Childhood
Rheumatic Diseases are a group of diseases
in children with similar presentations and
pathology. The underlying problem is inflammation
in the connective tissues of the body. The
cause is unknown and the precise diagnosis
is sometimes difficult to make. There are
many different childhood rheumatic diseases
and there are different criteria by which
they are diagnosed.
Juvenile Chronic
Arthritis
What
is JCA?
Juvenile Chronic Arthritis
(JCA) is a chronic condition that causes inflammation
in one or more joints before the age of 16
years. It is the most prevalent form of chronic
arthritis in children. There are different
disease patterns and they behave differently,
but all have joint inflammation in common.
What Causes JCA?
The cause of JCA is unknown.
Two theories are that it results from an infection
with an unknown organism, or that it is the
result of an autoimmune reaction. A person's
genetic make-up is also important and some
genetic markers are common in certain types
of rheumatic diseases.
When Should I Suspect
that my Child has JCA?
Limping, stiffness of joints
on waking and reluctance to use a limb may
be clues to the diagnosis. However, JCA may
be difficult to diagnose because young children
compensate well for loss of function and may
not complain of pain.
How do We Diagnose
JCA?
There are three main forms
of JCA, which are separated by how they begin.
In pauciarticular JCA, four or less joints
are involved in the beginning. This is the
form that usually begins in young girls as
a swollen knee. The important problems in
this condition are inflammation of the eye
and its effects on bone growth. The child
will need to be assessed closely by the ophthalmologist
and orthopaedic surgeon.
Polyarticular JCA is the form
in which five or more joints are affected,
and can begin at any age. Some of these patients
have adult type rheumatoid arthritis, which
began at an earlier age than usual.
The most worrisome form of
JCA is systemic onset type. This begins with
very high fever, skin rash and with evidence
of inflammation in internal organs as well
as joints. About 10% of children have this
type.
What are Some Problems
that May Occur in JCA?
Joint disease may result in
pain, stiffness and subsequently limited movement
in that joint. Wasting of muscles may also
occur. Altered growth is sometimes severe
and special shoes or even operations may be
needed. The eyes may be affected as mentioned
above.
How is it Treated?
-
Control
the inflammation with medications
Non-steroidal anti-inflammatory (aspirin-like)
drugs are often used to control the pain
and inflammation. They are enough for many
children, but occasionally, steroids are
sometimes used in severe cases but there
are side effects. Taking too much steroids
for a long time causes problems like slow
growth and weak bones. A child should be
taken off steroids or maintained at the
lowest dose where possible.
-
Prevent
or correct loss of range and function
Physical and occupational therapy are important
as they can prevent disability. Splints
and regular exercise are often effective.
-
Normal
physical and emotional development
Children should participate in normal school
activities and extra-curricular activities.
-
Education
and support of the patient and parents
The family should be aware that children
can get arthritis and must be properly diagnosed
and treated. Children should be encouraged
to reach their full potential and not be
limited by lowered expectations. Patients
should also be informed of resources and
support groups they can contact.
What
is the Doctor's Role?
Care
of the patient involves the paediatrician, ophthalmologist,
orthopaedic surgeon, therapist, social worker,
nurse and teacher. The paediatrician usually
co-ordinates this care. He also monitors for
any complications and institutes treatment when
needed.
What
is the Patient's Role?
With
proper therapy, children with all these forms
of arthritis will get better with time. Indeed
the vast majority will grow up to lead lives
without significant difficulty. It is important
that the patient should be actively involved
in his or her own care. He should participate
in physical and occupational therapy, engage
in regular activities and have a positive outlook
regarding his condition.
Dermatomyositis
What
is Dermatomyositis?
Dermatomyositis
is an inflammatory condition of skin and muscle.
Most often, it presents with increasing tiredness
and loss of muscle strength, accompanied by
a typical rash.
What
Signs are There to Look Out For?
Your
child may not be able to "keep up"
with other children, or has difficulty going
up and down stairs due to the muscle weakness.
He may also have a rash over the face, nail
beds and over the back of the joints of the
hand. These may occur gradually or rapidly,
and are often accompanied by a sense of being
unwell, joint pain and fever.
How
is it Diagnosed?
The
careful doctor can make a clinical diagnosis
from the history and examination. Laboratory
evaluation will show elevation of the muscle
enzymes. Other helpful investigations are the
electromyogram and muscle biopsy.
How
is it Treated?
Steroids
(prednisolone) are the treatment of choice for
mild to moderate dermatomyositis. In most situations,
this will result in improvement. For some children,
the skin rash is a significant problem, and
this can be treated with sun avoidance, sun
blocks and hydroxychloroquine.
For
those with persistent disease or unacceptable
steroid side-effects, methotrexate is beneficial.
Cyclophosphamide and IV gammaglobulin have also
been used.
A
program of physical and occupational therapy
to prevent continuing weakness and the development
of contractures is sometimes needed. Passive
movements are of benefit during the acute phase,
and active movements when the patient is better.
What
are Some Problems that May Arise?
These
are rare but important. The swallowing and gag
reflex should be assessed as choking is a possible
hazard. Pain in the abdomen due to inflammation
in the intestines, calcification of the skin,
eye problems and a change in behaviour, are
other rare complications.
What
is the Long Term Outlook?
The
long term outlook is generally good. Although
some children have severe disease which may
last for many years, most will eventually recover
nearly full function.
Systemic
Lupus Erythematosus
What
is Systemic Lupus Erythematosus?
Systemic
lupus erythematosus (SLE) is a challenging disease
with a wide variety of presentations. This is
because many systems of the body can be affected
separately or at the same time. The primary
problem in SLE is that the immune system is
persistently activated and this results in immune
complexes which are deposited in many parts
of the body.
Isn't
SLE Rare in Children?
SLE
remains an uncommon disease. It occurs more
commonly in girls and Orientals. However, it
should be considered in the differential diagnosis
of many symptoms and signs.
When
Do I Need to Think of SLE?
Many
manifestations of SLE are non-specific. These
include complaints of tiredness, fever, rash
on the face or elsewhere, joint pains and kidney
and blood problems.
Laboratory
tests are important in the diagnosis and assessment
of the child with SLE. These tests include the
blood count, blood chemistry panel, urine tests
and more specific tests such as anti-nuclear
antibody (ANA) and anti-DNA tests. There are
established criteria to make a diagnosis of
SLE and your doctor should be familiar with
these.
How
is it Treated?
Patients
with mild SLE can be treated with non-steroidal
anti-inflammatory drugs, hydroxychloroquine
and sunscreens. Children, however, tend to have
more active SLE and need to begin corticosteroid
therapy immediately. There are also those with
severe or life-threatening disease who need
high dose steroids and immunosuppressive agents
such as cyclophosphamide.
What
are Some Things to Look Out For?
SLE
is an unpredictable disease and may be fatal.
Kidney disease is one of the life threatening
complications of SLE. This may be reflected
as blood or protein in the urine, or as decreased
renal function.
Involvement
of the blood is also important and this may
present as bleeding, pallor or increased risk
of infection. Involvement of the brain is the
most difficult and may be due to the disease
or treatment with steroids.
What
is the Prognosis for My Child with SLE?
Our
understanding of the disease and the role of
genetics and environmental agents in its origin
has improved over the last ten years. In addition,
there have been refinements in the use of immunosuppressive
agents in those unresponsive to steroids. While
there are long-term concerns with regards to
treatment, the future is increasingly bright
for children with SLE who receive appropriate
care.
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