Division of Respiratory Medicine, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki City, Kanagawa, Japan.
Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki City, Kanagawa, Japan.
Sci Rep. 2023 May 23;13(1):8307. doi: 10.1038/s41598-023-35616-4.
Airway involvement in relapsing polychondritis (RP) can be debilitating and life threatening, often requiring interventional procedures. If standard therapies including systemic corticosteroid and immunosuppressive agents are ineffective, airway stenting is often required. Recently, biologics have been reported to be effective for RP, and the early administration of biologics may avoid airway stenting. To evaluate survival rates and treatment approaches, medical records of RP patients with airway involvement were reviewed. These cases were divided into the following groups: with and without malacia, stenting and non-stenting, and with and without biologics. Kaplan-Meier was used to calculate survival rates and log rank tests were used to analyze biologics groups. A total of 77 patients were enrolled. Airway stenting was performed in 13 patients, all of which developed airway malacia. The stenting group had significantly lower survival rates than the non-stenting group (p < 0.001). Stent-related complications were granulation tissue (85%) and mucostasis (69%). In the non-stenting group, a lower mortality rate was observed. A significantly higher survival rate was seen in patients administered biologics than without (p = 0.014). The early administration of biologics shows promise in preventing severe airway disorders that require airway stenting.
气道受累在复发性多软骨炎(RP)中可能会导致身体虚弱和生命威胁,通常需要介入治疗。如果标准治疗方法(包括全身性皮质类固醇和免疫抑制剂)无效,通常需要气道支架。最近,生物制剂已被报道对 RP 有效,早期使用生物制剂可能避免气道支架。为了评估生存率和治疗方法,回顾了气道受累的 RP 患者的病历。这些病例分为以下几组:有和无软骨软化症、支架和非支架以及有和无生物制剂。采用 Kaplan-Meier 计算生存率,采用对数秩检验分析生物制剂组。共纳入 77 例患者。13 例患者进行了气道支架治疗,所有患者均发生气道软骨软化症。支架组的生存率明显低于非支架组(p<0.001)。支架相关并发症为肉芽组织(85%)和黏液阻塞(69%)。在非支架组中,死亡率较低。与未使用生物制剂的患者相比,使用生物制剂的患者生存率显著提高(p=0.014)。早期使用生物制剂有望预防需要气道支架的严重气道疾病。