- 期刊
Relapsing Polychondritis Complicated by Tracheabronchial Stenosis: A Case Report and Literature Review
覆發性多發性軟骨炎(Relapsing Polychondritis)合併氣管－支氣管狹窄之併發症：病例報告及文獻回顧
摘要
覆發性多發性軟骨炎(RP)是一種軟骨的自體免疫性疾病。最常見的表現是鞏膜炎、耳朵和鼻子的軟骨炎。在這種疾病，如果不好好的治療，會發生氣管或支氣管的軟骨炎和罕見的嚴重性氣管狹窄、阻塞的威脅生命的併發症、或突然發生氣管塌陷。因為覆發性多發性軟骨炎的臨床表徵通常不會同時出現，這種疾病容易被主觀的誤診或延遲診斷。我們在這裡報告一位最初以耳朵的軟骨炎和鞏膜炎表現的24歲年輕患者，在接受了6個月不適當的免疫療法後，產生了嚴重氣管和支氣管狹窄。氣道的電腦斷層(CT)研究顯示了在聲門下區域產生了嚴重狹窄（70%狹窄）並且在氣管和支氣管都有中等程度的狹窄。而且受影響的氣管軟骨都腫脹起來。在以高劑量類固醇和免疫抑制藥物(azathioprine)治療1個月後，耳朵的軟骨炎和鞏膜炎都被控制得很好，但氣管狹窄依然沒有變化，而且需要外科開刀來防止氣道堵塞。我們認為如果增加了對這種疾病的了解也許會幫助我們及早診斷和適當的治療；氣管狹窄就會因而被防止產生。
並列摘要
Relapsing polychondritis is an autoimmune disease of the cartilage. The most common manifestations are scleritis, and chondritis of the ear and nose. If the disease is not treated well, inflammation of the cartilage in the trachea or bronchi may occur and develop into a rare life-threatening complication with severe airway narrowing, obstruction, or sudden onset of airway collapse. As the clinical manifestations of relapsing polychondritis do not usually present simultaneously, this disease is easily subjected to misdiagnosis or delayed diagnosis. We herein report a 24-year-old patient who was initially afflicted with chondritis of the ear lobes and scleritis, but was eventually complicated with severe tracheal and bronchial stenosis 6 months after inadequate immunotherapy. Computed tomography studies of the airway showed severe narrowing in the sub-glottic area (70% stenosis) and moderate narrowing in the trachea and bronchi. The affected cartilage of the trachea was also found to be swollen. After being treated with high-dose glucocorticosteroid and immunosuppressant drugs (azathioprine) for 1 month, the ear chondritis and scleritis were well controlled, but the airway stenosis remained unchanged and required surgery to prevent airway obstruction. We think that increased awareness of this disease may help us to diagnose it earlier and treat it more promptly, so as to prevent airway stenosis.