Relapsing polychondritis with isolated tracheal involvement and airway-only symptoms

Sheng Yuan Wang, Chia Tse Weng, Lili Cheng, Tang Hsiu Huang

Research output: Contribution to journalArticlepeer-review

Abstract

Relapsing polychondritis (RP) is a rare autoimmune disorder, characterized by the inflammation of cartilaginous structures and proteoglycan-rich tissues. Due to its rarity and the notoriously variable presentations, the diagnosis of RP could be challenging. We report an unusual case of RP with isolated tracheal involvement and very non-specific symptoms of exertional dyspnoea and dry cough. The initial chest radiograph showed long-segment narrowing of the trachea, and the computed tomography of the chest revealed thickened cartilaginous walls of the trachea, while the posterior membranous portion was spared. The tracheal narrowing was readily observed under bronchoscopy. The patient was treated with oral prednisolone. Although the subsequent course was transiently complicated by an episode of severe Pneumocystis jirovecii pneumonia with acute respiratory distress syndrome, the patient overall responded well to systemic corticosteroid therapy. No new symptoms developed during a two-year follow-up.

Original languageEnglish
Article numbere00651
JournalRespirology Case Reports
Volume8
Issue number7
DOIs
Publication statusPublished - 2020 Oct 1

All Science Journal Classification (ASJC) codes

  • Pulmonary and Respiratory Medicine

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