title: Chronic, eventually fatal, Kawasaki-like disease in an adult with spondylarthropathy responding to IVIG therapy.
abstract: We report on an unusual case of a 40-year-old Caucasian male displaying severe Kawasaki-like symptoms. The disease lasted for seven years before diffuse coronary aneurysms occurred, leading to the patient's death, despite ongoing treatment by intravenous immunoglobulins (IVIGs). The patient had also been suffering from a disabling inflammation of the spine, which was reported to have started at the onset of the disorder. Whereas neither NSAIDS, nor high doses corticosteroids, or anti-TNF drugs had a clear effect, the clinical features of spinal inflammation were highly sensitive to IVIGs, and were attributed definitively to HLA-B27-negative axial spondylarthropathy after bone scan and magnetic resonance imaging disclosed typical enthesitis of both heels and bilateral sacroiliitis.
authors.affiliation: Rheumatology Unit, CHU de Nantes, 44093 Nantes cedex 01, France.
authors.forename: Elise
authors.initials: E
authors.lastname: Soltner
authors.forename: Antoine
authors.initials: A
authors.lastname: Neel
authors.forename: Mourad
authors.initials: M
authors.lastname: Tiab
authors.forename: Stéphane
authors.initials: S
authors.lastname: Varin
authors.forename: Grégoire
authors.initials: G
authors.lastname: Cormier
authors.forename: Hervé
authors.initials: H
authors.lastname: Maisonneuve
authors.forename: Yves
authors.initials: Y
authors.lastname: Maugars
authors.forename: Gilles
authors.initials: G
authors.lastname: Tanguy
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Berthelot
mesh_terms: D000328:Adult; D000783:Aneurysm / Q000150:complications; D018771:Arthralgia / Q000150:complications; D003327:Coronary Disease / Q000150:complications; D017809:Fatal Outcome; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications*; D010146:Pain / Q000209:etiology; D025241:Spondylarthritis / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology
chemical_list: D016756:Immunoglobulins, Intravenous