Division of Respiratory Medicine, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, 216-8511, Japan.
BMC Pulm Med. 2023 Apr 3;23(1):108. doi: 10.1186/s12890-023-02400-z.
Relapsing polychondritis (RP) is a chronic and recurrent inflammatory disease of the cartilage tissues in the body. The cause of RP is unknown, and since it is a rare disease with symptoms that affect multiple organs, diagnosis is often delayed.
A 62-year-old woman with no smoking history visited our institution complaining of fever, cough, and dyspnoea. Chest CT showed a stenosis from the left main bronchus to the left lower lobe branch. Bronchoscopy visualised intense erythema and oedema at the left main bronchus, with airway narrowing. Biopsy of the ear revealed degenerative vitreous cartilage and fibrous connective tissue with a mild inflammatory cell infiltrate. She was subsequently diagnosed with RP and administered systemic corticosteroid therapy. Her symptoms improved rapidly, and post-treatment bronchoscopy revealed that although mild erythema of the airway epithelium remained, oedema markedly improved, and the airway stenosis was resolved.
We report a case where pre-treatment bronchoscopy was able to visually confirm RP at the acute stage. Since RP is difficult to diagnose, severe airway narrowing can occur prior to diagnosis. Therefore, to determine the stage of the disease, it is helpful to perform bronchoscopic observation before treatment. However, bronchoscopic observation before treatment should be performed by experienced bronchoscopists due to the risk of airway obstruction.
复发性多软骨炎（RP）是一种累及全身软骨组织的慢性、复发性炎症性疾病。RP 的病因不明，由于该病症状涉及多个器官且较为罕见，因此诊断常常被延误。
一名 62 岁女性，无吸烟史，因发热、咳嗽和呼吸困难就诊于我院。胸部 CT 显示左主支气管至左下叶支气管狭窄。支气管镜检查发现左主支气管有强烈的红斑和水肿，气道狭窄。耳部活检显示退行性玻璃体软骨和纤维结缔组织，伴有轻度炎症细胞浸润。随后诊断为 RP，并给予全身皮质类固醇治疗。她的症状迅速改善，治疗后支气管镜检查显示尽管气道上皮仍有轻度红斑，但水肿明显改善，气道狭窄得到缓解。
我们报告了一例在急性发作期行支气管镜检查可明确诊断为 RP 的病例。由于 RP 诊断困难，在诊断前可能会出现严重的气道狭窄。因此，为了确定疾病的阶段，在治疗前进行支气管镜观察有助于诊断。然而，由于存在气道阻塞的风险，在治疗前进行支气管镜观察应由经验丰富的支气管镜医生进行。
