Université René Descartes Paris V, AP-HP, and Centre de référence maladies auto-immunes et systémiques rares, Hôpital Cochin, AP-HP, Paris, France.
Nathalie Costedoat-Chalumeau, MD, PhD: Université René Descartes Paris V, AP-HP, Centre de référence maladies auto-immunes et systémiques rares, Hôpital Cochin, AP-HP, and INSERM U1153, Center for Epidemiology and Statistics Sorbonne Paris Cité, Paris, France.
Arthritis Rheumatol. 2016 Dec;68(12):2992-3001. doi: 10.1002/art.39790.
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.
We performed a retrospective study of 142 patients with RP who were seen between 2000 and 2012 in a single center.
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 13 ± 9 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.
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 hematologic malignancy. We identified 3 distinct phenotypes.
复发性多软骨炎（RP）是一种罕见的疾病，其特征为软骨组织的反复炎症和全身表现。关于这种疾病的数据仍然很少。本研究旨在描述患者特征和疾病演变，确定预后因素，并定义 RP 的不同临床表型。
我们对 2000 年至 2012 年在一家中心就诊的 142 例 RP 患者进行了回顾性研究。
在 142 例患者中，86 例（61%）为女性。首次出现症状时的平均年龄为 43.5±15 岁。患者存在以下类型的软骨炎：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（40%；n=57）。其他主要表现为关节（69%；n=98）、眼（56%；n=80）、听觉（34%；n=48）、心脏（27%；n=38）和皮肤（28%；n=40）。在平均 13±9 年的随访中，5 年和 10 年的生存率分别为 95±2%和 91±3%。多变量分析显示，死亡的相关因素为男性（P=0.01）、心脏异常（P=0.03）以及伴发骨髓增生异常综合征（MDS）（P=0.004）或其他血液恶性肿瘤（P=0.01）。聚类分析显示，将患者分为 3 组在临床上具有相关性，从而根据临床特征、治疗管理和预后将伴有 MDS 的患者、伴有气管支气管受累的患者和不具有这 2 种特征的患者分开。
本研究纳入了大量的明确 RP 患者，与既往研究相比，生存率有所提高。死亡的相关因素为男性、心脏受累和伴发血液恶性肿瘤。我们确定了 3 种不同的表型。
