Systemic Lupus Erythematosus

Systemic lupus is a presently-incurable illness of the immune system
whereby the patient’s immune system creates antibodies which instead of protecting the body from bacteria, viruses and other foreign matter attacks itself. This causes symptoms of extreme fatigue, joint pain, muscle aches, anaemia, general malaise and possibly destruction of vital organs.

Discoid lupus is a condition of the skin alone, and in a very few patients might develop into systemic lupus.

Lupus mainly attacks women during their child-bearing years (ages 15-50) but men and even young children can be affected. It is estimated that 1 in 750 women suffer from lupus in the UK, with the ratio of women to men being 9:1. Lupus is a worldwide disease more common in some races than others. The incidence in white women is 1 in 1000 compared with that in black women of 1 in 250 and Asian races also have a higher tendency to lupus. Only 10%of lupus patients are male.

Although few people have heard of lupus, worldwide it is acknowledged as being more common than leukaemia, muscular dystrophy and multiple sclerosis, which due to extensive publicity have become household names.

Lupus can present in a bewildering number of ways, even to the extent of mimicking other diseases such as rheumatoid arthritis, multiple sclerosis or ME. With the variety of presentations, lupus is difficult to diagnose and it can be overlooked, often for years. Unless the OP or consultant is alert to its possibilities. Many a time the patient has been told ‘it’s all in the mind’! A further problem the patient faces is that she or he can look quite well, eg “healthy” red cheeks are often in evidence.

Lupus is neither infectious nor contagious – the cause is not known though research has provided evidence implicating heredity, hormones and infections, including viruses. The disease lies dormant in the body until some trigger from outside the body sets the process in motion

What Are The Symptoms?

Although there are many possible manifestations of lupus, those listed below are some of the more common. Lupus is a disease which can present many different facets, rarely do two people have exactly the same symptoms, and these can vary from just one to many.

  • Joint aches and pains
  • Headaches, migraine
  • Permanent rash over cheeks
  • Kidney problems
  • Extreme fatigue and weakness
  • Oral ulcers
  • Increased risk of miscarriage
  • Hair loss
  • Rashes from sunlight
  • Depression
  • Recurring flu-like symptoms and/or night sweats
  • Inflammation of the tissues covering internal organs with associated chest
  • and abdominal pain
  • Poor blood circulation causing the tips of fingers and toes to turn white then blue on exposure to cold – Raynaud’s Phenomenon
  • Haematological disorders including anaemia
  • Seizures, mental illness or other cerebral problems
  • A person with lupus may have four or five symptoms. Some of these might recede, and/or others develop.

 

The two major symptoms in lupus appear to be:

Joint/muscle ache and pains and extreme fatigue and weakness

Information Courtesy of:
LUPUS UK  St. James House
Eastern RoadRomford
Essex RM1 3NH

Tel : 01708 731251
Fax: 01708 731252

The Triggers of LUPUS

  • What are the triggers ?
  • at puberty
  • during the menopause
  • after childbirth
  • after viral infection
  • through sunlight
  • as a result of trauma
  • after a prolonged course of medication

Lupus is often triggered in people where there is existing family history of lupus and/or other immune system illness, such as arthritis, rheumatism and MS.

Diagnosis

Study of many thousands of lupus patients across the world has led to the recognition of the following as possible early pointers of lupus. Only one or two of the pointers may be in evidence :

  • Rashes, facial or elsewhere
  • Teenage migraine
  • Agoraphobia/claustrophobia
  • Finger flexing difficulty
  • Menstrual cycle problems
  • Dry eyes/mouth
  • Teenage “growing pains”
  • Teenage glandular fever
  • Severe reaction to insect bites
  • Recurrent miscarriages
  • Family history of immune system illness
  • Low lymph count

The list is not exhaustive and, whilst the GP or specialist should be mindful of the above possibilities, he or she will be guided by the diagnostic criteria.

How is lupus diagnosed?

Lupus is a type of self-allergy, a disease of many manifestations and each patient’s profile or list of symptom5 may be different. The specialist may need to investigate any of: Skin Blood Fatigue Joints Heart Fever Kidneys Lungs Weight Loss
Swollen Glands Nervous System Weight Gain

In arriving at a firm diagnosis the physician will normally expect at least four of a list of 11 internationally accepted conditions to be present (either currently or at any time since the onset of the illness). These conditions include one or more blood tests, and the patient’s/family medical history will also be taken into account.
Diagnosis is usually achieved through a rheumatologist but other specialists may also be involved, e~ dermatologists, kidney specialists, cardiologists, obstetricians.

Suggestions on How to Cope with Lupus

Become well-educated about lupus.

Offset fatigue by rest and pacing daily activities. Develop priorities
The fatigue is not like the fatigue or tiredness from physical exertion.
Try to prepare for the up-and down nature of the disease. Plan alternative activities, alternate time schedules. Allow time for extra rest. Break down big, long-term goals into small, manageable steps that can be accomplished in short amounts of time.

Be open with family and friends about the unpredictable pattern of lupus, and how the disease affects you.

By “listening” to your pain as a signal. you can begin to control it.

Try to resolve stress, depression, pain, anger. Find positive ways to channel negative feelings.

Try to accept the things you cannot change rather than feeling constantly frustrated and upset over situations beyond your control.

Approximately 1/3 of lupus patients are photosensitive. Avoid direct and prolonged sun exposure and ultraviolet light from artificial sources (e.g. fluorescent lights). Wear broad-brimmed hats and cover other exposed parts of the body when out of doors in sunlight and use sunblock creams.

Although lupus does not directly affect the body’s capacity for sexual enjoyment, the disease can cause problems. Open, honest communication is necessary to overcome this.

If lupus has limited your hobbies and activities, find new ways to use your skills.

Ask for help if you need it. Family and friends, doctors and other health care professionals together with LUPUS UK are all sources of help and support.

The Treatment Of Lupus

There is at present no cure for Lupus but careful monitoring of the disease and a treatment programme with medication adjusted as appropriate enables the condition to be controlled, most patients being able to live a normal life span. The majority of lupus patients are in the ongoing care of their rheumatologists, and other specialists may also have involvement from time to time.

The symptoms and diagnosis in more detail:

THE DIAGNOSIS OF LUPUS

LUPUS is an autoimmune disease, a type of self-allergy, whereby the patient’s immune system creates antibodies which instead of protecting the body from bacteria, viruses and other foreign matter attack the person’s own body tissues. This causes symptoms of extreme fatigue, joint pain, muscle aches, anaemia, general malaise, and can result in the destruction of vital organs. It is a disease with many manifestations, and each person’s profile or list of symptoms may be different. LUPUS can mimic other diseases, such as multiple sclerosis and rheumatoid arthritis, making it difficult to diagnose by GPs as they see few cases of lupus and thus are not alert to its possibility.

Physicians are often cautious with their diagnosis as they do not want to label anyone until they are certain of LUPUS. Moreover, a careful review of the patient’s entire medical history is necessary, coupled with analysis of results obtained from tests relating to their immune status to provide accurate diagnosis. Currently there is no single test that can definitely say whether a person has LUPUS or not. Only by comprehensive examination and consideration of symptoms and their history can a diagnosis be achieved.

LUPUS is a complex disease in which almost every system in the body can be affected, and the diagnosis is based on a combination of symptoms, signs and test results. Once a diagnosis of LUPUS is made, the patient’s symptoms should be treated as necessary. The goal of the treatment is to control the symptoms and the disease so that the patient can lead as normal a life as possible.

Dr Graham R V Hughes MD FRCP
Consultant Rheumatologist St Thomas’ Hospital
London SE1 7EH

DIAGNOSIS OF LUPUS

DIAGNOSIS OF LUPUS or any other chronic illness may be established using the 5 Step Programme.

  1. Review patient symptoms
  2. Detailed physical examination
  3. Battery of tests
  4. Rule out other diseases
  5. Time is sometimes necessary to observe the course of the disease.

The FIRST PRINCIPLE in making a diagnosis of LUPUS is that the individual has clinical evidence of a multi-system disease, and several manifestations such as those listed below may be present:-

  • SKIN Rashes, Mouth Ulcers,HairLoss
  • JOINTS Pain, redness and swelling
  • KIDNEY Abnormal Urinanalysis
  • LINING MEMBRANE Pleurisy, Pericarditis, Peritonitis
  • BLOOD Haemolytic Anaemia,
  • Leukopenia
  • LUNGS Shortness of breath, cough
  • NERVOUS SYSTEM Convulsions, psychosis

THE SECOND PRINCIPLE is to examine the status of the immune system and how the cells that comprise the immune system are functioning in individuals having a suspicious clinical history. The most useful test is the ANA (Anti-Nuclear Antibody) test, supported by and in combination with the clinical history.

The onset of LUPUS can be gradual with new and different symptoms appearing over weeks, months or even years. The symptoms are often hard to describe and can come and go suddenly, therefore it may often be that the patient might begin to feel “it is all in the mind”. As a consequence such patients are frequently categorised as hypochondriacs.

The symptoms of LUPUS seem to fall into two categories, non-specific and specific.

NON-SPECIFIC SYMPTOMS

Fatigue

This is one of the most common and certainly one of the most prominent features of lupus. Patients often describe it as an ‘unnatural fatigue’. Its causes are not well understood. Often it precedes the diagnosis by months or years and only when treatment has been successfully started does the patient realise how major a feature it had been


Aches and pains

The majority of lupus patients suffer at some stage from joint and
Muscle pains. In many patients this presents as ‘pain all over”. In acute flares
of lupus the symptoms are often described as being ‘flu-like”. Unlike other rheumatic diseases such as rheumatoid arthritis, there is often very little to see in the way of joint swelling. It is not just the joints that are affected but the tendons and muscles as well. In the majority of cases the joint inflammation does not progress to permanent damage


Fevers

Fever is usually a feature of a flare of the disease. Fever is unusual when the disease is in a quiet phase: thus in an adult or a child known to have lupus who develops fever the possibility that a separate diagnosis – infection – might be present always needs consideration.


Rashes

A wine variety of skin rashes occur in lupus. Traditionally these are sun-sensitive (‘photsensitive’) but this is not always the case. The commonest rashes are on the cheeks (the butterfly rash across the nose and cheeks), on the elbows, on the palms and soles and on the V-neck area. The rashes vary from pinkish discolouration through to blisters and small pinpoint ‘blood spots” (purpura). Most rashes in lupus have a tendency to come and go.


Hair loss

Hair loss is one of the most important features of active lupus. It may be the first manifestation of the disease and is often first noticed by the patient as hair on the pillow. In some cases hair loss is patchy and even extreme, Fortunately, in the vast majority of patients the hair re-grows after successful treatment, though hair regeneration is often notoriously slow


Headaches

Headaches are a major feature of lupus. In some patients a history of headaches or “a typical migraine” go back to the patient’s teens and pre-date the diagnosis by many, many years. There is almost certainly a variety of causes of headaches in systemic lupus. One specific and important cause is “sticky blood” caused by the presence of antiphospholipid antibodies


Depression

Depression is an important feature of lupus. It is sometimes simply
attributed to being ‘unwell” or having tiredness and pain. However, in many patients it is far more important than this and is a primary feature of the disease, it sometimes responds well to management of the lupus itself and is clearly a central feature of the lupus process. In some patients the return of depression is a tell-tale sign that the lupus is flaring.


General symptoms

As almost every organ n the body maybe affected at sometime, the
symptoms and signs are legion and can include irritation of the eyes
(sometimes associated with dry eyes), swollen glands, mouth ulcers, chest
pain (pleurisy is, for example, important in active lupus), weight loss and ankle swelling. Other additional problems commonly experienced by patients may be high blood pressure, and Raynaud’s Phenomenon.


Specific Symptoms

To help distinguish LUPUS from other diseases, physicians of the American Rheumatism Association have established a list of 11 abnormalities which, when combined, point to LUPUS.

To make a diagnosis of LUPUS the patient must have had at least FOUR of these 11 manifestations at any time since the onset of the disease.

  1. MALAR RASH Fixed red rash over the cheeks
  2. DISCOID RASH Red patches of skin associated with
    scaling and plugging of the hair follicles
  3. PHOTOSENSITIVITY Rash after exposure to sunlight
  4. MUCOSAL ULCERS Small sores that occur in mucosal lining of
    mouth and nose
  5. SEROSITIS Inflammation of the delicate tissues covering
    internal organs and abdominal pain
  6. ARTHRITIS Very common in LUPUS, usually pain in the
    joints
  7. RENAL DISORDERS Usually detected by routine blood and urine
    analysis
  8. NEUROLOGICAL DISORDER Seizures or psychosis
  9. HAEMATOLOGICAL DISORDER Haemolytic Anaemia, Leukopenia,
    Thrombocytopenia
  10. IMMUNOLOGIC DISORDER -Tests on LE cells, anti-DNA and
    anti-Sm antibodies
  11. ANTI-NUCLEAR ANTIBODY (ANA TEST)

When found in the blood and the
patient is not taking drugs, it is
known to cause a positive test for
LUPUS in most cases, but is not
necessarily conclusive.

And helpful hints.

The American Rheumatism Association criteria has provided the benchmark for the classification of lupus for the last 20 years. Many thousands of lupus patients passing through St Thomas’ Hospital have led Dr Graham Hughes to offer the following 14 criteria aimed more towards diagnostic help and not to classification.

  1. Teenage ‘growing pains’
    Growing pains, at least in the UK, is a label widely used for joint pains in teenagers and seems to cover a spectrum of rheumatology from arthritis variants through to lupus.
  2. Teenage migraine
    Headache, cluster headache and migraine can be encountered and a strong history of teenage migraine may be of lupus significance, either at that time or subsequently.
  3. Teenage ‘glandular fever’
    Prolonged teenage glandular fever is a label which crops up time and time again in lupus patients and prolonged periods off school in many SLE patients is a recurrent theme.
  4. Severe reaction to insect bites
    This is a feature of so many lupus patients. Not only are they susceptible to insect bites but often reactions are severe and prolonged – the skin is a major organ affected by lupus.
  5. Recurrent miscarriages
    Lupus itself seems not to be a cause of recurrent miscarriage but where the antiphospholipid syndrome (APS) is present, recurrent spontaneous fetal loss is can be significant.
  6. Premenstrual exacerbations
    Although difficult to quantify, it is believed that significant pre-menstrual disease flare is sufficiently prominent in lupus to be included in this list. All rheumatic diseases are clinically influenced by the menstrual cycle.
  7. Septrin (and sulphonamide) allergy
    Adverse reactions to these drugs is quite common in lupus and the clinical onset of the disease may have coincided with the use of eg Septrin.
  8. Agoraphobia
    Agoraphobia/claustrophobia are often present at a time when lupus disease is active. A history of these
    conditions can be protracted, lasting for months or even years. In many cases the history is not volunteered or the episodes are in the interim considered unrelated to lupus.
  9. Finger Flexor Tendonitis
    Artbralgia and tenosynovitis are common features in lupus and although not specific, the finding of mild to moderate ten-finger flexor synovitis is a useful pointer in the presence of other lupus features. It is subtly yet significantly different in pattern from other arthritic diseases.
  10. Family history of autoimmune disease
    As the genetics and statistics of the various autoininiune diseases become better defined, the strength of a particular family history will become more precise. The family history is important, as lupus is genetically determined.
  11. Dry Shrmer’s test
    A ‘bone dry’ Shirmer’s test (levels of eye moisture)
    points towards one of the autoimmune diseases and in
    the patient with vague or nonspecific symptoms is
    worth its weight in gold.
  12. Borderline C4
    Genetic complement deficiencies have been known to be associated with lupus for over three decades and in the diagnostically difficult patient, especially where a family history is present, repeated borderline C4 levels can be significant indicators.
  13. Normal CRP with raised ESR
    An important diagnostic aid. A very low CRP in an otherwise inflammatory situation is strongly supportive of lupus or primary Sjogren’ s syndrome.
  14. Lymphopenia
    In the patient with non-specific complaints and unremarkable blood tests, a borderline or low lymph count can be overlooked. It can be common in lupus and is certainly worth inclusion among minor criteria.
Information Courtesy of:
LUPUS UK
St. James House
Eastern Road Romford
Essex RM1 3NH

Tel : 01708 731251
Fax: 01708 731252

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