Academic Commons

Presentations (Communicative Events)

Recurrent refractory Kawasaki disease

Costa Reis, P.; Nativ, S.; Starr, Amy Joan; Imundo, Lisa F.; Eichenfield, Andrew Howard

Background: Kawasaki disease is a common childhood vasculitis. Unrelenting fever after treatment with intravenous immunoglobulin (IVIG) occurs in 10-15% of patients and is associated with a greater risk of developing coronary aneurysms. Aim: Describe a very unique case of recurrent and refractory Kawasaki disease. Case report: A 3 year old boy presented with 3 days of fever, rash, pharyngeal and gingival erythema, and swollen extremities. Laboratory investigations revealed leukocytosis, C–reactive protein 25.8 mg/dl, and erythrocyte sedimentation rate 100 mm/hr. Echocardiography disclosed diffuse dilatation of all proximal coronary arteries. The child received IVIG (2g/kg) and aspirin (100 mg/kg/d) with no response. IVIG was repeated, followed by methylprednisolone 30 mg/kg for 3 days, but the child remained febrile. Infliximab (5 mg/kg) was thereupon employed with prompt defervescence. Low-dose aspirin was continued, as well as clopidogrel. Echocardiographic findings remained stable. Six months after the initial episode, the child again presented with fever, irritability, sore throat and nuchal rigidity. Physical examination revealed cracked, swollen lips, oropharyngeal erythema, posterior cervical lymphadenopathy, and rash. Desquamation of the distal extremities was observed some days later. Aneurysms were detected, involving the left and right main coronary arteries, as well as the left anterior descending coronary. Magnetic resonance angiography of the chest and abdomen revealed no other involved vessels. The child again received IVIG, pulse methylprednisolone, and infliximab, but remained febrile and developed significant arthritis, requiring daily prednisolone. He is now asymptomatic. Conclusions: Currently, recurrent and refractory Kawasaki disease still represents a therapeutic challenge.

Files

  • thumnail for 1546-0096-9-S1-P98.xml 1546-0096-9-S1-P98.xml binary/octet-stream 4.34 KB Download File
  • thumnail for 1546-0096-9-S1-P98.pdf 1546-0096-9-S1-P98.pdf binary/octet-stream 187 KB Download File

Also Published In

Title
Pediatric Rheumatology
DOI
https://doi.org/10.1186/1546-0096-9-S1-P98

More About This Work

Academic Units
Pediatrics
Medicine
Publisher
BioMed Central
Published Here
September 9, 2014
Academic Commons provides global access to research and scholarship produced at Columbia University, Barnard College, Teachers College, Union Theological Seminary and Jewish Theological Seminary. Academic Commons is managed by the Columbia University Libraries.