Sacco O, Fregonese B, Oddone M, Verna A, Tassara E, Mereu C, Rossi G A
Pulmonary Division, G. Gaslini Institute, Genoa, Italy.
Eur Respir J. 1997 Feb;10(2):494-6. doi: 10.1183/09031936.97.10020494.
Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications. An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. Bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient.
复发性多软骨炎(RP)是一种病因不明的罕见疾病,其特征是包括气管支气管树在内的许多器官的软骨结构发生炎症和破坏。若不进行治疗,死亡率很高,通常死于呼吸阻塞性并发症。一名8岁白人女孩,之前被诊断为RP,因呼吸困难加重被转诊至我科。支气管镜检查显示黏膜有局部炎症和纤维化改变,导致左主支气管起始处严重阻塞。该病情通过内镜钕钇铝石榴石(Nd YAG)激光成功治疗。7个月后对患者进行重新评估,结果显示存在支气管狭窄和软化,需要进行机械扩张并放置支气管内硅支架,患者对该支架耐受性良好。