SJOGREN’S SYNDROME
Systemic Features
By Dr Ian Griffiths, Dr Elizabeth Price,
Dr Clive Kelly
and Dr Patrick Venables
Advances in the understanding of Sjogren’s syndrome
during the past three decades have shown that the disorder
is extremely variable in its severity, presentation and
disease associations. The recognition that Sjögren’s
syndrome may be linked to clearly defined diseases (e.g.
rheumatoid arthritis) suggested the separation into primary
Sjogren’s syndrome (when no associated disease existed)
and secondary Sjogren’s syndrome (when a well defined,
associated disease was present). This article concentrates
on primary Sjögren’s syndrome. Even within this
group it is clear that in some cases the disease is limited
to dryness of eyes and mouth without widespread clinical
or laboratory features (non-systemic Sjogren’s syndrome)
whereas in others many other organ systems (e.g. joints,
blood vessels, skin etc.) may be affected (systemic Sjögren’s
syndrome). Some of the features in this latter group are
described together with approaches to treatment when necessary.
It is important to stress that only a small minority of
patients with Sjogren’s syndrome will develop these
problems, even if the disease goes on for many years.
1) FAILURE OF SECRETONS
Almost everyone with Sjogren’s syndrome complains
of dryness of mouth and grittiness of the eyes. This is
dealt with in detail in the rest of this booklet. It is
often not realised that other secretions can be affected
as well. This includes dryness of the air passages such
as nose and trachea. This tends to make the airways hypersensitive
to irritants and very few Sjogren’s sufferers are
current smokers (this may be a hidden benefit of the disease!).
Sjogren’s syndrome also affects the sweat glands resulting
in a dry skin which may be very sensitive to strong sunlight.
For this reason most sufferers use barrier creams in summer
and moisturising creams such as “E45” to keep
their skin in good condition. Occasionally patients mention
dry hair and a change in the brand of shampoo may alleviate
one of the less common manifestations of the disease.
The large bowel may be affected in a functional way, producing
what is sometimes called the “irritable bowel syndrome”.
This can cause lower abdominal pain and alteration in bowel
habit. Although the mechanism is not understood, it is possibly
similar to the “irritable airways” and may be
associated with low volume intestinal mucous secretions.
Treatments consist of increasing the fibre (bran) content
of the diet, and occasionally the use of anti-spasmodic
drugs.
Failure of mucus production in the bowel may cause constipation
in some patients. In general, simple measures such as ensuring
a high fibre content in the diet, are sufficient to control
these problems.
2) GYNAECOLOGICAL PROBLEMS
Many patients complain of vaginal dryness. Traditional lubricating
jellies such as “KY jelly” have only a short
lasting effect and need to be used frequently. “Replens”
(Unipath) has been recently introduced as a non-hormonal
vaginal moisturiser, which requires less frequent application
but is expensive. Both are available “over the counter”
without a prescription. Another product recently available
is “Astroglide” (Harlow Lubricants) which is
available by mail order. Oestrogen containing creams or
pessaries may be associated with a more sustained improvement
in the vaginal dryness. After an initial period of daily
use they need only be applied two or three times weekly
to maintain their effect. They are only available on prescription
and require medical supervision.
Surveys have shown no increase in infertility or miscarriage
in patients with SS and pregnancy is usually straightforward.
However, in about 5% of pregnancies in women with SS who
also have antibodies to Ro and La, the antibodies cross
the placenta and affect the babies’ heart rate and
can be detected on scans from about 12 weeks of pregnancy.
Expert treatment and the involvement of a paediatrician
from an early stage of pregnancy is recommended if this
abnormality is detected. It must be emphasised that the
presence of antibodies to Ro and La is not an indication
for abortion as the condition is rare. If the baby is affected
more than 90% do well.
Hormone replacement therapy (HRT) can improve vaginal dryness,
relieve hot flushes and improve overall well-being in menopausal
patients with SS. There is also a beneficial effect on bone
quality and long term treatment can help prevent the development
of osteoporosis (bone thinning). Previous concerns that
HRT might make SS worse are not supported by recent studies
and it may benefit some patients. There are many different
preparations available such as whether the patient has had
a hysterectomy, need to be taken into account by the prescribing
doctor.
3) NON-SPECIFIC SYSTEMIC FEATURES
Tiredness, lethargy and malaise, are features common to
many sufferers, sometimes amounting to a sense of complete
exhaustion. They are often not associated with any other
overt clinical features and can be a source of social frustration
as the person otherwise “looks well”. Sometimes
the tiredness follows a cyclical course during the day and
may also be worse pre-menstrually. Laboratory tests for
other causes of tiredness (e.g. thyroid disease and anaemia)
are usually normal. Treatment seems to make little difference
to this aspect of the disease and most sufferers find a
“coping strategy” to suit their needs (e.g.
a period of rest during the mid-afternoon).
Fever is a relatively uncommon feature which usually produces
no symptoms but can cause diagnostic confusion. Temperature
is rarely more than 1 or 2 degrees Celsius above normal
(370C), and tends not to fluctuate. Weight loss may occur
as a non-specific feature of very active disease and reverses
as the disease settles.
4) SYSTEMIC INVOLVEMENT
Joint involvement in arthritis is common but usually follows
a relatively mild course. Small joints in the hands and
feet tend to be affected. Pain is often more troublesome
than swelling, and inflammation of the joints leading to
destruction and deformity is rare in primary Sjogren’s
syndrome. Anti-inflammatory drugs (e.g. ibuprofen) may be
helpful for controlling joint involvement. True inflammation
of the muscles (myositis) is very rare but is recognised
as causing pain, tenderness and weakness of the muscles,
particularly around the shoulders and pelvis. Steroids may
be required.
Increased sensitivity of blood vessels to the cold causing
“white finger” (Raynaud’s phenomenon)
is common. The best management involves trying to avoid
the precipitation factor (e.g. wearing warm gloves) and
avoiding other aggravating factors (e.g. smoking). Sometimes
drugs (e.g. nifedipine) can help but results are often disappointing.
Involvement of the heart is rare, but pleurisy, causing
chest pain, and inflammation of the heart lining (pericarditis)
can occur.
The kidney may be involved rarely. Sjogren’s syndrome
can cause the kidney to lose its ability to excrete hydrogen
ions (acids) which requires treatment with oral sodium bicarbonate.
True inflammation of blood vessels may occur and is termed
“vasculitis”. This tends to cause rash or ulceration
of the skin in the legs. These problems may require steroid
or immuno-suppressive drugs.
There remains much uncertainty about the degree to which
brain and nerve tissue may be involved in Sjogren’s
syndrome. Migraine does appear to be more common, but evidence
of other problems (e.g. epilepsy and strokes) generally
seems to suggest that these are not increased (contrary
to some recent reports from the US). Some sufferers develop
true inflammation of the lungs not due to infection. This
can cause breathlessness on exertion and a non-productive
cough.
Many patients complain of enlargement of lymph nodes or
glands. They are not tender and cause no symptoms. Under
the microscope, the appearance again is one of “non-specific”
inflammation. The earlier studies on Sjögren’s
syndrome suggested that there was an increased risk of malignancy
(lymphoma) occurring in lymph glands. More recent studies
suggest this risk is now extremely low.
Mild anaemia, low white cell and platelet counts may be
detected but these very rarely cause symptoms or clinical
problems.
So the glands in the body release essential proteins (hormones)
into the blood circulation. Evidence is accumulating that
these glands may also become inflamed, and secondary to
this, become underactive. At present the strongest evidence
is for an increased risk of underactivity of the thyroid
gland occurring in some subjects. Treatment is with the
appropriate hormone replacement.
Although Sjogren’s does not interfere with the menopause,
a common question relates to the advisability/safety of
using HRT (Hormone Replacement Therapy) after the menopause.
Current evidence would suggest that HRT has no adverse affects
on Sjogren’s and the indications/precautions would
be the same as for any other individual.
CONCLUSIONS
Sjogren’s syndrome is a heterogeneous and, at times,
bewildering disorder. For many sufferers the disease will
remain localised in the eyes and mouth, whereas for others
differing and varied additional organs of the body may be
affected. Overall the involvement (in terms of severity)
of the other organs tends to be to a lesser degree than
that seen in the related disorder systemic lupus erythematosus.
Information Courtesy of:
BSSA
The BSSA is a self-help organisation for people with SS.
It aims to spread information about the disease and how
to alleviate it’s symptoms.
Unit 1
Manor Workshops
Nausea Wall Lane
NAILSEA
BS484DD
Tel: 01275 854215
Email: BSSAssociation@compuserve.com
Note:
WebHealth has additional articles about Sjogren's Syndrme at:
Systemic Features By:
Dr Ian Griffiths, Dr Elizabeth Price, Dr Clive Kelly and Dr Patrick Venables
and
The Problems of Sjogren's Syndrome By:
Dr CW Hutton, Consultant Rheumatologist, Demford Hospital, Plymouth
and
Sjogren's Syndrome and the Gastro-Intestinal Tract By
Doctor Richard Mount Ford MD, FRCP, FRCR.
Consultant Physician, Department of Gastroenterology, Bristol Royal Infirmary
and
Neurological problems in Sjogren's Syndrome by Doctor Annabel Coote and Doctor Michael Snaith, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield.
and
Sjogren's Syndrome and the Respiratory System
A Brief History of Sjogren's Syndrome By
Doctor Ian D Griffiths FRCR, Consultant Rheumatologist,
Freeman Hospital, Newcastle-Upon-Tyne
