復發性多軟骨炎

Relapsing Polychondritis

蘇煌仁(Huang-Jen Su) ； 杜宗陽(Tzong-Yang Tu) ； 廖文輝(Wen-Huei Liao) ； 蕭安穗(An-Suey Shiao)

《中華民國耳鼻喉科醫學雜誌》 34卷2期 (1999/03) Pp. 130-136

https://doi.org/10.6286/1999.34.2.130

摘要

背景：復發性多軟骨炎為臨床上少見的疾病，原因不明。特性包括耳、鼻、氣管、及關節的軟骨結構反覆發炎。最常見的是耳郭軟骨炎，病人因為耳郭突發性的紅腫熱痛求助耳鼻喉科醫師，通常誤認為是耳郭軟骨膜炎而給予抗生素治療合併切開引流，延誤病情。
方法：台北榮民總醫院自1986年1月至1998年3月間，根據Damiani及Levine於1979年修訂McAdam等診斷復發性多軟骨炎的準則，共蒐集了8例，經病例回溯性研究，依其年齡、性別、臨床表徵、實驗室與放射線等特殊檢查、治療方法及結果，加以分析討論。
結果：男性病患2例，女性病患6例，平均年齡51歲（30-71歲），追溯期自2個月至12年不等，其中有2例超過5年。除1例追蹤2個月後失去聯絡外，其餘7例中有3例死亡。侵犯部份以耳郭最常見，8例均有，其次是眼部有5例，鼻部有3例，呼吸道軟骨炎與聽覺平衡系統病變1例。於急性期中ESR值上升者有4例，合併有其他自體免疫疾病者有3例。至於治療效果，有1例僅使用非類固醇抗發炎製劑（NSAID），就達到臨床徵候緩解的結果，8例中有7例顯示對類固醇的治療有效。
紿論：對於沒有外傷或耳部手術的病史，理學檢查除耳郭有紅腫熱痛外，其他並無異狀的患者，醫師應提高警繉，將復發性多軟骨炎與耳郭軟骨膜炎做鑑別診斷。復發性多軟骨炎依症狀及病程可區分為輕微及嚴重兩群，而ESR值於急性期的高低，對於上述兩群，可以提供臨床診斷的參考。

關鍵字

復發性多軟骨炎 ； 耳郭軟骨膜炎

並列摘要

Background: Relapsing polychondritis (RP) is a rare disease of unknown etiology, characterized by recurrent inflammatory episodes of the cartilaginous structures of the ear, nose, trachea and joints. The pathogenesis is not completely understood. Auricular chondritis is the most common and earliest manifestation, presenting initially as painful swelling of the auricle.
Methods: From January 1986 to March 1998, 8 cases were collected in the department of otorhinolaryngology Veteran General Hospital-Taipei. They were diagnosed as RP according to the criteria proposed by Damiani and Levine in 1979. The age, sex, clinical presentation, laboratory data, pathology, treatment modalities, and follow-up of these patients were retrospectively reviewed.
Results: Two men and six women were included in the study. The median age was 51 (30-71 years). Auricular chondritis (8/8) and ocular damage (5/8) were the more frequent clinical manifestations. However, vestibule-cochlear damage (1/8) and laryngotracheal involvement (1/8) were less. An increased erythrocyte sedimentation rate (ESR) was found in 4 patients during the acute stage. During follow-up, 3 patients died within 3 years but 2 patients lived for longer than 5 years. Three patients had immune disease complications. One patient was treated with non-steroid anti-inflammatory drugs only and showed remission of signs with gradual subsidence of auricular swelling, tenderness and erythema. The others responded well to steroids.
Conclusions: When dealing with painful swelling and erythema of the auricle, it is important to make an accurate differential diagnosis between RP and auricular perichondritis because the treatment modalities of these two disease are different.

並列關鍵字

relapsing polychondritis ； auricular perichondritis