Case Presentation: A 4-month-old, Filipina female presented with 5 days of fever, irritability, and left jaw and neck swelling. Physical exam revealed bilateral conjunctivitis, blanching migratory macular erythema of the forehead and abdomen, and edema with erythema of the left neck that also obscured the angle of the left jaw. Ultrasound showed enlarged left parotid gland and left cervical adenopathy. Laboratory findings on admission were significant for pyuria (WBC 38/μL), thrombocytosis (539 TH/ μL), microcytic anemia (Hb 9.5 g/dL, MCV 61 fL), normal WBC count, CRP 5.4 mg/dL, ESR 63 mm, BNP 216 pg/mL. Patient was initially diagnosed with viral parotitis and viral sepsis and started on IV ampicillin-sulbactam for empiric treatment of possible bacterial parotitis. Physical exam on day 2 of admission revealed conjunctival injection with perilimbic sparing, cardiac gallop, slight erythema of hands and feet, and diffuse rash over the back, abdomen, and groin. Concern for Node-First Kawasaki Disease (KD) was raised based on findings of conjunctivitis with perilimbic sparing, sterile pyuria, thrombocytosis, elevated BNP and inflammatory markers, persistent fevers and rash despite antibiotics, and patient’s Asian ancestry. Initial echocardiogram demonstrated normal arteries, but low-normal ejection fraction of 58%. Antibiotics were discontinued and patient was administered IVIG and high-dose aspirin. Echocardiogram was repeated 48 hours later, which showed improved ejection fraction of 62%, but enlarged left anterior descending artery (Z-score 2.4). In response to this and persistent fever, she was administered Infliximab with subsequent resolution of fever. 48 hours later, echocardiogram showed improvement of coronary artery size. Patient was discharged on day 7 after >24 hours without fever. 4 months after discharge, the echocardiogram, ECG, and laboratory values had all normalized.
Discussion: KD is a systemic vasculitis of unknown etiology with complications of arrhythmias, myocarditis, and coronary artery aneurysms in ~25% of untreated cases (1). Early diagnosis and treatment of KD significantly reduces the risk of acquired heart disease (2). However, diagnosis of KD can be challenging given it is based on a constellation of clinical findings that may be presented incompletely or atypically, particularly for infants under 6 months old of age (3). Parotitis as an initial presentation of KD is unusual but has been documented in several case reports (4). Our patient had several features consistent with parotitis of viral etiology including parotid gland swelling, conjunctivitis, and rash; however, these findings in conjunction with persistent fever and rash, and laboratory results such as thrombocytosis, sterile pyuria, and elevated BNP raised concern for KD.
Conclusions: KD presentation is often atypical in infants less than 6 months old. Apparent parotitis that does not respond as expected to antibiotics, and especially when accompanied by the development of unusual features, should trigger investigation into possible Kawasaki Disease.