Læknablaðið : fylgirit - 01.05.1978, Page 12

yet to be determined.
The role of antinuclear antibodies, found in
up to 497o of patients, also remains to be clar-
ified. While comprising all 3 of the major
immunoglobulin classes, according to Barnett
of Los Angeles, the presence of IgG ANA
seems to be linked to more severe and prolonged
disease. Recent evidence by Schaller (in work
done at Taplow) also suggests that ANA may be
linked in some way to chronic iridocyclitis.
AbnormaUties of serum immunoglobulins may
have both prognostic and pathogenetic implications.
Our survey of 200 children revealed that patients
with elevated levels had a poorer functional status
and an increased incidence of hip involvement.
Ui a subsequent study of 148 chUdren at Boston’s
Robert Breck Brigham Hospital, 2 groups of
patients could be distinguished on the basis of
immunoglobulin levels. One group, with chronic
active disease, had elevated serum levels of IgG,
IgA, and IgM along with lowered complement in
sera and synovial fluid. Another group, with
selflimited active disease, had elevated levels
of only IgG and IgA along with raised serum
complement levels. These findings imply that
IgG antigammaglobuUns may activate the comple-
ment system in a different manner than do IgM
antigammaglobulins, and that absence of the
latter imparts a favorable, self-limiting course
of disease.
Selective IgA deficiency occurs in up to 4’la of
JRA patients as compared to 0.2% of controls.
IgA deficiency may thus be related to patho-
genesis as a reflection of some form of immuno-
logic disturbance.
WhUe answers to this and other vexing
immunologic problems are stUl needed, one of
our major advances is the rather simple dis-
closure that certain clinical manifestations of
RA are different in children than in adults.
In a comparative survey, we found fever,
particularly high fever, and rash to be far
more frequent in children than in adults with
RA. This is difficult to explain, as is the
greater frequency in children of generalized
lymphadenopathy, splenomegaly, hepatomegaly,
and pericarditis.
Another difference is the occurrence of chronic
iridocycUtis present in 97o of children but in
none of our adults. A monarticular onset
appears more often in juvenUe than in adultRA.
On the other hand, subcutaneous nodules are
present in fewer chUdren than in adults. And
on histologic study, the nodules of children with
RA are more apt to resemble those of rheumatic
fever than the nodules of adults with RA.
A segmented neutrophilic leukocytosis is
detected in chUdren at twice the frequency as in
■ adults, and the level of the white ceU count is
generaUy higher. A positive latex fixation test
(titer of 1:160 or greater) was present in only
19% of children, whereas in adults it was found
in 76%.
In the only other comparative study, Bywaters
found fever, splenomegaly, and pericarditis to
be twice as frequent in children than in adults,
and rash 6 times more frequent, whUe subcu-
taneous nodules and a positive Rose-Waaler
test were only one-third as prevalent in chUdren
as in adults.
Early diagnosis rests on the recognition of 3 .
distinct modes of onset. These are acute febrUe,
polyarticular, and oligoarticular, which includes
monarticular. The character, frequency, and
severity of initial systemic and articular mani-
festations provide the best clues to each type
of onset, as gleaned from our prospective study
of 100 patients.
The patient group includes 62 girls and 38
boys, whose average age at onset was 6.8years;
the youngest chUd being 6 weeks of age, the
oldest 15 years. The current average age is
20 years; the youngest is 14, the oldest 32.
Of our 100 chUdren, the onset was acute
febrile in 20, polyarticular in 48, and oligo-
articular in 32.
Acute febrile onset is accompanied by prominent
systemic manifestations, particuiarly high fever,
rash, lymphadenopathy, and splenomegaly. Joint
manifestations are variable; occasionally, only
arthralgia is present.
In polyarticular onset, or synovitis of more
than 4 joints, the arthritis predominates and
is frequently generalized and symmetric, simUar
to adult RA. Systemic manifestations are less
frequent than in acute febrUe onset, and fever
is low grade.
OligoarticiUar onset, with arthritis of 1 to 4
joints, or when confined to a single joint (mon-
articular), is the mildest form. Systemic signs,
with the notable exception of chronic iridocycUtis,
are minimal or absent.
Acute febrile onset continues to remain a
frequent, yet unfámiliar, presentation that
deserves wider recognition. It is also caUed
systemic, by the eponyms Still’s type or
Still's disease, or when arthritis is absent,
as the Wissler-Fanconi syndrome.
Bqys slightly outnumber girls; a predilection
for boys occurs only in this mode of onset.
Recognition is easy when the young patient
has obvious arthritis in addition to marv of the
characteristic systemic features.
In 10 of our 20 chUdren, however, only
arthralgia was present initially. The mean
interval from the onset of high fever to the
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