Læknablaðið : fylgirit - 01.05.1978, Page 13

development of arthritis proved to be 1.2 years
(the median, 2.5 months), with a range of 3
weeks to a remarkable span of 9 years. One
girl had periodic fever that recurred regularly
every 3 months for as long as 9 years (from
age 3 to 12) before she developed polyarthritis.
Initially, when only arthralgia is present, fever
and rash are of the greatest diagnostic value.
Most often, the' fever pattern is quotidian or
double quotidian, with one or two daily peaks
above 39°C (102°F), while hyperpyrexia (fever
to 40.5°C or 105°F) is observed only occa-
sionally. Typically, diurnal ranges are wide,
sometimes as much as 5°C (9°F) so that both
hyperpyrexia and subnormal temperatures occur
within the same day.
Fever usually responds to aspirin, but the
amount needed varies from patient to patient -
from 90 to 130 mg/kg daily. It proved to be
110 mg in this patient weighing almost 23 kilo-
grams.
Occasionally, as much as 130 mg/kg may be
required (as depicted here). Each time the
critical amount was reduced, by as little as
0.3 gm daily, fever promptly recurred.
McMinn & Bywaters have reported similar
observations. Pushing above 130 mg/kg does
little more than promote salicylate intcxication.
Up to 15% of patients with high fever fail to
respond to aspirin; in these, corticosteroids
should be tried.
A characteristic rash occurs in up to 9ö/o of
acute febrile patients. It consists of discrete
or confluent macules or maculopapules found on
the trunk, face, or extremities - including the
soles and palms. The eruption is usually non-
pruritic, but it may itch, sometimes intensely,
in about 3% of patients.
Occasionally constant, the rash is more often
evanescent, appearing briefly in the late after-
noon or early evening, often in conjunction with
fever spikes. From day to day, individual
macules tend to migrate and the degree of
erythema varies. The rash is most florid when
the skin has been subjected to mildtrauma, such
as the pressure of underclothing (the diaper in
this instance). This manifestation is known as
the Koebner phenomenon, and may by diagnos-
tically useful when parents report rash that is
not present when the child is being examined.
The typical rash may then be induced by lightly
scratching or rubbing the skin. Within several
minutes, blotches of macules will appear that
often persist for a day or two.
Generalized lymphadenopathy is frequent and
may be so prominent, particularly in epithrochlear
or axillary nodes, as to suggest leukemia or
lymphoma. Enlarged mesenteric lymph nodes
may cause abdominal pain or distention that may
suggest an acute surgical abdomen. In 3 of our
20 patients, such misdiagnosis led to an incon-
clusive and needless exploratory laparotomy.
Like high fever, rash, and lymphadenopathy,
splenomegaly occurs frequently, and was present
in 15 of our 20 children. Hepatomegaly is less
frequent, but when massive may be accompanied
by abdominal pain and distention as well as
abnormalities of liver function and nonspecific
histologic changes. In addition to direct hepatic
involvement, abnormalities of liver function may
also result from therapy with salicylates.
Cardiac involvement may have serious, if not
fatal, consequences, particularly myocarditis
because it may rapidly induce cardiac enlargement
and heart failure. Pericarditis, which is more
frequent than myocarditis, is usually a benign
manifestation that tends to recur. Precordial
pain or dyspnea are rarely present, and most
attacks remain asymptomatic and undetected
unless the child is monitored regularly for evan-
escent friction rub, cardiomegaly, ECG changes,
or - by the most sensitive of all indicators -
typical echocardiographic abnormalities.
Penumonitis or pleuritis frequently accompany
carditis but may also occur independently.
Iridocyclitis is rare (1 patient) while subcutaneous
nodules are notably absent in this mode of onset.
That prominent systemic manifestations can
also occur in adults with BA has gained renewed
interest as a result of 2 recent publications, one
entitled "Still's disease in the adult," the other
"adult onset JBA." Bywaters describes 14 adults,
all women, with an illness characterized by fever,
rash, polyarthritis, and a raised sedimentation
rate. The 9 adults reported by Aptekar had
similar manifestations except that all patients
were men. In both groups, serologic tests for
rheumatoid factor were uniformly negative, and
the onset usually occurred during early adult
years.
In polyartieular onset, the large joints are
most frequently involved. But the arthritis may
be generalized, like adult BA, with symmetrical
swelling of hand and wrist joints.
In younger children, a fusiform swelling
between the finger joints often replaces the
more typical swelling at the joints.
Scant attention has been focused on involvement
of the foot. Yet, the feet are affected in almost
half of all patients, often constituting the major
source of disability. The metatarsophalangeal
joints are usuaUy the first to become swollen
and tender, and may or may not be accompanied
by swelling of interphalangeal joints.
Affliction of the cervical spine is frequent. The
earliest X-ray changes include demineralization
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