复发性多软骨炎的临床特征：日本8例报告
Clinical Characteristics of Relapsing Polychondritis: A Report of 8 Cases in Japan.
作者信息
Tajiri Morihiro, Tominaga Masaki, Kinoshita Takashi, Nakamura Masayuki, Kaieda Shinjiro, Okamoto Masaki, Ida Hiroaki, Kawayama Tomotaka, Hoshino Tomoaki
机构信息
Division of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine.
出版信息
Kurume Med J. 2019 May 16;65(2):47-53. doi: 10.2739/kurumemedj.MS652006. Epub 2019 Mar 11.
OBJECTIVES
Relapsing polychondritis (RP) is a very rare autoimmune disorder characterized by recurrent episodes of inflammation and destruction of cartilaginous tissues. We examined the clinical characteristics, management, and outcomes of Japanese RP patients.
METHODS
We identified 8 RP cases in our department between 2003 and 2017. Detailed clinical features, testing, treatment, and outcomes were recorded.
RESULTS
The mean time from symptom onset to diagnosis was 9 months. Four cases presented with auricular chondritis and laryngotracheal involvement and 3 cases presented with a saddle nose deformity. Anti-type II collagen antibody was positive in 5 of 6 cases. Of 3 cases with associated diseases (rheumatoid arthritis, ulcerative colitis, and Sjögren's syndrome), 2 died of respiratory failure.
CONCLUSIONS
When RP is diagnosed, early computed tomography or pulmonary function testing is essential to enable early treatment. Undiagnosed airway involvement can cause tracheobronchial wall fibrosis, leading to fixed stenosis.
目的
复发性多软骨炎（RP）是一种非常罕见的自身免疫性疾病，其特征为软骨组织反复出现炎症和破坏。我们研究了日本RP患者的临床特征、治疗方法及预后情况。
方法
我们在2003年至2017年间在本部门确诊了8例RP病例。记录了详细的临床特征、检查、治疗及预后情况。
结果
从症状出现到确诊的平均时间为9个月。4例出现耳廓软骨炎和喉气管受累，3例出现鞍鼻畸形。6例中有5例抗II型胶原抗体呈阳性。在3例合并相关疾病（类风湿性关节炎、溃疡性结肠炎和干燥综合征）的患者中，2例死于呼吸衰竭。
结论
诊断RP时，早期计算机断层扫描或肺功能检查对于早期治疗至关重要。未被诊断出的气道受累可导致气管支气管壁纤维化，进而导致固定性狭窄。
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