title: Incomplete and atypical Kawasaki disease in a young infant: severe, recalcitrant disease responsive to infliximab.
abstract: This report describes a 7-week-old infant with incomplete and atypical Kawasaki disease, an acute vasculitis that predominantly affects infants and children. The patient was refractory to 2 doses of intravenous immunoglobulin and to high-dose intravenous methylprednisolone. He became afebrile only after 2 doses of infliximab. His prolonged, recalcitrant course was complicated by the development of peripheral gangrene and giant coronary artery aneurysms. Infants with incomplete and atypical Kawasaki disease are prone to intravenous immunoglobulin treatment failure and are at risk for the development of coronary artery aneurysms. In such patients, we suggest that consideration be given to early aggressive therapy with corticosteroids or infliximab added to intravenous immunoglobulin.
authors.affiliation: Department of Pediatrics, University of Virginia Health System, Charlottesville, Virginia 22908, USA.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: O'connor
authors.forename: Frank T
authors.initials: FT
authors.lastname: Saulsbury
mesh_terms: D000208:Acute Disease; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D001241:Aspirin / Q000627:therapeutic use; D003323:Coronary Aneurysm / Q000000981:diagnostic imaging; D005734:Gangrene / Q000188:drug therapy; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D000069285:Infliximab; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009080:Mucocutaneous Lymph Node Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy*; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use*; D014463:Ultrasonography
chemical_list: D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D016756:Immunoglobulins, Intravenous; D010975:Platelet Aggregation Inhibitors; D006493:Heparin; D000069285:Infliximab; D001241:Aspirin; D008775:Methylprednisolone