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.

journal_name

Joint Bone Spine

journal_title

Joint bone spine

authors

Soltner E,Neel A,Tiab M,Varin S,Cormier G,Maisonneuve H,Maugars Y,Tanguy G,Hamidou M,Berthelot JM

doi

10.1016/j.jbspin.2009.01.006

subject

Has Abstract

pub_date

2009-10-01 00:00:00

pages

559-61

issue

5

eissn

1297-319X

issn

1778-7254

pii

S1297-319X(09)00048-7

journal_volume

76

pub_type

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