Acute Respiratory Failure Derived From Subglottic Stenosis in a Patient with Relapsing Polychondritis

성문하 협착에 의해 급성 호흡부전이 발생된 재발성 다발성 연골염 1예

Relapsing polychondritis (RP) is rare, chronic, relapsing, and multisystemic inflammatory disease targeting the cartilaginous structures. Respiratory track involvement occurs in approximately half of the cases. Subglottic stenosis is a rare manifestation of RP. Here, we report a case of RP with a subglottic stenosis, resulting in acute respiratory failure. A 63-year old man was admitted complaining of multiple joint pain, general weakness, weight loss, throat pain, hoarseness, exertional dyspnea, and hearing difficulties. A laryngoscopy and neck CT revealed a subglottic stenosis. Four days after admission, he complained severe dyspnea resulting in acute respiratory failure. Immediately, a tracheostomy was done for airway preservation. After high dose steroid therapy, the general symptoms were improved. However, the subglottic stenosis was sustained. Thus, a laryngotracheal augumentation and stent insertion was performed. The speech valve was then replaced. The subglottic stenosis was managed with low dose steroid and monthly cyclophosphamide pulse therapy, and the patient has been followed up regularly.

저자들은 이개염이 동반되지 않고 안장코와 다발성 관절염, 청력 감소, 기관지 침범 등이 동반된 재발성 다발성 연골염 환자에서 성문하 연부조직의 심한 부종으로 상기도 폐쇄가 동반되어 급성 호흡부전이 발생된 환자 1예를 경험하고 문헌고찰과 함께 보고하는 바이다.