- 期刊
Relapsing Polychondritis Complicated with Nasal Septal Chondritis and Tracheobronchial Stenosis-Case Report and Literature Review
復發性多發性軟骨炎合併鼻中膈軟骨炎與氣管－支氣管狹窄之併發症－病例報告及文獻回顧
摘要
Relapsing polychondritis is a rare autoimmune disease of the cartilage characterized by recurrent inflammation and destruction of cartilaginous tissue. The common manifestations are chondritis of the ear, nose, trachea, and joints. This inflammatory condition is progressive and deteriorates if left untreated. As these manifestations are not usually present concurrently, the disease is subjected to misdiagnosis or delayed diagnosis. We describe a 25-year-old woman who initially had a fever of unknown origin and refractory nasal obstruction but was eventually diagnosed with relapsing polychondritis owing to bilateral auricular inflammation. Immunotherapy was administered accordingly. The disease continued to progress to manifestations of airway obstruction and tracheal stenosis. Severe narrowing of the trachea and collapse of the left bronchus were noted, and the pulmonary function tests revealed a fixed obstructive pattern. After pulse immunosuppressive therapy, respiratory symptoms were controlled, although respiratory function remained impaired. We believe that early notification of this disease may assist in early diagnosis, and prevention of severe airway complications.
並列摘要
復發性多發性軟骨炎為一種罕見的軟骨自體免疫疾病，臨床特徵為軟骨組織反覆發炎而破壞。最常見的表徵為鼻、耳、氣管、關節的軟骨炎。若是不接受治療，發炎狀態會趨漸惡化。由於復發性多發性軟骨炎的臨床表現大多不會同時發生，因此容易誤診或延遲診斷。本文中我們描述一位已持續1年的不明發燒、難治性鼻塞為表現的25歲女性，最終因雙側耳發炎而診斷為復發性多發性軟骨炎，接受免疫治療。疾病持續惡化表現出呼吸道阻塞、氣管狹窄。檢查發現嚴重氣管狹窄與左側支氣管塌陷且肺功能檢查呈現固定阻塞型。給予高劑量免疫抑制劑治療後，呼吸道症狀獲得控制，然而呼吸功能仍維持病損。我們認為及早獲知疾病可幫助早期診斷並因而避免發生嚴重的呼吸道併發症。