Incomplete Kawasaki disease with coronary artery involvement

doi:10.1016/S0022-3476(87)80503-6

The Journal of Pediatrics

Volume 110, Issue 3, March 1987, Pages 409-413

https://doi.org/10.1016/S0022-3476(87)80503-6 Get rights and content

We report four patients with Kawasaki disease in whom characteristic coronary artery abnormalities developed after illnesses that did not meet diagnostic criteria. An additional patient lacked a history of acute manifestations of Kawasaki disease, but severe Kawasaki-like arterial changes were noted at autopsy. Fever was present in four of the five patients, in three lasting from 7 to 14 days. Despite manifestation of few classic acute clinical features of Kawasaki disease, three of four patients had desquamation of the fingers and toes 10 to 14 days after onset of illness, and the fifth had desquamation several months prior to death. These patients were seen over a 2-year period during which 22 other children were seen with Kawasaki disease with coronary artery abnormallties. Thus, strict adherence to currently accepted criteria for diagnosis of Kawasaki disease may lead to failure to recognize incomplete forms of this illness, with potential sequelae of myocardial infarction or sudden death. This finding suggest that children with prolonged unexplained febrile illnesses, especially those associated with subsequent peripheral desquamation, should undergo echocardiography 3 to 4 weeks after onset of the illness. This practice would help to identify those patients with illnesses characterized by incomplete diagnostic criteria but in whom significant coronary abnormalities develop.

References (32)

SasaguriY et al.
Regression of aneurysms in Kawasaki disease: a pathological study
J Pediatr
(1982)
KatoH et al.
Fate of coronary aneurysms in Kawasaki disease: serial coronary angiography and long-term followup study
Am J Cardiol
(1982)
KohrRM
Progressive asymptomatic coronary artery disease as a late fatal sequela of Kawasaki disease
J Pediatr
(1986)
FlugelmanMY et al.
Acute myocardial infarction 14 years after an acute episode of Kawasaki's disease
Am J Cardiol
(1983)
GorgelsAPM et al.
Multiple aneurysms of the coronary arteries as the cause of sudden death in childhood
Am J Cardiol
(1986)
BurnsJC et al.
Coagulopathy and platelet activation in Kawasaki syndrome: identification of patients at high risk for development of coronary artery aneurysms
J Pediatr
(1984)
FukushigeJ et al.
Spectrum of cardiovascular lesions in mucocutaneous lymph node syndrome: analysis of eight cases
Am J Cardiol
(1980)
RobertsFB et al.
Polyarteritis nodosa in infancy
J Pediatr
(1963)
ChamberlainJL et al.
Infantile periarteritis nodosa with coronary and brachial aneurysms: a case diagnosed during life
J Pediatr
(1971)
ScottEP et al.
Periarteritis nodosa
J Pediatr
(1944)

Kawasaki disease—New York

MMWR

(1980)

Shulman ST, McAuley J, Pachman LM, Miller ML, Ruschhaupt DG. Epidemilogy of Kawasaki syndrome in an urban U.S. area...

PounderDJ

Coronary artery aneurysms presenting as sudden death 14 years after Kawasaki disease in infancy

Arch Pathol Lab Med

(1985)

McMartinDE et al.

Mutliple coronary-artery aneurysms in a child with angina pectoris

N Engl J Med

(1974)

KatoH et al.

Kawasaki disease: effect of treatment on coronary artery involvement

Pediatrics

(1979)

KorenG et al.

Probable efficacy of high-dose salicylates in reducing coronary involvement in Kawasaki disease

JAMA

(1985)

Cited by (163)

Kawasaki Disease
2017, The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic Approaches
Kawasaki disease (KD) is a multisystem acute self-limited febrile vasculitic syndrome of unknown etiology that predominantly affects previously healthy infants and young children. The diagnosis is based on characteristic clinical features with supplemental laboratory and radiographic criteria. To date, there is not a single specific definitive diagnostic test for KD. Serious complications include coronary arteritis, coronary artery aneurysms, stenosis, and thrombosis, which may lead to myocardial infarction, and, very rarely, rupture of a coronary aneurysm. KD is seen worldwide and is the leading cause of acquired heart disease in children in most developed countries including the United States and Japan. Although the underlying etiology remains unknown, the predominant theory involves an infectious trigger leading to an overwhelming inflammatory response in a genetically susceptibile host manifesting as the classic clinical features. Timely treatment with intravenous immunoglobulin (IVIG) and aspirin generally leads to dramatic improvement in the clinical manifestations and markedly reduces the likelihood of coronary abnormalities.
A Classification Tool for Differentiation of Kawasaki Disease from Other Febrile Illnesses
2016, Journal of Pediatrics
To develop and validate a novel decision tree-based clinical algorithm to differentiate Kawasaki disease (KD) from other pediatric febrile illnesses that share common clinical characteristics.
Using clinical and laboratory data from 801 subjects with acute KD (533 for development, and 268 for validation) and 479 febrile control subjects (318 for development, and 161 for validation), we developed a stepwise KD diagnostic algorithm combining our previously developed linear discriminant analysis (LDA)–based model with a newly developed tree-based algorithm.
The primary model (LDA) stratified the 1280 subjects into febrile controls (n = 276), indeterminate (n = 247), and KD (n = 757) subgroups. The subsequent model (decision trees) further classified the indeterminate group into febrile controls (n = 103) and KD (n = 58) subgroups, leaving only 29 of 801 KD (3.6%) and 57 of 479 febrile control (11.9%) subjects indeterminate. The 2-step algorithm had a sensitivity of 96.0% and a specificity of 78.5%, and correctly classified all subjects with KD who later developed coronary artery aneurysms.
The addition of a decision tree step increased sensitivity and specificity in the classification of subject with KD and febrile controls over our previously described LDA model. A multicenter trial is needed to prospectively determine its utility as a point of care diagnostic test for KD.
Endothelial dysfunction in children within 5 years after onset of Kawasaki disease
2013, Journal of Pediatrics
To evaluate endothelial function in children within 5 years after the onset of Kawasaki disease (KD).
A total of 46 children were enrolled prospectively as follows: 9 patients with KD and coronary artery lesions composed group 1, 15 patients with KD but without coronary artery lesions composed group 2, and 22 healthy age- and sex-matched children composed group 3. Flow-mediated dilatation (FMD) of the brachial artery, intima-media thickness of the common carotid artery, and biologic characteristics were compared among the 3 groups. Differences in the factors associated with endothelial function after KD were examined as well.
The mean age of the study group was 6.5 ± 1.7 years. The patients with KD were studied at a median interval of 3.3 years (IQR, 2.0-4.4 years) from the onset of disease. The percent FMD (%FMD) was significantly lower in group 1 patients (median, 4.4%; IQR, 2.6%-5.7%) compared with both group 2 (median, 9.1%; IQR 6.6%-10.7%; P < .01) and group 3 (median, 11.1%; IQR, 10.1%-13.9%, P < .01). The %FMD was statistically significantly lower in group 2 compared with group 3 (P < .05). There were no significant differences in the intima-media thickness among the 3 groups. There was a significant negative correlation between %FMD and the total duration of fever (r = −0.50; P = .013).
The children with KD already had arterial endothelial dysfunction within 5 years after the onset of illness. The longer the duration of fever, the greater the risk of inflammation-induced endothelial dysfunction.
Cardiac emergencies
2013, Emergency Medicine Clinics of North America
Citation Excerpt :
The cervical lymphadenopathy is generally unilateral, and usually 1 node is greater than 1.5 cm in diameter.76,77 Although most cases fulfill the principal diagnostic criteria, about 15% of cases have incomplete clinical presentations, with coronary artery complications.78 Therefore, any infant younger than 6 months with fever duration of 7 days or more, increase of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), and no other explanation for the febrile illness should have an echocardiogram.
L16. The specificities of pediatric vasculitis classification
2013, Presse Medicale
Kawasaki disease in 76 patients. Risk factors for coronary artery aneurysms
2011, Anales de Pediatria
La enfermedad de Kawasaki es una vasculitis sistémica aguda de la infancia, de etiología desconocida, considerada la principal causa de cardiopatía adquirida en la infancia en los países desarrollados. Por ello, es importante conocer las manifestaciones clínicas y las complicaciones de pacientes con enfermedad de Kawasaki en nuestro medio y buscar factores relacionados con la aparición de alteraciones cardiológicas.
Estudio descriptivo retrospectivo de 76 niños diagnosticados de enfermedad de Kawasaki desde enero de 1997 hasta mayo de 2008.
El 64,5% eran varones, con una edad media de 3 años y 4 meses. Las manifestaciones clínicas principales que presentaron fueron fiebre (media 8,13 días), exantema, hiperemia conjuntival bilateral, afectación bucal, afectación de extremidades, adenopatía única y artralgias. Entre los hallazgos analíticos destacaron leucocitosis, trombocitosis, proteína C reactiva (PCR) y velocidad de sedimentación glomerular elevadas, hipoalbuminemia, hiperbilirrubinemia, aumento de transaminasas y piuria estéril. Presentaron alteraciones coronarias 12 pacientes (15,7%), 2 insuficiencia mitral leve y 1 derrame pericárdico leve. Hubo una hepatitis colestásica. Todas las complicaciones se resolvieron sin secuelas.
Se identificaron como factores de riesgo para la aparición de aneurismas coronarios el sexo varón (OR = 1,24), exantema urticariforme (OR = 10,53) y PCR > 10 mg/dl (OR = 4,20).
Nuestros pacientes presentaron las manifestaciones clínicas y analíticas típicas de la enfermedad de Kawasaki. El 15,7% tuvo alteraciones coronarias leves. Son factores de riesgo de aparición de aneurismas coronarios el sexo masculino, exantema urticariforme y PCR elevada.
Kawasaki disease is an acute systemic vasculitis of childhood, of unknown origin, and is considered the leading cause of acquired heart disease in children. Therefore, it is important to know clinical manifestations and complications in children with Kawasaki disease in our environment and to look for risk factors for the development of cardiac complications.
Retrospective review of 76 children with Kawasaki disease evaluated from January 1997 to May 2008.
Of the patients studied, 64.5% were males. The mean age was 3 years and 4 months. The main clinical findings were fever (mean of 8.13 days), rash, bilateral non-exudative conjunctivitis, changes in lips and oral cavity, changes in the extremities, cervical lymphadenopathy and arthralgias. The most important laboratory findings were leucocytosis, thrombocytosis, elevated C-reactive protein and erythrocyte sedimentation rate, hypoalbuminaemia, hyperbilirubinaemia, elevated serum transaminases and sterile pyuria. Twelve of the patients (15.7%) developed coronary artery aneurysms, two patients had a mild mitral insufficiency and one patient with a mild pericardial effusion. There was one case of cholestatic hepatitis. All the complications were resolved without sequelae.
Male sex (OR = 1.24), an urticarial exanthem (OR = 10.53) and a C-reactive protein > 10 mg/dl (OR = 4.20) were identified as risk factors for coronary aneurysms.
Our patients had the typical clinical and laboratory findings of Kawasaki disease. Mild coronary artery complications were observed in 15.7% of the patients. Male sex, an urticarial exanthem and an elevated C-reactive protein are risk factors for coronary aneurysms.

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^*: Supported in part by The Kawasaki Research Fund of The Children's Memorial Hospital, Chicago, and by Grants HL 34545 and AM 3069203 from the National Institutes of Health.

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Original articleIncomplete Kawasaki disease with coronary artery involvement*

J Pediatr

Am J Cardiol

J Pediatr

Am J Cardiol

Am J Cardiol

J Pediatr

Am J Cardiol

J Pediatr

J Pediatr

J Pediatr

Kawasaki disease—New York

MMWR

Coronary artery aneurysms presenting as sudden death 14 years after Kawasaki disease in infancy

Arch Pathol Lab Med

Mutliple coronary-artery aneurysms in a child with angina pectoris

N Engl J Med

Kawasaki disease: effect of treatment on coronary artery involvement

Pediatrics

Probable efficacy of high-dose salicylates in reducing coronary involvement in Kawasaki disease

JAMA

Original article
Incomplete Kawasaki disease with coronary artery involvement*