Kawasaki disease diagnostic criteria
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dildar Hussain, MBBS [2]
Overview
Kawasaki disease is diagnosed clinically (by medical signs and symptoms), and there are no specific laboratory tests that can tell if someone has Kawasaki disease. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen a physician several times. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, and juvenile idiopathic arthritis. Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis, and include, mucositis (erythema of the palatine mucosa), fissured erythematous lips, "strawberry tongue", rash (polymorphus, usually urticarial erythematous rash mainly in external extremities. The rash can spread to trunk), extremities changes (edema of hands and feet, erythema of palms & soles, desquamation of fingertips, bilateral non-exudative conjuctival erythema), and cervical lymphadenopathy of at least 15 milimeters.
Diagnostic Criteria
- Kawasaki disease is diagnosed clinically (by medical signs and symptoms), and there are no specific laboratory tests that can tell if someone has Kawasaki disease.
- It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen a physician several times.
- Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, and juvenile idiopathic arthritis.
Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis.
(1) Mucositis - Usually erythema of the palatine mucosa, fissure erythematous lips, and "strawberry tongue"
(2) Rash - The rash can spread to trunk and is polymorphus, usually urticarial erythematous rash, and is mainly in external extremities.
(3) Extremities changes - Including edema of hand and feet, erythema of palms & soles, and desquamation of fingertips
(4) Bilateral non-exudative conjuctival erythema
(5) Cervical lymphadenopathy of least 15 milimeters
- Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria.
- In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease.
- In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.
AHA Scientific Statement on Kawasaki Disease
Recommendations for Cardiovascular Assessment for Diagnosis and Monitoring During the Acute Illness
| Class I |
| "1. Echocardiography should be performed when the diagnosis of KD is considered, but unavailability or technical limitations should not delay treatment.(Level of Evidence: B) " |
| "2. Coronary arteries should be imaged, and quantitative assessment of luminal dimensions, normalized as Z scores adjusted for body surface, should be performed.(Level of Evidence: B) " |
| "3. For uncomplicated patients, echocardiog- raphy should be repeated both within 1 to 2 weeks and 4 to 6 weeks after treatment.(Level of Evidence: B) " |
| "4. For patients with important and evolving coronary artery abnormalities (Z score >2.5) detected during the acute illness, more fre- quent echocardiography (at least twice per week) should be performed until luminal dimensions have stopped progressing to determine the risk for and presence of thrombosis.(Level of Evidence: B) " |
| Class IIa |
| "1. To detect coronary artery thrombosis, it may be reasonable to perform echocardiography for patients with expanding large or giant aneurysms twice per week while dimensions are expanding rapidly and at least once weekly in the first 45 days of illness, and then monthly until the third month after illness onset, because the failure to escalate thromboprophylaxis in time with the rapid expansion of aneurysms is a primary cause of morbidity and mortality . (Level of Evidence: C) " |