Pediatric Rheumatology Online Journal
Vol 2, No. 2 2004 (114-118)
http://www.pedrheumonlinejournal.org
Barbara E. Ostrov, MD
Professor of Pediatrics and Medicine
Chief, Division of Pediatric Rheumatology
Pediatric Rheumatology and Rheumatology
The
Introduction
As pediatric rheumatologists, the
finding of swollen, boggy joints is helpful when definitively diagnosing the
various forms of juvenile idiopathic arthritis (JIA). In addition, change in the synovitis allows
accurate monitoring of our patients as we treat them. However, when we see patients with minimal
swelling but stiffness along with flexion contractures as well as other
evidence of an inflammatory process (lab changes and/or other symptoms, such as
uveitis or rash), the presence of “dry” synovitis must be considered.
Case example
The patient is a 19 year old who
presented at 9 years of age with high spiking
fevers, leukocytosis, severe microcytic anemia, ESR of 85
mm/hr and a diffuse polyarthritis. A
diagnosis of systemic JIA was made. He
never had recurrence of the systemic features subsequently, but had a
persistent polyarthritis (“systemic onset with a polyarticular course”) associated with severe
morning stiffness. He improved with high
dose then tapered doses of corticosteroids, along with NSAIDs and methotrexate
as well as physical therapy. Over the
next 5-6 years, his disease was well controlled with this regimen except for
hand and hip stiffness. Hip radiographs
revealed joint space narrowing bilaterally.
He had persistent stiffness and diffuse flexion contractures with
minimal swelling of all hand joints.
In 1999, etanercept was added to his regimen. Within 4-6 months time, the residual hand contractures completely resolved; the steroids were ultimately discontinued. No hand stiffness remained and he was able to make a fist normally. Some residual bony hypertrophy was noted of his PIP and DIP joints. His hip range of motion was mildly limited but no hip pain or morning stiffness are reported.
He is currently stable on etanercept twice a week, indomethacin twice a day and weekly injected methotrexate. This is an example of dry synovitis of hand joints in JIA. This case exemplifies the lack of response of the dry synovitis to methotrexate and low dose steroids but resolution of this type of synovitis once etanercept was added to the patient’s regimen.
Development of
criteria for dry synovitis
In 1974, Levinson gave a detailed
description of “dry” synovitis: “…
little overt synovitis, but the gradual development of limitation of movement
and appearance of deformities… no systemic features and there is little in the
way of constitutional disturbance.”
[Levinson J. JRA, Current
Diagnosis 4 (Editor F. Howard), 1974;
Saunders, Phila.] This
description was reiterated by Ansell [Ansell BM, Arden Surgical Management of
JCA. 1978] and she emphasized its
presence in polyarticular juvenile arthritis.
There are almost no references to
“dry” synovitis in more recent web-based or printed publications. Dorland’s On-Line Medical Dictionary defines
it as “synovitis but with little effusion.”
In a search of MedLine in February, 2003, only one reference including
this term was mentioned in a child with eosinophilic fasciitis [Patrone and
Kredich; Am J Dis Child; 1984; 138:363-5]. Scleroderma and related disorders, not
surprisingly, often present with hand joint contractures and minimal synovial
bogginess, which would be typical of “dry” synovitis. Other than these situations, no cases of dry
synovitis in other connective tissue diseases have been reported.
But - are there other differences
between “dry” and “wet” synovitis in JIA?
Does this finding signify a “scleroderma-like” process? Do these variants respond differently to
therapy? If there are significant
differences between patients with “dry” versus “wet” synovitis, then possibly
drug and treatment trials need to separate these groups. Finally, is there a difference in prognosis
between these forms of arthritis?
In order to answer these
questions, we need to begin by setting preliminary criteria for diagnosing
“dry” synovitis. Then, we can assess
its’ presence in our patient population and the response to current
therapies. It would be important to
determine if there are any differences in response to therapy, especially as
newer drugs, including additional biologics, become available.
Preliminary
Criteria:
Joint pain and stiffness reported in the patient history for
at least 3 months.
PLUS: minimal joint effusion and minimally palpable synovial
tissue on examination.
AND: association with the following 3 criteria:
1. morning stiffness of > 1 hour
2. loss of range of motion, with or without
contractures, of involved joints detected on physical examination
3. improvement in the symptoms and
physical findings with appropriate medical therapy
Retrospective Chart Review:
A retrospective chart review of 50
sequential out-patient visit charts of children with JIA, was performed to
determine which subsets were most associated with the presence of dry synovitis
using the above criteria. Only those
patients followed and treated for at least 6 months were included in my chart
review.
Diagnoses with inflammatory arthritis:
|
|
Wet synovitis (number / %) |
Dry synovitis (number / %) |
Both(number / %) |
|
Pauciarticular JIA |
18 (100%) |
0 |
0 |
|
Polyarticular JIA |
16 (70%) |
8 (30 %) |
8 (30%) |
|
Systemic onset JIA |
6 (86%) |
1 (14%) |
1 (14%) |
Overall, of the 50 children, 17% had dry synovitis. No child with pauciarticular disease had dry synovitis. Dry synovitis almost always occurred exclusively in hand joints.
An attempt was made to review the
differential response to various therapies between the patients (or joints)
with dry synovitis versus wet synovitis (as in the case above). However, since all the JIA patients with dry
synovitis also had wet synovitis in other joints, this was impossible to assess
retrospectively. One thing appeared
clear from the chart review (data not shown).
NSAIDs definitely were not adequate for the severe dry hand synovitis in
the polyarticular and systemic JIA patients.
As in the case example, anti-TNF therapy seemed to make the most
difference in those cases.
DISCUSSION
In order to try to answer the
questions raised at the beginning of this dialogue, I would propose the
following steps:
1. Development of a multi-center
prospective database to assess/revise the proposed preliminary criteria for the
diagnosis of dry synovitis. It would be important to use the new ILAR
criteria.
2. Use of the database to collect
information on diagnosis and treatment of these patients. Overlap diagnoses with features of
scleroderma plus JIA would have to be clearly delineated.
3. Using the database, one could
assess the differing response to therapy of children with dry synovitis versus
wet synovitis. Although it would be
tedious, it would be important to assess the differing response to treatment of
each type of synovitis (dry synovitis of hands versus wet synovitis of knees in
the same patient, for example).
4. If the database is collected over
several years, a differential prognosis between these forms of synovitis may be
noted.
5. Based on these data, subsequent
recommendations about the inclusion, exclusion or separation of these patients
in research and drug therapy trials could subsequently be made.
The issue of dry synovitis is too much a matter of
speculation and anecdotal opinion. It would be an important step to study dry
synovitis with this or other efforts and change our opinions to more
evidence-based medicine.
To comment on concept and proposal regarding dry synovitis
please e-mail PROJ at lww@uchicago.edu. Comments may be published in May/June PROJ
issue.