Shimizu Jun, Yamano Yoshihisa, Kawahata Kimito, Suzuki Noboru
Department of Immunology and Medicine, Institute of Medical Science, and Division of Rheumatology and Allergology, St. Marianna University School of Medicine, Kawasaki, Japan.
Medicine (Baltimore). 2018 Oct;97(42):e12837. doi: 10.1097/MD.0000000000012837.
Relapsing polychondritis (RP) is a multisystem disorder of cartilaginous tissues. Previously, we found that patients with respiratory involvement and patients with auricular involvement were mutually exclusive in the RP cohort, which suggests a strong inverse relationship between respiratory and auricular involvement. Here, we examined the clinical manifestation patterns in a subgroup of patients with respiratory involvement (R subgroup) and a subgroup of patients with auricular involvement (A subgroup) and investigated the clinical and laboratory characteristics of each subgroup.There were 47 patients (19.7%) and 118 patients (49.4%) allocated to the R and A subgroups, respectively. Saddle nose deformity and a progressive disease course were observed frequently in the R subgroup. Arthritis, conjunctivitis, and CNS involvement were observed frequently in the A subgroup.The remaining RP patients formed a third subgroup of patients that had both respiratory involvement and auricular involvement. We designated this subgroup as the O (overlap) subgroup, and 75 patients (31.4%) were allocated to the O subgroup. Disease duration in the O subgroup (5.70 ± 0.64 years) was significantly longer than that in the A subgroup (4.12 ± 0.45 years) and relatively longer than that in the R subgroup (4.80 ± 0.63 years).We found that cardiovascular involvement was more predominant in the O subgroup than in the R and A subgroups. Higher concentrations of serum matrix metalloproteinase (MMP)3 were observed in the O subgroup than in the R and A subgroups.We measured serum MMP3 concentrations in another patient cohort including 22 newly recruited RP patients. MMP3 concentrations were significantly higher in the O subgroup (n = 10) than those in the R subgroup (n = 6) and A subgroup (n = 10).RP patients in the R and A subgroups had different characteristics from each other, and the overlap of respiratory and auricular involvement was an important prognostic factor in patients with RP. Cardiovascular involvement was not observed in the R subgroup in RP patients. The current study may provide insights into the classification and treatment of RP.
复发性多软骨炎（RP）是一种软骨组织的多系统疾病。此前，我们发现呼吸受累患者和耳部受累患者在RP队列中相互排斥，这表明呼吸和耳部受累之间存在强烈的负相关关系。在此，我们检查了呼吸受累患者亚组（R亚组）和耳部受累患者亚组（A亚组）的临床表现模式，并研究了每个亚组的临床和实验室特征。分别有47例患者（19.7%）和118例患者（49.4%）被分配到R亚组和A亚组。R亚组中鞍鼻畸形和疾病进展过程较为常见。A亚组中关节炎、结膜炎和中枢神经系统受累较为常见。其余RP患者形成了呼吸和耳部均受累的第三组患者。我们将该亚组指定为O（重叠）亚组，75例患者（31.4%）被分配到O亚组。O亚组的疾病持续时间（5.70±0.64年）明显长于A亚组（4.12±0.45年），相对长于R亚组（4.80±0.63年）。我们发现心血管受累在O亚组中比在R亚组和A亚组中更常见。O亚组中血清基质金属蛋白酶（MMP）3的浓度高于R亚组和A亚组。我们在另一个包括22例新招募的RP患者的队列中测量了血清MMP3浓度。O亚组（n = 10）中的MMP3浓度明显高于R亚组（n = 6）和A亚组（n = 10）。R亚组和A亚组的RP患者具有不同的特征，呼吸和耳部受累的重叠是RP患者的一个重要预后因素。RP患者的R亚组中未观察到心血管受累。本研究可能为RP的分类和治疗提供见解。
