The juvenile spondyloarthropathies constitute are a group of chronic
inflammatory diseases of the joints (arthritis) and tendon attachments
to certain bones (enthesitis) affecting, predominantly, the lower
limbs and, in some cases, the pelvic and spinal joints (sacroliitis
- buttock pain and spondolytis - lower back pain).
In some cases, the onset of symptoms is triggered by enteric (gastro-intestinal)
or urogenital bacterial infections (reactive arthritis). Juvenile
spondyloarthropathies are significantly more common in people
carrying HLA-B27, a genetic marker, which predispose individuals
to this disease, although why this is the case is not fully understood,
The prevalence of some clinical features at onset and severity
throughout the course of the disease, differ in the childhood
form to those of adults, but may still resemble adult onset spondyloarthropathies.
Patients with juvenile idiopathic arthritis, classified into the
enthesitis related arthritis group (see JIA information), are
included in the group of juvenile spondyloarthropathies.
diseases are called juvenile spondyloarthropathies?
Despite some controversy, juvenile spondyloarthropathies include
the same diseases belonging to the group of adult spondyloarthropathies,
including ankylosing spondylitis, reactive arthritis (and Reiter’s
syndrome), psoriatic arthritis (of the spondyloarthropathy type),
and arthritis associated with inflammatory bowel disease (of the
spondyloarthropathy type). Some children, not fulfilling diagnostic
criteria for the disease enlisted above, may have the so-called
Other conditions, specifically the seronegative enthesopathy and
arthropathy (SEA) syndrome and enthesitis related arthritis refer
to juvenile spondyloarthropathies.
common is it?
Spondyloarthropathies are one of the most frequent forms of chronic
arthritis in childhood and account for about 30 % of children
with chronic arthritis.
Spondyloarthropathies are seen more often in boys, with the disease
starting mainly between 10 and 15 years of age. Since a great
part of the patients carry a genetic predisposing factor (HLA-B27)
the frequency of juvenile and adult spondyloarthropathies in the
general population and even in certain families (familial history),
depends on the frequency of this marker in the normal population.
are the causes of the disease?
The cause and precise mechanisms that cause juvenile spondyloarthropathies
are unknown. It is thought that the mechanisms that might be responsible
for the disease include several components of the immune system.
Spondyloarthropathies are associated with other kinds of chronic
inflammation for example of the bowel, the genito-urinary tract,
or the skin. Infections by some micro-organisms (i.e. Salmonella,
Shigella, Yersinia, Campylobacter and Chlamydia) may play a role
in triggering the arthritis in some children (i.e. reactive arthritis).
Large numbers of patients with a juvenile spondyloarthropathy
carry the genetic marker HLA-B27. This does not mean that every
individual carrying this genetic factor will have spondyloarthropathy.
For example, if the frequency of HLA-B27 in the population is
10 %, only 1 % of this population will develop the disease. If
someone else in the close family is affected with spondyloarthropathy,
the presence of HLA-B27 increases the risk to about 25 % for an
individual of that family to develop a spondyloarthropathy. Spondyloarthropathies
are present with a higher frequency in members of the family of
an affected child when compared to families of non-affected children.
Genetic factors, and in particular HLA-B27, refer to disease susceptibility.
They are not sufficient for disease development. The agreement
in the scientific community is that these diseases are multifactorial,
which means that they are the result of a combination of genetic
predisposition and exposure to environmental factors (probably
infections) that are still unknown.
it be prevented?
Prevention is not possible since the causes of the disease are
unknown. It is not useful to test other siblings for the HLA-B27
if they do not have any symptoms that can be linked to a spondyloarthropathy
Spondyloarthropathies are not contagious diseases.
are the main symptoms?
Juvenile spondyloarthropathies have common clinical characteristics:
1) Most common symptoms include joint pain and swelling, and limited
mobility of the joints.
2) Many children have oligoarthritis of the lower limbs. Oligoarthritis
means that the disease involves four or less joints. Those developing
chronic disease may have polyarthritis. Polyarthritis means that
the articular involvement is more extensive and affects five or
3) Arthritis mainly involves the joints of the lower limbs: the
knee, the ankle, the mid-foot, and the hips. Less frequently,
arthritis involves the small joints of the foot.
4) Some children may have arthritis of any joint of the upper
limbs, particularly the shoulders.
Enthesitis, the inflammation of the enthesis (the site where a
tendon or ligament attaches to the bone) is very frequent in children
with spondyloarthropathies. Commonly affected entheses are located
at the heel, in the mid-foot and around the kneecap. Most common
symptoms include heel pain, mid-foot pain and swelling, and kneecap
Chronic inflammation of the enthesis may lead to bony spurs (bony
overgrowth). These spurs occur particular in the heel causing
It is the inflammation of the sacroiliac joint, located in the
rear of the pelvis. It is rare at onset and most frequently occurs
five to 10 years after the onset of arthritis.
The most common symptom is alternating buttock pain.
Lumbar pain; spondylitis
Involvement of the spine is very rare at onset, but may occur
later in the disease course. The most common symptoms include
low back pain, morning stiffness, and reduced mobility. Low back
pain is frequently accompanied by neck and chest pain.
In the spine, long-term disease may cause the formation of bridges
between the spinal bones (“bamboo spine”). This occurs
in only few patients and after a long disease duration. It is,
therefore, almost never observed in children.
Acute anterior uveitis is an inflammation of the iris of the eye.
It is not frequent. The eye is acutely red and painful. Immediate
control by the ophthalmologist (the eye doctor) is necessary.
A small subset of children with spondyloarthropathy may have psoriasis
Psoriasis is a chronic skin disease with patches of scaling skin
mainly located on the elbows and the knees. The skin disease may
precede arthritis by years. In other patients the arthritis can
already exist several years before a first psoriasis spot occurs.
Some children with intestinal inflammatory disorders may develop
Inflammatory bowel disease (IBD) is used to designate chronic
bowel inflammation of unknown origin. These diseases are called
Crohn’s disease or ulcerative colitis.
the disease the same in every child?
The spectrum is wide. While some children have mild and short-term
disease, others have severe, long-term and disabling disease.
the disease in children different from the disease in adults?
Juvenile spondyloarthropathies differ from adult spondyloarthropathies
in some aspects.
1) Peripheral (limbs) joints are much more frequently affected
in the beginning of the disease in contrast to the more frequent
axial (spine) involvement in adults.
2) In children, the hip is more frequently affected.
is it diagnosed?
Doctors say it is a juvenile spondyloarthropathy if the onset
of the disease is before the age of 16, the arthritis lasts for
more than six weeks and the characteristics fit into the clinical
pattern described above (see definition and symptoms). The diagnosis
of specific spondyloarthropathies (i.e. ankylosing spondylitis,
reactive arthritis, etc.) is based on specific clinical and radiographic
It is clear that these patients should be treated and followed
by a paediatric rheumatologist.
is the importance of tests?
The HLA-B27, a cellular marker that is positive in up to 80-85
% of patients with juvenile spondyloarthropathies, is useful in
orientating the diagnosis. Its frequency in the general healthy
population is much lower (5–12 % depending on the region).
Therefore, it is not the presence of HLA-B27 by itself, but its
association with the characteristic signs and symptoms of spondyloarthropathies,
which has relevance.
Exams, such as erythrocyte sedimentation rate (ESR), or C-reactive
protein (CRP), give information about general inflammation and,
indirectly, about disease activity. They are useful in disease
management, although this is based much more on clinical manifestations
than on laboratory examinations. Laboratory tests are also used
to monitor possible side effect of the treatment (blood cell count,
liver and kidney function). X-ray examinations are useful to follow
disease evolution and assess joint damage caused by the disease.
Computer tomography (CT scan) and magnetic resonance imaging (MRI)
may be useful, especially in children, to evaluate the involvement
of sacroiliac joints.
it be treated or cured?
There is no curative treatment since the cause of spondyloarthropathies
is unknown. However, therapy can be very useful to control disease
and prevent damage.
are the treatments?
Treatment is based mainly on the use of drugs in combination with
physiotherapy and rehabilitation procedures that preserve joint
function and contribute to prevent deformities.
1) Non-steroidal anti-inflammatory drugs (NSAIDs). They are symptomatic
anti-inflammatory and antipyretic (fever reducing) medications.
Symptomatic means that they serve to control symptoms due to inflammation.
The most widely used in children are naproxen and ibuprofen. Aspirin,
although effective and cheap, is much less used nowadays due to
its risk of toxicity. They are usually well tolerated and gastric
discomfort, the most common side effect, is uncommon. NSAIDs are
not prescribed in conjunction with one another, but one NSAID
may be effective were another has failed.
2) Joint injections are used when one or very few joints are involved
and when joint deformity is a real possibility. The drug injected
is a long-acting steroid preparation.
3) Sulphasalazine is indicated in children that have a chronic
course of disease, despite adequate therapy with NSAIDs and steroid
injections. It is added to previous NSAID therapy (which has to
be continued) and its effect becomes fully evident only after
several weeks or months of treatment.
Experience with other drugs, including methotrexate, is limited.
New perspectives have been introduced in the last few years with
anti-TNF drugs (called biological agents). They selectively block
tumor necrosis factor (TNF), an essential mediator in the inflammatory
process. Yet there are no studies known about the effect or risks
of this kind of treatment in patients with juvenile spondyloarthropathies.
4) Corticosteroids have a role in the short-term management of
patients that are more severely ill. Topical steroids (eye drops)
are used in the treatment of acute anterior uveitis. In more severe
cases, peribulbar steroid injections or systemic steroid administration
may be required.
5) Orthopaedic surgery is usually needed for prosthetic joint
replacement, in the case of severe joint damage, particularly
in the hip.
6) Physiotherapy is an essential component of treatment. It must
be started early and should be performed routinely to maintain
range of motion, muscle trophysm and strength and to prevent,
limit or correct articular deformities. Moreover, if axial involvement
is prominent, the spine has to be mobilised and respiratory exercises
are the side effects of drug therapy?
The drugs used in the treatment of juvenile spondyloarthropathies
are usually well tolerated. Gastric intolerance, the most frequent
side effect of NSAIDs, is less common in children than in adults.
NSAIDs can cause increase in the blood levels of some liver enzymes,
but this is a rare event with drugs other than aspirin.
Sulphasalazine is reasonably well tolerated; the most frequent
side effects are stomach problems, elevated liver enzymes, low
white blood cells count and skin rash. Periodic laboratory examinations
are needed to monitor toxicity.
Methotrexate is also well tolerated. Gastroenteric side effects,
such as nausea and vomiting, are not uncommon. The administration
of folinic or folic acid is effective in reducing the frequency
of liver problems. Hypersensitivity reactions to methotrexate
can occur, but are rare. Periodic laboratory examinations are
needed to monitor toxicity.
The long-term use of steroids in significant dosage is associated
with several important side effects. These include stunting growth
and osteoporosis. Steroids at high doses cause a marked increase
in appetite, which can in turn lead to obesity. It is, therefore,
important to instruct children to eat foods that can satisfy the
appetite without increasing caloric intake.
long should the treatment last for?
It should last as long as symptoms and disease activity persist.
Disease duration is unpredictable. In some patients, arthritis
responds very well to NSAIDs. In these patients the treatment
can be stopped rapidly, within months. In other patients, with
a more prolonged or aggressive course of disease, sulphasalazine
and other medications are needed for years. Complete treatment
withdrawal is then considered only after prolonged and complete
about unconventional and complementary therapies?
There is no proof that any unconventional therapy has an effect
in the juvenile spondyloarthropathies.
long will the disease last for? What is the prognosis (predicted
outcome) of the disease?
The disease course can be different from one patient to another.
In some patients the arthritis disappears quickly, with little
treatment, within a period of months. In others the disease will
have periods of remission and recurrence. In other patients, arthritis
may follow an unremitting course.
At the beginning of disease symptoms are confined to peripheral
joints and the enthesis (tendons) in the vast majority of patients.
With disease progression, some of them may develop involvement
of the sacroiliac joints and the spine. These patients and those
with persistent peripheral arthritis, carry the higher risk of
developing joint damage in adulthood.
At the beginning of the disease it is impossible to predict the
could the disease affect the child and family’s daily life?
During the periods of active arthritis, almost every child will
experience limitations to some extend in daily life. Since mainly
the lower limbs are affected, walking and sports are the domains
were the limitations are felt the most.
Major attention should be paid to the psychological impact of
the disease on the child and family. A chronic disease is a difficult
challenge for the whole family and, of course, the more serious
the disease, the harder it is to cope. It will be difficult for
the child to cope properly with his disease if the parents don’t.
The parents often develop an abnormal attachment towards their
sick child and, in order to prevent possible problems, become
overprotective. This can cause the child to feel inadequate, which
can damage personality development with worse long-term damage
than the disease itself. A positive attitude from parents who
support and encourage the child to be independent as much as possible,
despite the disease, will be extremely valuable to help the child
to overcome difficulties. If the family cannot endure the burden
of the disease, psychological support is needed.
It is extremely important for the child to attend school regularly.
There are a few factors that may cause problems for school attendance,
including difficulty in walking, minor resistance to fatigue,
pain and stiffness. It is, therefore, important to explain to
teachers possible needs, such as proper desks and regular movements
during school hours to avoid articular stiffness. Patients should
take part, whenever possible, in gym lessons and the considerations
discussed below in the issue of sports, have to be taken into
School is a place where a child learns how to become an autonomous
person, productive and independent. Parents and teachers have
to do whatever they can to make the sick child participate to
school activities in a normal way, in order to have academic success.
A normal school life is also vital to ensure the child develops
a good capacity for communication with peers and adults.
Playing sports is an essential aspect of the everyday life of
a normal child. Therefore, the general tendency is to leave patients
to play the sports they want and to trust that they will stop
if a joint hurts. Although mechanical stress is not beneficial
in an inflamed joint, it is assumed that the little damage that
could ensue is much smaller that the psychological damage of being
prevented from playing sports with friends because of the disease.
This choice is part of a more general attitude to encourage the
child to be autonomous and to cope with the limits imposed by
Apart from these considerations, it is better to favour sports
in which mechanical stress to the joints is absent or minimal,
such as swimming and riding a bike.
There is no evidence that diet can influence the disease. In general
the child has to take a balanced, normal diet for his age. Overeating
has to be avoided in patients taking steroids since steroids increase
climate influence the course of the disease?
There is no evidence that climate can affect the disease.
the child be vaccinated?
Since most of the patients are treated with either NSAIDs or sulphasalazine,
a normal vaccination scheme can be advised. If a patient is being
treated with an immunosuppressive therapy (steroids, methotrexate,
anti-TNF etc.) vaccination with live attenuated viruses (such
as anti-rubella, anti-measles, anti-parotitis, anti-polio Sabin)
have to be postponed because of the increased risk of infections.
Vaccines that do not contain living viruses (anti tetanus, anti
diphtheria, anti polio Salk, anti hepatitis B, anti pertussis,
pneumococcus, haemophilus, meningococcus) can be administered,
the only theoretical risk being vaccination failure due to the
about sexual life, pregnancy, birth control?
There are no restrictions for having a normal sexual life or pregnancy
due to the disease. Yet, in patients taking medications, one should
always be very careful about the possible toxic effects of these
drugs on the foetus.
There is no reason for not having children, despite of the genetic
aspects of the disease. The disease is not lethal and even if
the predisposing genetic factor should be inherited, the siblings
have a much greater chance of not developing a spondyloarthropathy
than of develop one.
the child have a normal adult life?
This is one of the main goals of the therapy and it can be reached
in the majority of cases. Therapies for these kinds of diseases
in childhood have improved dramatically in the last ten years.
The combined use of pharmacological treatments and rehabilitation
is now able to prevent joint damage in the majority of patients.
Yet in patients with chronic disease, joint damage may be important
and can limit the patient’s daily life and professional