AB0957 ATYPICAL PRESENTATION AND EARLY RECURRENCE OF KAWASAKI DISEASE IN A FEMALE INFANT: CASE REPORT
Authors: Rodrigo del Toro Rojas, Patricio Javier Flores Lopez, Luis Fernando Sanchez Espino, Cesar Adrian Martinez Longoria, Hector Enrique Valdes Garza
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Kawasaki Disease (KD) is the second most common vasculitis in the pediatric population and the leading cause for pediatric acquired heart disease in developed countries. It is commonly diagnosed in the Mexican pediatric population, epidemiology in this country is not established, since cases are not usually reported to the healthcare system. The clinical features are quite variable, but diagnosis and prompt treatment will decrease morbidity and mortality. Coronary artery aneurysms are the most common complication, which represent the leading cause of acute coronary syndrome before 40 years of age. Recurrence of KD is estimated to be around 3% in Japanese patients and 1% in the United States, nevertheless, this data in Latin-American children is unknown. It usually affects patients before reaching 3 years old and within 2 years of the initial attack, presenting with an abrupt onset and higher complication rates, requiring aggressive workup, treatment and follow-up. <h3>Objectives</h3> To review an atypical presentation of KD and early recurrence in a 7-month-old female. <h3>Methods</h3> We present a 7-month-old female diagnosed with KD at 48 days old. She presented to the emergency unit with irritability, high and persistent fever and acholia. During workup, she was found with cholestasis and gallbladder hydrops. Negative CSF, blood and urine cultures were documented during hospitalization, and fulfilled the KD criteria. The cardiac ultrasound revealed coronary abnormalities: a RCA of 2.8mm (Z-Score +4.79), LCA of 3.3mm (Z-Score +5.69) and LAD of 2.4mm (Z-SCORE +4.25), which fit into classification 3 and 4, as small and medium aneurysms, according to AHA 2017. Immunoglobulin (2g/kg) and aspirin (80mg/kg) were administered and she was discharged 36 hours after the IVIg infusion, afebrile and with ambulatory follow-up, 30 days later the coronary abnormalities showed RCA of 2.0mm (Z-Score +2.3), LCA of 3.0mm (Z-Score +6), LAD 2.3mm (Z-Score +2.9). Six months later, she presented fever for 6 days, irritability and polymorphous rash. On physical exam BCGitis, non-suppurative conjunctivitis and pallor in hands and feet, elevated CRP and ESR, leukocytosis, thrombocytosis and sterile leukocyturia. Echocardiography reported RCA od 2.4mm (Z-Score +4.8), LCA of 3.1 (Z-Score +4.2) and LAD of 2.3mm (Z-Score +3.2), diagnosing KD recurrence, admitting the patient for IVIg and aspirin administration. <h3>Results</h3> The patient was treated with IVIg and aspirin. Follow-up by Cardiology determined improvement of Z-Scores. Recurrence occurred with worsening of the cardiac abnormalities. Cardiac prognosis is importantly affected due to the atypical age, vascular abnormalities and repeated vasculitic process. Rheumatologic consult should be considered since disease like Takayasu Arteritis, Polyarteritis Nosa and ADA2 deficiency need to be ruled out. <h3>Conclusion</h3> KD needs prompt diagnosis and treatment due to the potential consequences when delayed. Clinical suspicion is important due to the possible atypical presentation. As with this patient, age, gender and presentation are not exclusive. Despite adequate treatment, recurrence and worsening of the cardiac abnormalities occurred. Both KD events before 1 year old and with atypical presentations. Rheumatologic and cardiac follow-up need to be stringent through lifetime, to determine pharmacologic treatment, as ell as physical activity and reproductive counseling. <h3>References</h3> [1] McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, Treatment and Long-term Management of Kawasaki Disease: A scientific Statement for Health Professionals From the American Heart Association. Circulation2017;135(17):e927-e99. <h3>Disclosure of Interests</h3> None declared