覆發性多重軟骨炎

Relapsing Polychondritis-- a Case report

覆發性多重軟骨炎乃是極為罕見且多見於白種人的自體免疫性病病，至今全世界累積病例約六百例。國內過去無完整病例報告。一位二十五歲男性因為兩側耳朵變形，聲音沙啞以及發燒求診。各種培養皆呈陰性，以支氣管鏡檢查，耳鼻喉科檢查以及喉部電腦斷層證實喉部軟骨變形，入院期間陸續發生肋骨軟骨炎和鞏膜上皮發炎。耳部軟骨切片更佐證覆發性多重軟骨炎的診斷。經過類固醇治療後，體溫恢復正常，聲音沙啞改善且ESR及白血球值亦降至正常範圍。

Relapsing polychondritis is rather rare among all kinds of autoimmune diseases and always reported in Cauchasian population. A Chinese case of pathology-proved relapsing polychondritis was reorted herein, who had manifestations of fever, hoarseness, and bilateral auricular and nasal bridge deformities. This 25-year-old man suffered from intermittent fever and hoarseness for 3 weeks. Bilateral auricular deformities were also noted. Fever work-up including infectious foci, autoimmune profiles and tumor markers were all negative. Elevated ESR and leukocytosis with thrombocytosis were found. Laryngeal tomography and CT revealed laryngeal deformity. Progressive parasternal pain and episcleritis occurred subsequently, and nasal bridge deformity eventually developed. Auricular cartilage biopsy showed acute and chronic chondritis. After corticosteroid treatment, his fever subsided and hoarseness improved with concomitant decline of acute reactants.