LUPUS
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 Road
Romford
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
