title: A patient with a severe glottic stenosis and saddle nose.
abstract: We present the case of a 39-year-old man with a severe glottis stenosis. The saddle nose, images of laryngotracheal stenosis and the (FDG) positron-emission tomography/computed tomography lead to a final diagnosis of relapsing polychondritis. In the patient a coexistent myelodysplastic syndrome was diagnosed. Moreover, the elevated total IgG4 exceeding 135 ml/dl requested additional immunochemistry for detection of IgG4-bearing plasma cells in the biopsies. The patient underwent an allogenic stem cell transplantation and died on day 40 after the transplantation because of an acute steroid-resistent graft vs host.
authors.affiliation: a Department of Immunology, Allergology, Rheumatology , University of Antwerp and Antwerp University Hospital , Antwerp , Belgium.
authors.forename: Vito
authors.initials: V
authors.lastname: Sabato
authors.affiliation: b ENT, Head and Neck Surgery , University of Antwerp and Antwerp University Hospital , Antwerp , Belgium.
authors.forename: Olivier M
authors.initials: OM
authors.lastname: Vanderveken
authors.affiliation: c Nuclear Medicine , University of Antwerp and Antwerp University Hospital , Antwerp , Belgium.
authors.forename: Tim
authors.initials: T
authors.lastname: Van den Wyngaert
authors.affiliation: b ENT, Head and Neck Surgery , University of Antwerp and Antwerp University Hospital , Antwerp , Belgium.
authors.forename: Carl
authors.initials: C
authors.lastname: Van Laer
authors.affiliation: a Department of Immunology, Allergology, Rheumatology , University of Antwerp and Antwerp University Hospital , Antwerp , Belgium.
authors.forename: Didier
authors.initials: D
authors.lastname: Ebo
mesh_terms: D000328:Adult; D003251:Constriction, Pathologic / Q000209:etiology; D005931:Glottis / Q000473:pathology*; D006801:Humans; D008297:Male; D009666:Nose / Q000473:pathology*; D011081:Polychondritis, Relapsing / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology
keywords: IgG4-related diseases; Saddle nose; glottic stenosis; relapsing polychondritis