title: High dose Anakinra for treatment of severe neonatal Kawasaki disease: a case report.
abstract: We report an 11-week-old female who presented with Kawasaki disease (KD) complicated by macrophage activation syndrome (MAS). The infant presented to the hospital with persistent fever, cough, diarrhea, and emesis, among other symptoms. Her condition quickly began to decompensate, and she developed classic features (conjunctivitis, rash, cracked lips, distal extremity edema) prompting a diagnosis of acute KD. The patient was treated with standard therapy for KD including three doses of intravenous immunoglobulin (IVIG), aspirin, and high dose glucocorticoids with no change in her condition. Due to a high suspicion for MAS, high dose anakinra therapy was initiated resulting in dramatic clinical improvements. She also received one dose of infliximab for concern for coronary artery changes, and over the course of several months, anakinra and high dose glucocorticoids were tapered. Nearly complete reversal of echocardiogram changes were observed after 8 months, and the infant is now off all immunosuppressive therapy. In this case report, we briefly review the importance of early recognition of MAS in pediatric patient populations with rheumatic diseases, and we suggest early initiation of anakinra therapy as a rapid and effective treatment option.
authors.affiliation: University of Alabama School of Medicine, 1720 2nd Avenue South FOT 1203, Birmingham, AL 35294-3412, UK.
authors.forename: Ashley
authors.initials: A
authors.lastname: Shafferman
authors.affiliation: Division of Pediatric Rheumatology, Helen Devos Children's Hospital, 35 Michigan St NE Suite 4150, Grand Rapids, MI 49503, USA.
authors.forename: James D
authors.initials: JD
authors.lastname: Birmingham
authors.affiliation: Division of Pediatric Rheumatology, University of Alabama at Birmingham, 1600 7th Avenue South CPP 210, Birmingham, AL 35233-1711, USA.
authors.forename: Randy Q
authors.initials: RQ
authors.lastname: Cron
mesh_terms: D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D018501:Antirheumatic Agents / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D016903:Drug Monitoring; D004359:Drug Therapy, Combination; D042241:Early Diagnosis; D061345:Early Medical Intervention; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D000069285:Infliximab; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage*; D055501:Macrophage Activation Syndrome / Q000188:drug therapy / Q000209:etiology; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D062072:Patient Acuity; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D018501:Antirheumatic Agents; D005938:Glucocorticoids; D053590:Interleukin 1 Receptor Antagonist Protein; D000069285:Infliximab
keywords: Anakinra; Interleukin-1 receptor antagonist; Kawasaki disease; Macrophage activation syndrome; Neonate; Secondary hemophagocytic lymphohistiocytosis