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Fever may not be a necessary diagnostic criterion for Kawasaki disease
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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