Guo Shaobo, Li Longzhao, Wang Lu
School of Nursing, Beijing University of Traditional Chinese Medicine, Beijing, China.
Respiratory Disease Center, Dongzhimen Hospital, Beijing University of Chinese Medicine, Beijing, China.
Medicine (Baltimore). 2025 Jun 20;104(25):e42987. doi: 10.1097/MD.0000000000042987.
Relapsing polychondritis (RP) is a rare inflammatory disease that presents clinically with severe dyspnea and respiratory distress. Differentiating it from amyloidosis can be diagnostically challenging. In cases of severe respiratory distress, timely bronchoscopic intervention, including laser tracheobronchoplasty and glucocorticoids (GCs) spraying, is crucial. Efficacy of this combined approach has not been reported to treat onset of RP.
We report a complex case of RP in a 56-year-old male. Over the past 2 years, the patient experienced recurrent episodes of dyspnea, cough, and expectoration. Chest computed tomography (CT) scans indicated tracheal wall thickening, and airway stenosis and deformation. Recently, the patient presented with severe dyspnea and respiratory distress, leading to a definitive diagnosis of RP.
The diagnosis of RP was established based on the patient's medical history, CT scans, clinical signs (noting cartilage collapse and swelling in the ears, nasal collapse), and endoscopic findings (severe airway stenosis and collapse).
The patient was treated with noninvasive ventilation, budesonide, GCs for anti-inflammatory effects, and moxifloxacin for anti-infective therapy. Due to worsening conditions, invasive ventilation was used for 4 days. An emergency bronchoscopic examination was performed, followed by sputum aspiration, laser tracheobronchoplasty for airway reshaping, and endotracheal intubation to stabilize oxygen saturation and alleviate symptoms. Cyclophosphamide was administered.
The patient experienced significant relief from dyspnea, and no recurrence was observed within 1 month after the completion of treatment.
When RP is exacerbated by infection, leading to progressive dyspnea and causing acute respiratory distress, it is difficult to distinguish based on CT scans. In situations where pathological results are not promptly available, endoscopic diagnosis and intervention are merited, we recommend performing laser tracheobronchoplasty early during bronchoscopy and spraying GCs to reduce mucosal edema.
复发性多软骨炎（RP）是一种罕见的炎症性疾病，临床上表现为严重的呼吸困难和呼吸窘迫。将其与淀粉样变性进行鉴别诊断具有挑战性。在严重呼吸窘迫的病例中，及时进行支气管镜干预，包括激光气管支气管成形术和糖皮质激素（GCs）喷雾，至关重要。尚未有关于这种联合方法治疗RP发作疗效的报道。
我们报告了一例56岁男性的复杂RP病例。在过去2年中，该患者反复出现呼吸困难、咳嗽和咳痰。胸部计算机断层扫描（CT）显示气管壁增厚、气道狭窄和变形。最近，患者出现严重呼吸困难和呼吸窘迫，最终确诊为RP。
根据患者的病史、CT扫描、临床体征（注意到耳部软骨塌陷和肿胀、鼻塌陷）以及内镜检查结果（严重气道狭窄和塌陷）确诊为RP。
患者接受了无创通气、布地奈德、具有抗炎作用的GCs以及抗感染治疗用的莫西沙星。由于病情恶化，进行了4天的有创通气。进行了紧急支气管镜检查，随后进行痰液抽吸、激光气管支气管成形术以重塑气道，并进行气管插管以稳定氧饱和度并缓解症状。给予了环磷酰胺。
患者呼吸困难明显缓解，治疗完成后1个月内未观察到复发。
当RP因感染而加重，导致进行性呼吸困难并引起急性呼吸窘迫时，基于CT扫描很难区分。在无法及时获得病理结果的情况下，值得进行内镜诊断和干预，我们建议在支气管镜检查早期进行激光气管支气管成形术并喷雾GCs以减轻黏膜水肿。
