复发性多软骨炎：临床表现、疾病活动度及预后
Relapsing polychondritis: clinical presentations, disease activity and outcomes.
作者信息
Sharma Aman, Law Arjun Dutt, Bambery Pradeep, Sagar Vinay, Wanchu Ajay, Dhir Varun, Vijayvergiya Rajesh, Sharma Kusum, Gupta Ashok, Panda Naresh K, Singh Surjit
机构信息
Department of Internal Medicine, Postgraduate Institute of medical education and research, Chandigarh, India.
University of Queensland, Bundaberg Hospital, Bundaberg, Australia.
出版信息
Orphanet J Rare Dis. 2014 Dec 20;9:198. doi: 10.1186/s13023-014-0198-1.
BACKGROUND
Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India.
METHODS
A retrospective study with all patients meeting Damiani and Levine's modification of McAdam's diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index (RPDAI)], treatment and outcomes were recorded.
RESULTS
Ten men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), dermal (26%), cardiovascular (11%) and laryngotracheal involvement (11%) characterized the clinical presentations. The median RPDAI was 31 (range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years).
CONCLUSIONS
Apart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.
背景
复发性多软骨炎是一种罕见疾病，其特征为富含软骨和蛋白聚糖的结构发生炎症。由于全球范围内仅有少数已发表的单中心病例系列，我们描述了在印度北部一家三级中心对26例患者的治疗经验。
方法
对所有符合达米亚尼（Damiani）和莱文（Levine）对麦克亚当（McAdam）诊断标准修改版的患者进行回顾性研究。记录临床细节、检查、疾病活动度评估[复发性多软骨炎疾病活动指数（RPDAI）]、治疗及结果。
结果
10名男性和16名女性（中位年龄45岁）符合诊断标准。临床表现以耳软骨炎（96%）、关节炎（54%）、听力障碍（42%）、眼部病变（42%）、皮肤病变（26%）、心血管病变（11%）和喉气管受累（11%）为特征。RPDAI中位数为31（范围9 - 66）。两名患者在观察期间死亡。总生存率为92.3%（中位生存期13.5年）。
结论
除喉气管受累减少外，印度的复发性多软骨炎在临床上与其他地区记录的模式相似。
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