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Relapsing polychondritis

机译：复发性多发性软骨炎

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PURPOSE: This article describes a clinically-diagnosed case of relapsing polychondritis (RP), attended at the Hospital S?o Paulo, and presents a literature review of the subject. SOURCE OF RESEARCH: The literature review was made via Medline (1990-96), Lilacs (1980-96), textbooks of rheumatology, and some articles about the history of the disease. In Medline, 113 articles from 1990 to 1996 were found, and there were 23 articles from 1980 to 1996 in Lilacs. RESEARCH PROCEDURE: We reviewed the articles available at BIREME (Biblioteca Regional de Medicina) with the primary focus being on the disease in question. SUMMARY: RP is a rare disease of unknown etiology described initially by Jackson-Wartenhorst in 1923 and characterized by a recurrent and acute inflammatory process that causes the collapse of the cartilaginous structures and their subsequent replacement by fibrous connective tissue. The cartilage most commonly attacked is that of the auricle of the ear and nasal septum, while the cartilage of the trachea, larynx, epiglottis, ribs, and articulations may also be involved. Ocular inflammations and systemic reactions with fever are also described. In 1976, McAdam presented a complete prospective study of 23 patients, reviewed the 136 cases described up until that time, and then proposed diagnostic criteria which were later expanded by Damiani and Levine. Currently, more than 500 cases have been described. CONCLUSION: Although a rare disease, better knowledge of it is needed, as RP may be lethal with tracheal collapse and obstruction of respiratory pathways, making precise diagnosis and adequate therapeutic intervention necessary.
机译：目的：本文描述了在圣保罗医院就诊的复发性多发性软骨炎（RP）的临床诊断病例，并提供了对该主题的文献综述。研究来源：文献综述是通过Medline（1990-96），Lilacs（1980-96），风湿病学教科书以及有关该病史的一些文章进行的。在Medline中，发现1990年至1996年的113篇文章，以及1980年至1996年在丁香花中的23篇文章。研究程序：我们回顾了BIREME（Biblioteca Regional de Medicina）上的文章，主要关注该疾病。简介：RP是一种罕见的病因不明的罕见疾病，最初由Jackson-Wartenhorst在1923年描述，其特征是反复发作的急性炎症过程，该过程导致软骨结构塌陷并随后被纤维结缔组织取代。最常见的软骨是耳和鼻中隔耳的软骨，而气管，喉，会厌，肋骨和关节的软骨也可能参与其中。还描述了眼部炎症和发烧引起的全身反应。 1976年，McAdam提出了一项对23例患者的完整前瞻性研究，回顾了当时为止描述的136例病例，然后提出了诊断标准，随后由Damiani和Levine进行了扩展。当前，已经描述了500多个案例。结论：尽管RP是一种罕见疾病，但仍需进一步了解，因为RP可能致命，并伴有气管塌陷和呼吸道阻塞，因此必须进行精确的诊断和适当的治疗干预。

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