Kushima Natsumi, Yanagihara Toyoshi, Himuro Naoko, Shundo Yuki, Hamada Naoki, Fujita Masaki
Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, JPN.
Department of Nephrology and Rheumatology, Fukuoka University Hospital, Fukuoka, JPN.
Cureus. 2025 Mar 24;17(3):e81101. doi: 10.7759/cureus.81101. eCollection 2025 Mar.
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.
复发性多软骨炎(RP)是一种罕见的系统性免疫介导疾病,可导致软骨组织反复发炎。气道受累是一个重要的预后因素;然而,由于症状不具特异性,诊断往往会延迟。我们报告了一例38岁女性病例,该患者出现咽部不适、咳嗽和胸痛。初步检查显示炎症标志物升高和贫血。胸部CT扫描显示肋软骨周围软组织强化以及气管和支气管壁增厚。支气管镜检查显示从喉部到主支气管有广泛炎症,呼气时左支气管狭窄约90%,提示气管软化。根据临床表现、影像学检查、支气管镜检查和血清学检查,作出了RP的诊断,符合麦克亚当(McAdam)和达米亚尼(Damiani)标准。尽管肺功能测试持续显示阻塞性通气功能障碍,但使用皮质类固醇(0.6 mg/kg泼尼松龙)和甲氨蝶呤治疗后,炎症标志物和影像学表现迅速改善。该病例强调了早期支气管镜检查在评估RP气道受累方面的重要性,即使呼吸道症状较轻时也如此,并强调需要在诊断益处与潜在风险之间取得平衡,特别是对于那些用力肺活量降低的患者。早期诊断和及时干预,包括考虑使用生物制剂,可能会防止病情进展至严重气道受损。