A Case of Relapsing Polychondritis With Severe Obstructive Ventilatory Defect Caused by a Single Flare‐Up

Relapsing polychondritis (RP) is a rare progressive autoimmune disorder affecting cartilaginous and/or proteoglycan-rich structures. Tracheobronchial involvement is observed in almost half of RP cases and significantly impacts the prognosis. We present a case of RP with a rapidly progressive obstructive ventilatory defect caused by a single flare-up, despite significant improvements in the thickening of the airway walls after starting steroid administration. Although the airway walls appeared normal on computed tomography after starting treatment, tracheobronchial cartilage tissues were considered to have been extensively destroyed by chondritis, leading to tracheobronchomalacia and resulting in dynamic respiratory collapse. Preventing fibrosis of the airway walls caused by recurrent flare-ups will be extremely important to avoid fixed airway stenosis.

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Publication Details

Journal
Respirology Case Reports
Published
2025-11-01
DOI
https://doi.org/10.1002/rcr2.70415
Primary Topic
Otitis Media and Relapsing Polychondritis
Type
article
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article

A Case of Relapsing Polychondritis With Severe Obstructive Ventilatory Defect Caused by a Single Flare‐Up

Ryusei Nakagawa, Hiroko Watanabe, Akira Kishimoto, Yuichi Dai et al.
Respirology Case Reports
Otitis Media and Relapsing Polychondritis
article

A Case of Relapsing Polychondritis With Severe Obstructive Ventilatory Defect Caused by a Single Flare‐Up

Ryusei Nakagawa, Hiroko Watanabe, Akira Kishimoto, Yuichi Dai, Tomohiro Namiki, Hiroaki Ishikawa, Tohru Sakamoto, Toshihide Inui, Hiroya Sunabe
article en

Abstract

Relapsing polychondritis (RP) is a rare progressive autoimmune disorder affecting cartilaginous and/or proteoglycan-rich structures. Tracheobronchial involvement is observed in almost half of RP cases and significantly impacts the prognosis. We present a case of RP with a rapidly progressive obstructive ventilatory defect caused by a single flare-up, despite significant improvements in the thickening of the airway walls after starting steroid administration. Although the airway walls appeared normal on computed tomography after starting treatment, tracheobronchial cartilage tissues were considered to have been extensively destroyed by chondritis, leading to tracheobronchomalacia and resulting in dynamic respiratory collapse. Preventing fibrosis of the airway walls caused by recurrent flare-ups will be extremely important to avoid fixed airway stenosis.

Respirology Case ReportsVol. 13(11)
Tsukuba Memorial Hospital (JP)
Openalex Percentile: Top 50%
Otitis Media and Relapsing Polychondritis
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A Case of Relapsing Polychondritis With Severe Obstructive Ventilatory Defect Caused by a Single Flare‐Up — Ryusei Nakagawa, Hiroko Watanabe, et al. · Respirology Case Reports (2025) | TGRS Research Map | TGRS