累及气道的复发性多软骨炎2例并文献复习

目的提高对侵犯气管、支气管的复发性多软骨炎的认识,减少误诊和漏诊率。方法分析我院2例累及气道的复发性多软骨炎的临床资料。结果1例患者因就诊时较晚,最后死于呼吸衰竭,另1例患者较早得到诊断,激素治疗预后较好。结论对伴有气管、支气管受累的复发性多软骨炎及时正确诊断,在疾病早期糖皮质激素治疗能显著改善症状,延缓疾病进展。     

累及气道的复发性多软骨炎27例治疗分析

目的分析累及气道的复发性多软骨炎患者的临床特点及治疗转归。方法回顾性分析气道受累的复发性多软骨炎患者的临床资料。结果 27例累及气道的复发性多软骨炎患者,女性15例,男性12例,平均年龄52岁(14～65岁),以呼吸道症状起病的23例(85.2%),病程1个月～15年。11例管腔狭窄明显者经保守治疗无效后置入气道内支架,1例支架置入失败,置入术后第2日气促评分1.1±0.3,较治疗前明显缓解(P0.05),放置支架患者均出现不同程度的肉芽增生、气道再狭窄及呼吸道感染。3例行气管切开术,3例行气管切开及支架置入术。13例管腔狭窄轻者及1例支架置入失败者给予保守治疗,均病情稳定出院,出院时气促评分1.3±0.2,较治疗前缓解(P0.05)。结论复发性多软骨炎累及气道时,首选内科保守治疗,保守治疗无效时可行支气管镜检查评估管腔狭窄程度,管腔狭窄程度严重者可选择介入治疗,能迅速缓解症状,但并发症较多。     

以呼吸道损害为唯一表现的复发性多软骨炎1例并文献回顾

目的探讨以呼吸道损害为唯一表现的复发性多软骨炎(RP)的临床特征、诊断及治疗。方法分析我院1例经计算机体层摄影术CT及支气管软骨活检术后病理诊断的RP患者的临床资料,并结合文献进行复习。结果患者为老年男性,以发热、咳嗽、咳痰为主要表现,肺功能示阻塞性通气功能障碍,胸部CT示气管主支气管管壁增厚,纤维支气管镜检查示气管支气管软骨环消失、管腔狭窄,病理检查为慢性炎症。予以糖皮质激素治疗有效。结论 RP以呼吸道单独受累的较为罕见,而呼吸系统受累常提示预后不佳,是该疾病死亡的主要原因。呼吸系统首先受累时,诊断是困难的且病情容易被耽误。影像学检查及软骨活检有助于诊断。治疗方式包括糖皮质激素或糖皮质激素联合免疫抑制剂等药物治疗及纤维支气管镜下气道成形术。     

复发性多软骨炎致气管狭窄1例及文献复习

复发性多软骨炎是一种罕见的以软骨组织炎症为特征的自身免疫性疾病,其特点是软骨组织复发性、退化性炎症,表现为全身多处软骨和结缔组织受累.复发性多软骨炎常累及呼吸道,导致气管、支气管软骨的缺失,管壁塌陷,气管狭窄等.最终多因呼吸道感染、呼吸衰竭而死亡,预后极差.由于早期症状隐匿,表现复杂,并且很多医师对该病缺乏认识,临床易导致漏诊、误诊、误治.现将我院收治的1例复发性多软骨炎报道如下.     

2例复发性多软骨炎累及气道的临床护理

临床资料2例复发性多软骨炎(relapsing polychondritis,RP)患者均为中年男性,平均年龄42岁,入院前有咳嗽,咳痰,气促症状(时间从6月至1年不等),在院外行肺功能及CT检查考虑:1慢性阻塞性肺疾病;2气管狭窄原因可能性大[1]。我院纤支镜下检查示:气管、左右主支气管黏膜明显肿胀,呈纵行皱襞样改变,致管腔变窄,软骨环消失(声门、气管膜部未累及)。内镜诊断符合多发性软骨炎表现。2例患者无其他     

复发性多软骨炎20例临床分析

目的：提高对复发性多软骨炎的认识。方法：收集并分析20例复发性多软骨炎患者的临床和实验室检查资料。结果：发病年龄35－86岁，平均53．4岁，平均病程1．1年。90％的患者均有耳软骨炎；80％有呼吸系统受累，其中75％有鼻软骨炎，4例出现严重的气管、支气管软骨炎；部分患者有关节、皮肤、心脏、血管和神经系统受累；20％合并有其他风湿性疾病或自身免疫病。结论：气管、支气管软骨受累是病情严重的信号。早期诊断，给予激素和免疫抑制剂治疗是改善预后的关键。     

中心气道狭窄的介入治疗进展

中心气道是指气管、隆突、左右主支气管及中间段支气管。中心气道本身病变阻塞管腔或管外压迫可导致中心气道狭窄或阻塞,出现严重的呼吸困难,甚至窒息死亡。李龙芸[1]报道中心气道狭窄的原因是很多的,常见的原因有气管-支气管的原发或转移性恶性肿瘤、各种气道良性肿瘤、炎性肉芽肿、气管支气管内膜结核、肺移植的吻合口狭窄、气管插管或切开的疤痕狭窄;少见的原因有多发性软骨炎、气道淀粉样变性等。中心管道狭窄严重影响病人通气,必需采取一切措施解除严重的中心气道狭窄,改善病人的通气状况,本文就中心气道狭窄介入治疗的一些进展进行综…     

3例复发性多软骨炎气道狭窄探讨支架置入与取出时机

正复发性多软骨炎(relapsing polychondritis,RP)是临床少见病,累及气道时可出现气管支气管狭窄,除药物治疗外,气道内支架置入能迅速缓解呼吸困难症状,但因其存在并发症,所以置入与取出支架的时机需严格把握。本文就3例RP探讨气道狭窄支架置入与取出时机。病例资料病例1:54岁男性,因"间断咳嗽、咳痰伴活动后气短11月,加重1月"入院。患者11月前无明显     

Y型硅酮支架治疗复发性多软骨炎1例并文献复习

正复发性多软骨炎(relapsing polychondritis,RP)是一种以软骨组织和结缔组织的复发性退化性炎症为特点的累及多系统的少见自身免疫性疾病~([1])。RP临床表现多样,早期诊断困难,尤其以呼吸道症状起病的患者,常因误诊致病情进展至气道塌陷,出现严重呼吸困难,是引起死亡的主要原因之一。当RP累及气管-支气管造成软骨破坏、塌陷、气道狭窄时,可以考虑植入永久性支架~([2])。我院2017年     

复发性多软骨炎气管损伤（附1例报告）

复发性多软骨炎气管损伤（附１例报告）首都医科大学附属北京安贞医院普内科（１０００２９）陶旨琴复发性多软骨炎（ＲｅｌａｐｓｉｎｇＰｏｌｙｃｈｏｎｄｒｉｔｉｓ简称ＲＰ）是一种原因不明的罕见疾病。是侵犯全身软骨、器官组织的结缔组织疾病、开始多以耳、鼻、喉及...     

Efficacy of three-dimensional computed tomography for evaluating airway tract involvement in patients with relapsing polychondritis

The airway tract involvement is known to be one of the most important prognostic factors in patients with relapsing polychondritis (RP). Sequential evaluations of airway tract involvements are necessary, however, the insertion of flexible bronchoscope into the affected airway tract may exacerbate their airway tract stenosis. Three-dimensional computed tomography (3D-CT) showed stenosis of the trachea and bilateral main bronchi in 2 patients with RP. 3D-CT is an effective non-invasive method for evaluating airway tract involvement in RP.     

Airway manifestations of relapsing polychondritis: treatment with cyclophosphamide and placement of bronchial stents

We report the case of a 41-year-old female patient with relapsing polychondritis and severe respiratory involvement. The patient presented with acute respiratory failure requiring endotracheal intubation. Bronchoscopy revealed tracheal collapse and inflammatory stenoses with dynamic collapse of the major airways. We describe a multidisciplinary therapeutic approach, consisting of immunosuppressive treatment, bronchoscopic placement of self-expandable stents into the collapsing bronchi, and tracheotomy. In addition, we report the effectiveness of oral cyclophosphamide for treatment of relapsing polychondritis with severe respiratory involvement after failure of other immunosuppressive agents. The problem of severe respiratory complications in patients with relapsing polychondritis and the need for a multidisciplinary approach is discussed.     

Placement of an ultraflex nitinol stent for severe tracheobronchial obstruction in a case of relapsing polychondritis]

A 59-year-old man, who had been treated for bronchial asthma since 2000, was hospitalized with high fever and productive cough in November 2003. Chest radiography on admission showed consolidations in both lower lung fields, and computed tomography demonstrated anteroposterior narrowing of both main bronchi. A physical examination revealed deformity of auricular cartilage and saddle nose, and we diagnosed him relapsing polychondritis (RP). When he was readmitted 4 months later because of severe tracheobronchial stenosis and respiratory failure he required mechanical ventilation, but it was difficult to wean him from the ventilator. Self-expandable metallic stents were placed in the left main bronchus and the trachea. After the procedure, he was successfully weared from mechanical ventilation. Since airway complications of RP can be fatal, stent implantation should be considered in the management of RP with airway manifestations.     

复发性多软骨炎五例分析

目的　探讨复发性多软骨炎 (RP)的临床特征、治疗方法 ,特别是RP合并严重气道狭窄的介入治疗。方法　结合文献资料回顾分析 5例临床病例。结果　RP是一种以全身多器官软骨结构及结缔组织反复发作性炎症为特征的自身免疫性疾病。目前以皮质类固醇激素、免疫抑制剂及氨苯砜为主的药物治疗常不能有效控制病变发展 ,其中气道软骨受累最危险 ,常需介入性对症治疗如气管造口、气管内插管、支架等。通过气道内可扩张的金属支架安置术成功缓解了一名RP患者因广泛气道软骨塌陷所致的严重呼吸困难。结论　对有特征性临床表现的病例应及早确诊并予药物治疗 ,当出现气道软骨受累时应及时给予压力支持及介入治疗以维持气道开放     

A case of relapsing polychondritis initially presenting with bronchial chondritis

Abstract

A 51-year-old woman presented with one-month history of fever, productive cough, dyspnea, hoarseness and polyarthritis. Computed tomography (CT) depicted diffuse bronchotracheal stenosis, which deteriorated in exhalation. Three-dimensional CT revealed airway stenosis from the trachea to the lobar bronchi. We made a diagnosis of relapsing polychondritis (RP). Administration of high-dose glucocorticoid and oral cyclophosphamide resolved the symptoms and the bronchial stenosis. It was noteworthy that all features presented were bronchial chondritis and polyarthritis. RP should be considered when patients have polyarthritis with respiratory symptoms. The dynamic expiratory CT and three-dimensional image reconstructions are useful for detecting and following up bronchotracheal involvement.     

Placement of an expandable metallic stent (Ultraflex) for tracheobronchial stenosis in relapsing polychondritis]

A 57-year-old man was admitted to our hospital because of fever, cough and polyarthralgia. A physical examination revealed polyarthritis and saddle nose. Chest computed tomography showed stenosis of the trachea and both main bronchi. Relapsing polychondritis (RP) was diagnosed, and steroid therapy was started. Dyspnea at rest appeared suddenly. Bronchoscopy showed stenosis from the trachea to both main bronchi. An expandable metallic stent (Ultraflex) was placed in the trachea and both main bronchi. After the procedure, bronchoscopy showed a widely patent airway, and his symptoms were improved. Since airway complications of RP can be fatal, tracheobronchial stent placement should be considered in the management of RP with airway stenosis.     

A case of relapsing polychondritis successfully assessed for airway diameters under application of bi-level positive airway pressure (bilevel PAP)]

A 52-year-old man was admitted to our department because of hypercapnic respiratory failure. His illness had begun with scleritis 5 years before, followed by swelling of the fingers and auricular cartilages and saddle nose 3 years before, when a clinical diagnosis of relapsing polychondritis was made. His chest CT after intubation revealed diffuse airway narrowing with complete collapse of both main bronchi on expiration. Though his clinical status was stabilized after initiation of mechanical ventilation and pulse-dose corticosteroid treatment, he was difficult to wean from the ventilator with a conventional on-off trial. We therefore assessed the diameters of the central airways using dynamic CT under application of bilevel PAP, which confirmed an improvement in airway patency. He was then successfully weaned from artificial ventilation using bilevel PAP, initially through tracheotomy and subsequently via a nasal mask. To our knowledge, this is the first report that confirms the advantage of bilevel PAP objectively for a patient with labile airways.     

Chronic atrophic polychondritis. A case report

Chronic atrophic polychondritis is a rare systemic disease characterized by recurring inflammation of cartilage of the ear, nose, larynx and of the tracheobronchial tree. Nowadays, it is considered to be an autoimmune disease. The case of a 25-year-old female patient hospitalized in our department for treatment of a dyspnea is herein presented. ORL laryngoscopic examination revealed a small sized larynx with a pronounced narrowing of its lumen and very "floppy" cartilage. Bronchoscopic findings were a circumferential stenosis along the entire trachea and the right major bronchi. Results from biopsies performed on the trachea and thyroid cartilage were in favor of chronic atrophic polychondritis. The course of the disease was characterized by an aggravation of the dyspnea with episodes of asphyxia resulting in a tracheotomy. The tracheotomy has not been closed to this day, due to the underlined disease of the patient.     

Acute airway obstruction in relapsing polychondritis: treatment with pulse methylprednisolone

We describe 2 adolescents with relapsing polychondritis who developed acute airway obstruction. Both were successfully treated with intravenous steroids for this complication following failure with oral steroids. Early respiratory tract involvement in younger patients seems predictive of a poor outcome and aggressive therapy with intravenous high dose steroids and/or immunosuppressive drugs appears to be indicated.