EDITORIAL
Oligoarticular
JIA: Is it a benign disease?
Thomas J. A. Lehman MD
Hospital for Special Surgery
I see the issues of oligoarticular JIA
in the context of the differences between “textbook answers” and “clinical
reality.” We all often refer to the need
for published and refereed information. What better guarantee of “truth” can there be
than peer review and publication? Yet
I believe that evidence-based medicine can have its limits. For example, which
of us publishes our mistakes? Or repeat
the same mistake several times to prove that the unfortunate outcome was not
just a coincidence? Despite admonitions
for journals to publish negative research results, those brave or foolish
enough to attempt to publish such information so that others might learn from
this mistakes often have these efforts rejected.
Physicians who have been in clinical
practice for a prolonged period may become aware that there is a body of
information which has never been formally codified in journals or textbooks,
but is often accepted as “truth.” It may
range from the simple statement by an attending to a fellow that, “We use or
don’t use that approach here,” to an informal comment at a national meeting by
a fellow physician who says simply, “We have had the same experience many
times. So we just don’t do that
anymore.” Yet textbooks and the literature
may not mention these observations at all.
Our knowledge of oligoarticular JIA
(persistent and extended) may be one of the best examples of this type of
information. After all, a few textbooks
and informational resources for parents may still group HLA B27-positive, late
onset oligoarticular JIA in a teenager with a 3 year old young child with
oligoarticular JIA who has a single swollen knee and who is
Like many of us, I’ve
occasionally seen a child for a second opinion who has been diagnosed to have
oligoarticular JIA and who is not doing well. The parents have been told, have
read, believe, and hope that the disease is self-limited and mild. They may
experience a disconnect when their rheumatologist makes an assessment that the
disease is no longer benign and wants to start methotrexate, a drug they hear
might have a lot of possible side effects. The parents may wonder how the
doctor can recommend methotrexate when the child has a benign disease that they
are going to grow out of. Often the
answer may be that the child really does need to be on methotrexate. Their
original doctor may have lost some credibility by not have advising the parents
initially and frequently thereafter that not all pauciarticular children do
well.
It’s important to be clear. True oligoarticular JIA, persistent or
extended, is not always a benign disease.
Every textbook agrees the most common complications of true oligoarticular
JIA are leg length discrepancy and uveitis.
But let’s not forget that the typical case of oligoarticular JIA is a
young child (typically 2 – 6 years of age) with a rapidly developing body
image, and a variety of important physical and mental developmental milestones
to accomplish. Impose pain, swelling, and limitation of motion, morning
stiffness and gel effect in this setting and there may be nothing benign about
the condition at all. It is likely that
the arthritis had been ongoing for weeks to months before the child reached a
pediatric rheumatologist. Even if no
boney overgrowth is present, these effects will have a significant psychosocial
impact and a developmental impact if the arthritis is left untreated. When boney overgrowth has developed because
the disease has been untreated for too long there will be an aberrant gait,
possible flexion contractures, and perhaps the need for a lift. These may be minor issues to the physician
who is caring for a possibly mild to minimal arthritis problem but may be major
issues for the family and child which can have life long consequences.
The consequences of uveitis and other
joint involvement are even more profound.
Fortunately most children with oligoarticular JIA do not develop uveitis,
but for those who do develop it may have a major, life-changing
complication. Yes, minor uveitis may
respond well to topical steroids. But ever try putting drops in a small child’s
eyes four to six times a day? It’s often a major stress. Are all the struggling
and worry and exasperation that may transpire benign for the child or
parents? Not likely. We are not even
considering the consequences for children who do have visual damage. The
articles in this issue of PROJ illustrate other systemic disease that may occur
in a child with oligoarticular JIA. The clinician should watch for involvement
of other joints, even the TMJ and atlo-epistropheal.
What does all this mean? I would suggest that if we take
oligoarticular JIA disease lightly, we may convey the wrong information to the
family, verbally and non-verbally. If we
seem unconcerned and overly optimistic, why should the parents be conscientious
about giving the medicine and getting the eye exams? Furthermore, we need to be conscientious
about making sure you get the disease under control. Controlling the information will control the
boney overgrowth and minimize the pain, swelling, limitation of motion, and
discomfort. All of these issues have a major and long lasting impact on the
family and child. There is also plenty of experience to suggest that vigorous
NSAID therapy may help to control uveitis.
It may not be enough, but it is incorrect to assume it has no role.
I favor an aggressive approach to
treatment of these children. For example, children whose oligoarticular disease
does not promptly come under control need to be treated aggressively to bring
the disease under control. In my
opinion, if you’ve been treating the child for three months and tried more than
one NSAID without an adequate response, this is not a typical oligoarticular
response, no matter what joint is involved.
(Note: We are also only too aware that NSAIDs in the 2005 are not
necessarily benign drugs.)
It may be time to get more aggressive with
a remissive drug and not allow the inflammation to continue. I do occasionally
hear rheumatologists say things like, “I would never use methotrexate in an
oligoarticular child.” I agree that it is unusual for us to have to use
methotrexate or biologics in a “true oligo”, e.g., with one to three joints
involved, but it may be crucial to that child’s well-being. Joint injections
with corticosteroids may be helpful, but seldom stop aggressive disease like a
remissive drug. An occasional child may have only one or two knees involved,
but still develop a destructive arthritis in a knee that requires a remissive
drug. Some oligoarticular children may have major functional problems that
interfere with their normal lives and may need methotrexate or etanercept. This is a simple message: Don’t rule out
aggressive treatment.
I would also
caution against defining oligoarticular JIA by counting joints. It may fit the original JRA criteria, but it
suits the 1970’s and 1980’s better than 2005. Long ago I showed my mentor Dr.
Virgil Hanson a child with a diffusely swollen ring finger – three joints
involved total. When he called it
“pauciarticular JRA” I commented that it didn’t look like any “pauci” I’d ever
seen. “Well, it probably isn’t the same
thing,” he promptly agreed. “But it fits
the criteria and we don’t know what else to call it.” Well that was almost thirty years ago. Now we do know a bit better what to call it
(psoriatic, extended oligo) and we know it behaves very differently and needs
to be treated very differently. If the
child’s disease in front of you is not behaving like typical oligoarticular
JIA, I would suggest that you don’t just count joints and think that the
resultant number limits your therapeutic options. You are responsible for
getting the best result for the child in front of you and kids always don’t
read the book or follow perfectly criteria written down by a committee. The
rheumatologists on those committees (including the ones that established the
JRA and the JIA criteria) knew that there were exceptions and expected
rheumatologists to allow for exceptions, too. Some of these children will
develop extended oligoarticular disease, develop complications, or never had
oligo disease in the first place.
Let me suggest some hypothetical
guidelines for predicting that a child may turn out to be an “exception”:
1)
2)
Hemoglobin less than 10.5
3)
Involvement of any joint other than the knee raises the
possibility of an exception:
a)
ankles–could be an oligoarticular but could be
polyarticular, systemic, or psoriatic.
b)
toes–very unusual to be an oligo-more likely psoriatic or
poly JIA.
c)
wrists–unusual-think poly, systemic, or psoriatic.
d)
finger PIP’s or MCP’s–rare in oligos-think polyarticular,
systemic, or psoriatic disease also.
e)
elbows–unusual-think psoriatic, systemic, or polyarticular
disease.
f)
Hips or lumbar spine–never in oligo’s-suggests
spondyloarthropathy or psoriasis early in disease course; for the hips alone,
if hip problems occur late, it may suggest systemic or polyarticular disease.
g)
cervical spine-uncommon-think extended oligoarticular
disease or polyarticular or systemic.
In conclusion, I
would suggest that we all maintain a health respect for oligoarticular JIA.
True, it does
not have the risk of polyarticular or
systemic JIA or most spondyloarthropathies, but it is not a benign condition in
some children. Be cautious in your predictions of early remission and an
excellent prognosis. Be aware of the complications of uveitis, unusual
involvements like TMJ disease or cervical spine disease, or destructive
arthritis. Most important, be ready to treat aggressively if the child’s
arthritis starts to deviate from the typical oligoarticular course.