Relapsing Polychondritis Can Be Characterized by Three Different Clinical Phenotypes
Relapsing Polychondritis Can Be Characterized by Three Different Clinical Phenotypes
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DOI：
10.1002/art.39790
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发表时间：
2016-12-01
影响因子：
13.3
通讯作者：
Piette, Jean-Charles
中科院分区：
文献类型：
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作者：
Dion, Jeremie;Costedoat-Chalumeau, Nathalie;Piette, Jean-Charles
Objective.复发性多关节炎是一种罕见的疾病，其特征是软骨组织的复发性炎症和全身表现。关于这种疾病的数据仍然很少。进行这项研究是为了描述患者特征和疾病演变，识别预后因素，并定义RP的不同临床表型。方法。我们对2000年至2012年在一家单中心就诊的142例RP患者进行了回顾性研究。142例患者中，86例（61%）为女性。首次出现症状时的平均+/- SD年龄为43.5 +/- 15岁。患者有以下类型的中耳炎：耳（89%; n = 127）、鼻（63%; n = 89）、喉（43%; n = 61）、气管支气管（22%; n = 32）和/或肋软骨炎（40%; n = 57）。其他主要表现为关节（69%; n = 98）、眼科（56%; n = 80）、听前庭（34%; n = 48）、心脏（27%; n = 38）和皮肤（28%; n = 40）。在平均+/- SD随访1369年时，5年和10年生存率分别为95 +/- 2%和91 +/-3%。多变量分析中与死亡相关的因素为男性（P = 0.01）、心脏异常（P = 0.03）和伴随骨髓增生异常综合征（MDS）（P = 0.004）或其他血液系统恶性肿瘤（P = 0.01）。聚类分析显示，将患者分为3组具有临床相关性，从而将伴有MDS的患者、伴有气管支气管受累的患者和不伴有2种特征的患者在临床特征、治疗管理和复发方面分开。与以前的研究相比，这一大型系列明确RP患者的生存率有所改善。与死亡相关的因素是男性、心脏受累和伴随的血液系统恶性肿瘤。我们鉴定了3种不同的表型。
Objective. Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on this disease remain scarce. This study was undertaken to describe patient characteristics and disease evolution, identify prognostic factors, and define different clinical phenotypes of RP.Methods. We performed a retrospective study of 142 patients with RP who were seen between 2000 and 2012 in a single center.Results. Of the 142 patients, 86 (61%) were women. The mean +/- SD age at first symptoms was 43.5 +/- 15 years. Patients had the following chondritis types: auricular (89%; n = 127), nasal (63%; n = 89), laryngeal (43%; n = 61), tracheobronchial (22%; n = 32), and/ orcostochondritis (40%; n = 57). The main other manifestations were articular (69%; n = 98), ophthalmologic (56%; n = 80), audiovestibular (34%; n = 48), cardiac (27%; n = 38), and cutaneous (28%; n = 40). At a mean +/- SD followup of 1369 years, the 5- and 10-year survival rates were 95 +/- 2% and 91 +/- 3%, respectively. Factors associated with death on multivariable analysis were male sex (P = 0.01), cardiac abnormalities (P = 0.03), and concomitant myelodysplastic syndrome (MDS) (P = 0.004) or another hematologic malignancy (P = 0.01). Cluster analysis revealed that separating patients into 3 groups was clinically relevant, thereby separating patients with associated MDS, those with tracheobronchial involvement, and those without the 2 features in terms of clinical characteristics, therapeutic management, and prognosis.Conclusion. This large series of patients with definite RP revealed an improvement in survival as compared with previous studies. Factors associated with death were male sex, cardiac involvement, and concomitant hemato logic malignancy. We identified 3 distinct phenotypes.