支气管镜观察复发性多软骨炎患者支气管软骨的异常畸形及随后出现的气道狭窄。
Bronchoscopic observation of unusual deformities of bronchial cartilage and subsequent airway narrowing in respiratory relapsing polychondritis.
作者信息
Kawano Tetsuya, Matsuse Hiroto, Kinoshita Naoe, Tsuchida Tomoko, Nishino Tomoya, Fukushima Chizu, Hayashi Tomayoshi, Kohno Shigeru
机构信息
2 Department of Internal Medicine, Nagasaki University Hospital, Nagasaki, Japan.
出版信息
Am J Case Rep. 2012;13:114-7. doi: 10.12659/AJCR.883091. Epub 2012 Jun 19.
BACKGROUND
Relapsing polychondritis (RP) is a rare inflammatory disease characterized by recurrent chondritis and inflammation of other proteoglycan-rich tissues. An RP patient with co-existing respiratory tract problems could have a poor prognosis.
CASE REPORT
We reported a case of RP died with recurrent suffocation. At the early stage in this case, unusual deformities of bronchial cartilage were observed. Following systemic corticosteroid therapy, these deformities disappeared, and typical diffuse mucosal edema and dynamic collapse of airways developed.
CONCLUSIONS
These bronchoscopic abnormalities could be the early stage of RP.
背景
复发性多软骨炎（RP）是一种罕见的炎症性疾病，其特征为反复出现软骨炎以及其他富含蛋白聚糖组织的炎症。患有呼吸道问题的RP患者预后可能较差。
病例报告
我们报告了一例因反复窒息死亡的RP患者。在该病例早期，观察到支气管软骨出现异常畸形。经过全身糖皮质激素治疗后，这些畸形消失，随后出现典型的弥漫性黏膜水肿和气道动态塌陷。
结论
这些支气管镜检查异常可能是RP的早期表现。
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