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

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 cobblestone 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.

Posterior Reversible Encephalopathy Syndrome: A Review of the Literature

“…Among the risk factors, immune system activation/disruption has recently been considered a critical initial step in the pathogenesis of PRES and plays a pivotal role in the development of the disease ( 17 , 18 ). Immunosuppressant or cytotoxic drugs, such as cyclosporin A, tacrolimus/FK-506, methotrexate, sirolimus, lenvatinib, bevacizumab, carboplatin, and paclitaxel, are also significant predisposing factors ( 5 , 12 , 19 - 22 ).…”