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以呼吸道症状为首发的复发性多软骨炎一例

复发性多软骨炎( RP) 是一种原因不明、少见的、累及全身多系统的疾病, 表现为反复发作和缓解及进展性炎性破坏性病变等特点, 常累及软骨和其他全身结缔组织。本病好发于耳、鼻、咽、喉、气管、支气管和关节等软组织, 进而可累及多系统。部分患者以呼吸道受累为首发表现, 现报告1 例。．．．．．．

Airway Involvement in Relapsing Polychondritis: Clinical Analysis of Two Cases and Literature Review

Objective To explore the diagnosis and treatment of airway involvement in relapsing polychondritis. Methods The clinical data of two patients with relapsing polychondritis with airway involvement were reported and the relative literatures were reviewed. Results The two patients were both old males, with clinical manifestations of cough, dyspnea, and fever. They were misdiagnosed in a other hospital. The pulmonary function tests showed obstructive ventilatory impairemnt. On inspiratory CT, tracheal / tracheobronchial wall thickening and airway stenosis, with or without tracheal cartilage calcification were common findings. The tracheal cartilages thickeness and membranous wall were normal. On expiratory CT scans, functional abnormalities were identified such as tracheobronchomalacia. The patients were relieved by medication of corticosteroids or with immunodepressant. Conclusions The relapsing polychondritis with airway involvement is easy to be misdiagnosed. Chest CT examination is a valuable method for diagnosis of relapsing polychondritis. Corticosteroids and immunodepressant can improve the outcome.

以呼吸道受累为首发表现的复发性多软骨炎15例临床分析

目的提高对复发性多软骨炎累及呼吸道的认识。 方法回顾分析2004年4月至2013年7月收治的以呼吸道受累为首发表现的15例复发性多软骨炎患者。 结果15例患者多有咳嗽、发热、胸闷气促、声音嘶哑、听力下降或耳聋、耳廓塌陷、鼻梁塌陷、眼结膜充血、关节痛等临床症状。气道CT或CT三维重建可见气管、支气管管壁广泛增厚, 不规则增厚, 管腔明显狭窄, 咽喉部软组织水肿。13例行气管镜检查, 可见气管、支气管腔狭窄, 黏膜肿胀, 部分段支气管管腔闭塞, 支气管软骨环消失。7例行肺功能检查, 6例为重度混合性通气功能障碍, 1例为轻度混合性通气功能障碍。主要给予糖皮质激素联合免疫抑制剂治疗, 对有气道塌陷和重度狭窄的患者辅助手术及介入治疗。随访12例, 6例治疗后症状缓解; 6例病情反复后再入院, 其中2例死于呼吸衰竭。 结论复发性多软骨炎累及呼吸道临床表现多样, 气道CT或CT三维重建、气管镜、肺功能检查有助于诊断。糖皮质激素联合免疫抑制剂治疗有效。

气道内超声协助诊断呼吸道受累复发性多软骨炎一例及文献复习

目的提高对呼吸道受累的复发性多软骨炎(RP)的临床认识,以及气道内超声对气道壁疾病的诊断价值。 方法对1例呼吸道受累的RP患者气道内超声协助临床诊疗过程进行分析,并结合文献复习。 结果首发呼吸道症状有声嘶、咳嗽、咳痰、喘息、呼吸困难等,CT检查有气管、支气管狭窄、气管软骨变形等,支气管镜表现有不同程度的声带水肿、声门下环状软骨塌陷、声带麻痹、喉腔狭窄和主气道、左右支气管及其分支弥漫或局限狭窄、充血水肿、软骨结构消失。气道内超声显示气道壁分层模糊,软骨层破坏增厚、形状不规则。 结论呼吸道受累的RP临床容易误诊,气道内超声对气道壁疾病具有潜在的诊断价值。

Clinical characteristics of dynamic benign tracheobtonchial stenosis: two cases report

Objective To improve the knowledge on dynamic benign central airway stenosis through two typical cases. Methods The clinical features, imaging findings, and bronchial morphologic changes of two cases characterized by dynamic benign central airway stenosis were retrospectively analyzed. The etiologies for the two cases were tracheobronchomalacia (TBM) and excessive dynamic airway collapse (EDAC), respectively. Results Central airway stenosis and reversible airway obstruction were common clinical characteristics for the two cases. However, there were identifiable differences on imaging findings and bronchial morphologic changes between the two cases. Multidetector computed tomography showed sabre-sheath trachea and narrowed trachea in coronal position for TBM, while small sized trachea in exhalation phase and narrowed trachea in sagittal position for EDAC. Bronchoscopy displayed narrowed airway, swelling mucosa, and the absence of annular cartilage for TBM, while crescent airway with membranacea part protruding to lumen in inspiration phase, and the integrity of annular cartilage for EDAC. Conclusion Multidetector computed tomography and bronchoscopy examinations are valid methods to distinguish TBM and EDAC, which are both characterized by dynamic benign central airway stenosis.

应用硅胶假体矫正复发性多软骨炎所致鞍鼻畸形一例

18 例以下呼吸道受累为首发表现的复发性多软骨炎临床分析

目的分析以下呼吸道受累为首发表现的复发性多软骨炎的临床特征，以提高诊断和治疗水平。方法收集四川大学华西医院 2015 年 1 月至 2018 年 12 月收治的以下呼吸道受累为首发表现的复发性多软骨炎住院患者 18 例临床资料，进行回顾性分析，并进行相关文献复习。结果18 例患者中，男 13 例，女 5 例，年龄 26～71 岁。症状：咳嗽 17 例（94%），咳痰 16 例（89%），气紧 15 例（83%），发热 7 例（39%），声嘶 6 例（33%），胸痛 4 例（22%）。计算机体层成像（CT）及支气管镜发现狭窄或黏膜肿胀增厚 17 例（94%）。1 例行气管切开，安置气管套管。11 例单用糖皮质激素治疗，6 例使用糖皮质激素联合免疫抑制剂治疗，1 例放弃治疗。结论复发性多软骨炎患者在疾病早期临床症状无特异性，易造成不重视，同时易出现误诊漏诊。CT、气管镜检查及正电子发射计算机体层成像对确诊有较大临床意义。糖皮质激素和免疫抑制剂治疗有效。