Published in Infectious Diseases in Children December 2009
Fever may not be a necessary diagnostic criterion for Kawasaki disease
A 3-month-old boy admitted to Cincinnati
Children’s Hospital Medical Centerwith all of the classic symptoms
of Kawasaki disease except fever experienced multiple coronary aneurysms,
according to a published case report — emphasizing the difficulty of
diagnosing the disease in infants.
Remittent, high fever has been among the most
consistently manifested symptoms of the disorder since the first case reports
in the 1960s. “Existing guidelines consider the presence of fever for at
least five days a requirement for the diagnosis of classic and incomplete
Kawasaki disease, and the description of Kawasaki disease without fever is
virtually nonexistent in the published data,” the researchers wrote.
Despite the presence of nonexudiative bilateral
conjunctivitis; erythematous lips and tongue; rash; and prominent cervical
lymphadenopathy, physicians delayed administration of IV immunoglobulin and
aspirin therapy due to normal axillary temperatures around 36·C.
An IV Ig dose of 2 g/kg along with 80 mg/kg of oral
aspirin daily was initiated after echocardiograms revealed aneurysms of the
proximal right, the distal left anterior descending and circumflex coronary
arteries. The patient’s left coronary system normalized, and his right
coronary artery diameter reduced from 7 mm (z score=19) to 3 mm
(z score=3.1) after a second dose of IV Ig was administered and
long-term initiation of low-dose aspirin and warfarin (Coumadin, Bristol-Myers
Squibb).
Warfarin was discontinued nine months after the initial
presentation, and the patient continues to grow and develop 2.5 years later.
“It is unclear why our patient did not develop a
fever typical of Kawasaki disease,” the researchers wrote. “We
speculate that a decreased ability to mount a fever response may be present in
some young infants, further contributing to the difficulty to diagnose Kawasaki
disease in this age group.”
Hinze CH. Pediatr Infect Dis J. 2009;28:927-928.
PERSPECTIVE
Young infants with Kawasaki disease are very frequently difficult to
diagnose because their manifestations of the illness are often quite subtle and
can be fleeting. It is highly unusual for fever to be completely absent, but
this gives us an opportunity to contrast the Japanese and the U.S. criteria for
the diagnosis of Kawasaki disease.
The U.S. criteria require five days of fever and the presence of at
least four of the five classic features (rash, oral changes, extremity changes,
eye findings and cervical adenopathy). The Japanese criteria, on the other
hand, require five of six classic features, with fever being one of the six
rather than a separate requirement. Therefore, in Japan a small but finite
fraction of children diagnosed with Kawasaki disease lack fever but meet the
other five criteria.
The young infants with a few but not all the features of Kawasaki
disease remain highly challenging, and this group can be at the highest risk
for development of coronary aneurysms among all Kawasaki disease patients if
not treated with IV Ig. I believe that liberal assessment of inflammation
markers (erythrocyte sedimentation rate and/or C-reactive protein) in patients
in whom there is reasonable suspicion of Kawasaki disease can provide an
important clue to the diagnosis, as they are very often markedly elevated at
least by the fourth or fifth day of illness to levels substantially higher than
those expected in children with acute viral illnesses or drug reactions.
– Stanford T. Shulman, MD
Infectious Diseases in Children Editorial Board
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