Kawasaki disease (KD), is an acute, self-limiting vasculitis of unknown aetiology. It was first described by Tomisaku Kawasaki in 1967 as mucocutaneous lymph node syndrome.  Kawasaki disease has now become the leading cause of acquired heart disease in children in North America and Japan and is increasingly recognized as a worldwide problem [1]. It occurs in young children, with a yearly incidence of 80 to 90 per 100,000 children younger than 5 years in Japan and 10 per 100,000 children younger than 5 years in the United States 2 The proportion of KD patients younger than 6 months of age in relation to all KD patients is approximately 10%3, KD in India (as also in other developing countries) is probably being misdiagnosed as having viral exanthemata (especially measles) and other febrile illnesses [2]. The diagnosis of KD is based on clinical criteria and exclusion of other disease with similar manifestation. In India, extrapolation of hospital based data from Chandigarh between January 2009 to December 2014 has shown upward trends in incidence of KD (1.0 in 2012 to 9.1/100,000 in 2009.) peak incidence is below 5yrs of age [3].

KD is medium sized artery vasculitis and is the predominant cause of acquired heart disease with predilection for coronary arteries leading to coronary aneurysm in 25% of untreated KD patients.Diagnosis of KD is made on clinical parameter in absence of specific lab tests. American Heart Association (AHA) 2004 laid down the guidelines for diagnosis of KD which were updated in 2017[4-5].

Classic KD is diagnosed with fever persisting for least 5 days along with

At least four of the five principal clinical features:

Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosa.

Changes in Extremities

Acute:  Erythema of palms, soles; edema of hands and feet.

Subacute

Periungual desquamation of fingers and toes in weeks 2 and 3, after onset of fever and may extend to involve the palms and soles.

Chronic

Deep transverse grooves across the nails (Beau’s lines) seen 1-2 months later.

Rash

Polymorphous exanthema (diffuse maculopapular, urticarial, erythroderma, erythema-multiforme like, not vesicular or bullous), primarily involving the trunk and extremities.

Conjuctivitis

Bilateral bulbar conjunctival nonexudative injection, sparing the limbus.

Cervical lymphadenopathy is the least common of the principal features. Lymph node enlargement (> 1.5 cm diameter), usually unilateral and confined to the anterior cervical triangle.

A careful history may reveal that ≥ 1 principal clinical features were present during the illness but resolved by the time of presentation

Exclusion of other diseases with similar findings (e.g., scarlet fever, viral infections like measles, adenovirus, enterovirus, Stevens-Johnson syndrome, toxic shock syndrome, drug hypersensitivity reactions, systemic juvenile idiopathic arthritis).

According to AHA (2017) guidelines; KD is one of the differential in infants if [5]:  Infants < 6 months old have prolonged fever have (1) irritability; (2) unexplained aseptic meningitis; (3) unexplained or culture-negative shock; (4) cervical lymphadenitis unresponsive to antibiotic therapy; (5)retropharyngeal or parapharyngeal phlegmon unresponsive to antibiotic therapy.

A high degree of suspicion in case of prolonged fever is needed to make a diagnosis of KD,

We would like to present a case of KD in an infant where the disease manifestation appeared as early as 59 days after birth. Our experience highlights the need to consider KD even in an infant with a case of prolonged fever.

Case Report

A previously healthy, 59 days old male infant, developed high grade fever, which was followed 4 days later by reddish discoloration of lips and conjunctival congestion, followed 2 days later by maculopapular rash on trunk and extremities. He was admitted to another hospital with above complaints Initial possibility of sepsis was kept and treated with antibiotics.  As child didn’t show any improvement of clinical symptoms and signs,and, lab parameters indicated thrombocytosis, raised acute phase reactants, a diagnosis of Kawasaki Disease was made as infant fulfilled the criteria for KD laid down by AHA (2017). Infant was administered immunoglobulin and his complaints became passive. Infant was referred to us on low dose acetylsalicylic acid for further management and cardiologist evaluation. Infant reported to our hospital on 88 day of life and 29 day of illness. Child was asymptomatic at presentation, but lab parameters showed persistant leucocytosis (25200/ml), thrombocytosis (918000/ml), anaemia (Hb: 9.3 gram/dl), raised ESR (100 m1m at 1^st hour) and raised CRP (167 mg/L).

Echocardiography revealed mild pericardial effusion with ectasia of right coronary artery/ LMCA and proximal LAD of 6mm with normal left and right ventricular global systolic function. In view of large coronary artery aneurysm with high risk of thrombotic complications, he was started on subcutaneous injections of Enoxaparin and oral Prednisone. 

In view of persistently raised acute phase reactants and follow up echocardiography after 9 days showed presence of hyperechoeic lesion in RCA suggestive of suspected thrombus and aneurysm of size 6.4mm, he was administered  infliximab infusion by intravenous route ( TNF-alpha inhibitor).

Patient was discharged from hospital with low dose acetylsalicylic acid, enoxaparin and prednisone and is on follow up.

Kawasaki disease primarily affects  young children,  80% patients are under the age of 4 years with its peak incidence is at 9 to 11 months of age. KD is extremely uncommon in young infants below 3 months.[6] Tsuchida et al in a Japanese database study, reported only 1.7% of all KD patients series in the age group younger than 3 months [6]. The youngest reported patient in the literature is a 12 days old neonate [8].^.  In infants,  atypical presentations (longer duration of illness before diagnosis, less common incidence of conjunctivitis, rash and extremity change, and lower value of C-reactive protein) are common, and this may result in a delay in diagnosis and effective treatment [9-10].  KD is quite rare under the age of 3 months, but vasculitis is severe and coronary artery involvement is more common, rapid and  severe [6,9-10] as was also seen in our case.

Delay in the diagnosis, contributed to the higher incidence of coronary aneurysms.

Diagnosis is based on clinical criteria, we used AHA criteria for the diagnosis. 

The current guidelines recommend that infants <6months of age, with fever for longer than 7days should be investigated for a systemic vascular response(i.e erythrocyte sedimentation rate and CRP levels.

KD can affect any age group, but, is quite rare in young infants and manifests atypically but with more severe vasculitis and coronary artery abnormalities. High index of suspicion and awareness among clinician is the key to early diagnosis, management and prevention of the complications in any child with fever of unknown origin.

1. Newburger J.W. et al. “Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.” Pediatrics, vol. 114, no. 6, 2004, pp. 1708–1733.

2. American Heart Association. “Diagnostic guidelines for Kawasaki disease.” American Journal of Diseases of Children, vol. 144, no. 11, 1990, pp. 1218–1219.

3. Son M.B.F. and J.W. Newburger. “Kawasaki disease.” Pediatrics in Review, vol. 39, no. 2, 2018, pp. 78–90.

4. Newburger J.W. et al. “Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.” Circulation, vol. 110, no. 17, 2004, pp. 2747–2771.

5. McCrindle B.W. et al. “Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association.” Circulation, vol. 135, no. 17, 2017, pp. e927–e999.

6. Rowley A.H. “Kawasaki syndrome.” Krugman’s Infectious Diseases of Children. 11th ed., edited by A.A. Gershon, P.J. Hotez and S.L. Katz, Mosby Inc., 2004, pp. 323–335.

7. Tsuchida S. et al. “Epidemiology of infant Kawasaki disease with a report on the youngest neonatal case reported in Japan.” Acta Paediatrica, vol. 85, no. 8, 1996, pp. 995–997.

8. Brenner J.L. et al. “Severe Kawasaki disease in infants: two fatal cases.” Canadian Journal of Cardiology, vol. 16, no. 8, 2000, pp. 1017–1023.

9. Stanley T.V. and K. Grimwood. “Classical Kawasaki disease in a neonate.” Archives of Disease in Childhood Fetal and Neonatal Edition, vol. 86, no. 1, 2002, pp. F35–F36.

10. Zsadanyi J. et al. “Fatal outcome of Kawasaki syndrome in a 4-week-old infant.” Acta Paediatrica, vol. 85, no. 8, 1996, pp. 995–997.

11. Rowley A.H. “Kawasaki disease: novel insights into etiology and genetic susceptibility.” Annual Review of Medicine, vol. 62, no. 1, 2011, pp. 69–77.