Læknablaðið : fylgirit - 01.05.1978, Page 15

and OUTCOME of these 100 patients.
While there is no way of predicting the subse-
quent course of disease for the individual patient,
our observations suggest three major patterns
that are largely determined by the mode of onset.
Ten of the 20 patients with an acute febrile
onset followed a POLYCYCUC ACUTE FEBRILE
COURSE, having as few as one to as many as
10 attacks yearly - primarily of high fever and
rash - and little or no arthritis. The remaining
10 patients developed POLYARTHRITIS or simul-
taneous arthritis of more than 4 joints. All 48
patients with a polyarticular onset remained
POLYARTHRITIC. Of the 32 'children with an
oligoarticular onset, 24 remained OUGO-
ARTHRITIS (also known as pauciarticular arthri-
tis) - defined as arthritis of 1 to 4 joints, while
8 became POLYARTHRITIC. Thus, the major
pattern of disease course proved to be POLY-
ARTHRITIC, a pattern present in 68 patients.
At this time, 15 years after we first saw
them, 63 of our patients are in complete re-
mission; there is no evidence of systemic or
articular activity, they are not taking any medi-
cation. Of the remaining 37 patients, 34 are
currently active and 3 have died.
Let us now look at the functional outcome of
these 100 patients. Only 13 are in the unfavor-
able American Rheumatism Association functional
classes IH and IV, or capable of little or no self
care. Each of them has had a course of poly-
arthritis which has been unremitting and deform-
ing. Also, of these 13, 10 had progressive hip
involvement, and 3 died. (I shall return to this
column shortly).
Of the 13 with unremitting polyarthritis, 7 had
serious problems of growth and development.
Note the obvious impairment of growth, including
breast development, in this 17-year-old patient
(on your left) when compared to her normal twin
sister (on your right).
Six of the 7 patients with stunted growth
developed pronounced micrognathia. Chin
recession appeared to correlate with progressive
cervical involvement, which also contributed to
the shortened stature of these patients. This
suggests that the hyperemia of adjacent cervical
inflammation may be causative, contrary to
popular belief that it is due to temporomandibular
involvement for which there was no correlation.
To illustrate - at the age of 12, this patient
had minimal zygapophyseal encroachment only at
C2-C3. Four years later, there was diffuse
apophyseal fusion - and, because of early
epiphyseal closure, underdevelopment of vertebral
bodies C^ to Cg.
This girl died. She had an acute febrile onset
at the age of 2 and within a year developed fiorid
polyarthritis. Secondary amyloidosis was detected
at this time. She survived till the age of 8,
when she died of renal failure. The remaining
2 deaths occurred in males, one 19 from post-
surgical sepsis after knee synovectomy, the other
25, of suicide.
When we analyzed functional outcome according
to the presence of rheumatoid factor and subcu-
taneous nodules, our findings did not bear out
previous impressions that rheumatoid factor is
associated with a less favorable prognosis. Of
our 23 patients with a latex fixation titer of
1:80 or greater, only 4 had a poor end result.
These 4 comprise 17 per cent of the 23 patients
who were seropositive, an incidence essentially
similar to the 13 per cent of our entire series
of 100 patients who were in functional classes
m and IV. Of the 9 patients with nodules, 3
or 33 per cent are currently incapacitated.
Subcutaneous nodules, therefore, signify a poor
prognosis.
So look for them. They occur most often at
the elbow but can occur over any pressure point,
such as the heel.
Returning to chronic iridocyclitis, patients
with oligoarthritis (all of whom achieved an
excellent functional outcome) paradoxically were
more prone to develop chronic iridocyclitis. In
faet, of the 9 children with iridocyclitis, 6 had
oligoarthritis, and 2 were blind in 1 eye when
first referred to us, the result of cataract in
one patient, a 6-year old, and of band keratopathy
in the other, an 8-year-old. Note the calcium
deposits in the cornea.
Our routine procedure for slit-lamp examina-
tions (instituted 15 years ago) is shown here.
These are performed monthly in patients with
previous iridocyclitis (because of the tendency
for recurrence), every 3 months in those with
minimal arthritis (because of their enhanced
susceptibilify), and every 6 months in all other
patients.
Of the 9 patients with iridocyclitis, 3 had a
recurrence, including the 2 with unilateral blind-
ness. Our routine ophthalmologic screening
enabled us to detect each silent recurrence and
5 initial asymptomatic cases early enough
for successful treatment so that visual loss has
not occurred in any of these 8 patients.
During the past 15 years, 88 of our 100
ehildren have been maintained only on aspirin.
Of the remaining 12 patients, 4 also needed oral
steroids for more than one year (3 because of
protracted iridocyclitis) and 8 received gold
therapy.
Although I am greatly encouraged by the gener-
ally favorable prognosis for this conservative
program of management, I do not wish to give
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