times, and thus recognise renal involvement earlier. There is also a role for monitoring side effects of the medication they receive.

Conclusion

Many practice nurses throughout the country may have one of our patients on your list. I know from experience how much they would value your support and advice. I have recently given our patients a questionnaire about our advice line. In it I asked who they would like to have to help them apart from the lupus unit. Many of them said they would like the staff at the G.P's surgery to have knowledge and to be able to support them. Many specifically stated the practice nurse. The numbers of patients is quite large and growing and as you can now see the symptoms and effects of the disease can be quite serious. For this reason it would be helpful for our patients to have the sort of support that diabetic patients receive.

Updated 11/2008

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