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Early Diagnosis of Relapsing Polychondritis With Airway Involvement: A Case Report
Natsumi Kushima1, Toyoshi Yanagihara1, Naoko Himuro2
1Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, JPN.
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.
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