Relapsing Polychondritis with a Cobble-stone Appearance of the Tracheal Mucosa, Preceded by Posterior Reversible Encephalopathy Syndrome

Takahide Ikeda; Motochika Asano; Yoshihiko Kitada; Kouichiro Taguchi; Yuichi Hayashi; Kazuo Kajita; Hiroyuki Morita

doi:10.2169/internalmedicine.4095-19

This article has now been updated. Please use the final version.

Relapsing Polychondritis with a Cobble-stone Appearance of the Tracheal Mucosa, Preceded by Posterior Reversible Encephalopathy Syndrome

Takahide Ikeda, Motochika Asano, Yoshihiko Kitada, Kouichiro Taguchi, Yuichi Hayashi, Kazuo Kajita, Hiroyuki Morita

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Keywords: relapsing polychondritis, posterior reversible encephalopathy syndrome, tracheal stenosis

JOURNAL OPEN ACCESS Advance online publication

Article ID: 4095-19

DOI https://doi.org/10.2169/internalmedicine.4095-19

The final version of this article is now available: Vol. 59 (2020), No. 8 pp. 1093-1097

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Abstract

A 25-year-old woman had convulsions and disturbance of consciousness. Head magnetic resonance imaging (MRI) showed punctate areas in the occipital lobes with increased signals on T2-weighted imaging. The MRI abnormalities responded well to steroid pulse therapy, so we made a diagnosis of posterior reversible encephalopathy syndrome (PRES). Three months later, she developed a fever and dyspnea. Chest computed tomography revealed marked thickness of the tracheal and bronchial wall, and bronchoscopy showed a cobble-stone appearance of the tracheal mucosa, indicative of relapsing polychondritis (RPC). We consider that PRES had developed due to autoimmune vasculitis in the brain with RPC.

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