Early Diagnosis of Relapsing Polychondritis With Airway Involvement: A Case Report

Relapsing polychondritis (RP) is a rare systemic immune-mediated disease that causes recurrent inflammation of cartilaginous tissues. Airway involvement is a significant prognostic factor; however, diagnosis is often delayed because of nonspecific symptoms. We report the case of a 38-year-old woman who presented with pharyngeal discomfort, cough, and chest pain. Initial tests revealed elevated inflammatory markers and anemia. A chest CT scan showed soft tissue enhancement around the costal cartilage and thickening of the tracheal and bronchial walls. Bronchoscopy demonstrated extensive inflammation from the larynx to the main bronchi, with approximately 90% narrowing of the left bronchus during exhalation, suggestive of tracheomalacia. Based on clinical findings, imaging, bronchoscopy, and serological tests, a diagnosis of RP was made, fulfilling McAdam's and Damiani's criteria. Although pulmonary function tests continued to show obstructive ventilatory impairment, treatment withcorticosteroids (0.6 mg/kg prednisolone) and methotrexate resulted in rapid improvement in inflammatory markers and imaging findings. This case highlights the importance of early bronchoscopy in assessing airway involvement in RP, even when respiratory symptoms are mild, and emphasizes the need to balance the diagnostic benefits against potential risks, particularly in those with reduced forced vital capacity. Early diagnosis and timely intervention, including consideration of biologics, may prevent progression to severe airway compromise.

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Journal
Cureus
Published
2025-03-24
DOI
https://doi.org/10.7759/cureus.81101
Citations
1
Primary Topic
Otitis Media and Relapsing Polychondritis
Type
article
Field-Weighted Citation Impact
1.67
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article

Early Diagnosis of Relapsing Polychondritis With Airway Involvement: A Case Report

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1 citations
Cureus
Otitis Media and Relapsing Polychondritis
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article

Early Diagnosis of Relapsing Polychondritis With Airway Involvement: A Case Report

Toyoshi Yanagihara, Masaki Fujita, Naoko Himuro, Naoki Hamada, Yuki Shundo, Natsumi Kushima
article en
1 citations

Abstract

Relapsing polychondritis (RP) is a rare systemic immune-mediated disease that causes recurrent inflammation of cartilaginous tissues. Airway involvement is a significant prognostic factor; however, diagnosis is often delayed because of nonspecific symptoms. We report the case of a 38-year-old woman who presented with pharyngeal discomfort, cough, and chest pain. Initial tests revealed elevated inflammatory markers and anemia. A chest CT scan showed soft tissue enhancement around the costal cartilage and thickening of the tracheal and bronchial walls. Bronchoscopy demonstrated extensive inflammation from the larynx to the main bronchi, with approximately 90% narrowing of the left bronchus during exhalation, suggestive of tracheomalacia. Based on clinical findings, imaging, bronchoscopy, and serological tests, a diagnosis of RP was made, fulfilling McAdam's and Damiani's criteria. Although pulmonary function tests continued to show obstructive ventilatory impairment, treatment withcorticosteroids (0.6 mg/kg prednisolone) and methotrexate resulted in rapid improvement in inflammatory markers and imaging findings. This case highlights the importance of early bronchoscopy in assessing airway involvement in RP, even when respiratory symptoms are mild, and emphasizes the need to balance the diagnostic benefits against potential risks, particularly in those with reduced forced vital capacity. Early diagnosis and timely intervention, including consideration of biologics, may prevent progression to severe airway compromise.

CureusVol. 17(3)
Fukuoka University Hospital (JP)
Openalex Percentile: Top 19%
Otitis Media and Relapsing Polychondritis
1.67
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