复发性多软骨炎并发喉气管狭窄

目的分析复发性多软骨炎(relapsing poly- chondritis,RP)并发喉气管狭窄的临床特点和预后。方法回顾性分析1996～2006年间我院收治的6例RP并发喉气管狭窄患者的临床资料。结果6例RP并发喉气管狭窄患者,喉气管狭窄范围弥漫广泛,其中4例应用带蒂或游离组织移植行喉气管成形术,2例行胸段气管扩张术。5例手术成功,效果巩固未复发。结论因RP并发喉气管狭窄的病例,治疗更加棘手,但是如根据病情选择适当的手术时机和方法仍可取得满意的疗效。     

复发性多软骨炎1例

患者 ,男 ,48岁。自 1 998年 1 0月以来 ,先后发生右拇指腱鞘炎、右足跟疼痛、多发性关节疼痛及右眼球结膜充血。曾在多家医院做过多种详尽检查未发现异常 ,分别按“右拇指腱鞘炎”、“右足跟骨质增生”、“风湿性关节炎”和“右眼巩膜炎”治疗 ,症状未减轻。 2 0 0 1年 4月底双耳廓开始红肿、胀痛 ,左重右轻 ,外院诊断 :双耳廓化脓性软骨膜炎。 7月1 2日来我院耳鼻咽喉科就诊 ,经详细复习患者在各医院就诊的病历记录 ,拟诊为复发性多软骨炎 (ｒｅ ｌａｐｓｉｎｇｐｏｌｙｃｈｏｎｄｒｉｔｉｓ,ＲＰＣ)而收治入院。体检未见异常。专科检查 :…     

复发性多软骨炎(附3例报告及文献复习)

目的:研究复发性多软骨炎的临床表现、诊断与鉴别诊断以及治疗与预后.方法:总结3例诊断为复发性多软骨炎患者的临床表现及治疗方法.例1先出现鼻梁塌陷,后出现声嘶、呼吸困难.气管切开后带管出院,未确诊.4个月后又因双耳胀痛,胸骨旁隐痛、发热,再次住院,诊断为复发性多软骨炎.例2首发症状亦是鼻梁塌陷,2年后出现右眼突,诊断为此病.例3首发症状为耳郭红、肿、热、痛,诊断为耳郭软骨膜炎.3个月后出现声嘶,按喉炎治疗,效果不明显 ,后诊断为复发性多软骨炎.结果:经用类固醇、硫唑嘌呤等药物治疗后, 分别随访1.5年、2年、6年无复发.结论:复发性多软骨炎患者应行药物治疗.     

复发性多软骨炎

复发性多软骨炎病因不清，常与结缔组织病并存，发病机理可能与免疫介导有关。由于本病无特异性病理改变，易误诊或漏诊，本文对其进行综述。     

复发性多软骨炎2例

例 1 　 男 ,45岁 ,4年前常感左侧胸部、胸骨柄处疼痛和双手指关节肿痛 ,在医疗所按风湿病治疗 ,时重时轻。 2年后出现鼻腔阻塞感 ,常有较多干痂擤出 ,约半个月后突然发生鼻梁凹陷 ,且逐渐加重 ,当地医生按鼻炎治疗无明显效果。半年前左眼球发胀 ,渐见突出 ,伴有声音嘶哑。近 2个月来病情加重 ,出现视力减退、头痛、复视和进行性呼吸困难 ,在某院行气管切开 ,术后拔管困难 ,于 1 997年4月 2 8日转入我院治疗。检查 :左眼裸眼远视力0 .0 2 ,眼球明显外突 ,眼球突出左 1 8mm,右 1 4mm,且向颞侧运动明显受限 ,球结膜充血 ,角膜、瞳孔及眼底均…     

复发性多软骨炎26例临床分析

目的：探讨复发性多软骨炎的临床特征和早期诊疗方法。方法：对26例患者分别采用抗生素、激素及免疫抑制剂药物治疗,采取气管切开、气管内金属支架植入和无创呼吸机治疗。结果：23例（88.5%）耳廓软骨受累,20例（76.9%）呼吸道软骨受累,18例（69.2%）关节软骨受累,15例（57.7%）鼻软骨受累,6例（23.1%）眼部受累,4例（15.4%）耳蜗和（或）前庭受累。部分患者有心脏、血管、肾脏受累。1例严重肺部感染死亡,1例呼吸衰竭死亡,其余24例病情稳定或有不同程度缓解。结论：复发性多软骨炎临床表现多样,主要集中在耳鼻咽喉科方面,呼吸系统受累预后不良。早期诊断、早期治疗可控制病情,改善预后。     

侵犯喉气管支气管的复发性多软骨炎

分析喉,气管和支气管受侵的复发性多软骨炎临床特点及预后。方法回顾性分析１３例侵犯喉,气管和支气管的ＲＰ患者临床资料。结果１９８３年－１９９８８年间我院收治２０例ＲＰ中,１３例累及喉,气管和支气管,病变范围为弥漫性或局限性,病变部位：喉,气管,支气管。     

复发性多软骨炎

目的提高对复发性多软骨炎(relapsingpoly-chondritis,RP)的认识和早期诊断水平。方法结合文献回顾分析34例RP临床病例资料,重点分析RP在耳鼻咽喉科方面的表现。结果全部34例均有耳、鼻、喉或气管等部位的受累表现,累及其中两处以上者27例(79%),以这几个部位的表现为首发症状或体征者鼻19例(56%),喉19例(56%),气管18例(53%),支气管18例(53%),关节或/和肋软骨22例(65%),眼部15例(44%)。首诊于耳鼻咽喉科者10例。首次发作确诊者仅13例(38%)。确诊前病情反复发作最多者达8次。结论RP的早期表现不典型,易误诊漏诊。早期确诊的关键是熟知该病的临床特点和诊断标准。     

复发性多软骨炎11例临床分析

目的：提高对复发性多软骨炎的认识和早期诊断水平。方法：回顾性分析11例复发性多软骨炎病例，并通过文献复习，对其资料进行分析。结果：耳廓软骨炎首发者9例，关节炎首发者2例；累及呼吸道者2例，其中死亡1例。11例中初诊误诊9例，误诊率达81．82％。结论：该病临床表现复杂多样，可侵犯软骨和结缔组织，涉及多个器官，易误诊、漏诊。病变累及呼吸道者预后不良。为降低病死率，改善预后，应早期诊断和及时治疗，而熟悉本病的临床表现对早期诊断有重要意义。     

以气道受累为主的复发性多软骨炎临床诊治进展

复发性多软骨炎(RP)是一种以软骨及富含蛋白多糖的组织受累为主的免疫性多系统疾病,该病十分罕见,临床表现因受累部位不同而差异较大,目前仍无特异性辅助检查作为有效确诊手段,临床诊断较困难,以气道受累为主的患者,可隐匿起病,症状不典型,易误诊和漏诊,但气道受累时病情进展较快,晚期出现喉气管狭窄时治疗相当棘手且预后不佳,给气道管理带来了更大的挑战,因此针对气道受累的RP患者,临床医生应尽可能鉴别并提高早期诊断率,治疗上不仅要采取全身治疗,还要辅以局部治疗管理气道,本文就气道受累的复发性多软骨炎的诊治进展作一综述,为临床医师提供参考。     

Corneal autoimmunity in a patient with relapsing polychondritis

Summary We report our experiences with a patient with relapsing polychondritis, in whom circulating antibodies against corneal epithelium were determined by immunofluorescence before and after medical treatment. The results provide further evidence for an autoimmune pathogenesis of the disease.     

Anti-labyrinthine antibodies in a patient with relapsing polychondritis

Relapsing polychondritis is a rare inflammatory disease that causes destruction of cartilaginous tissue in various anatomical regions. We report here about a 55-year-old female patient with relapsing polychondritis that involved the right auricle, both audiovestibular organs and both eyes. The patient presented with persisting inflammation of the right auricle, sudden lower-frequency hearing loss, acute moderate vertigo with nausea and mild ocular symptoms. Immunofluorescence assays were used for the detection of antibodies against the cochlea and the vestibular organ and demonstated the presence of circulating antibodies against the audiovestibular organ. No staining for anti-corneal IgG was detected. Improvement of clinical disease was achieved by treatment with systemic steroids and vasodilator drugs, and long-term medication with low-dose corticosteroids. Received: 21 October 1998 / Accepted: 13 January 1999     

A case of relapsing polychondritis with respiratory symptom manifestation alone

Relapsing polychondritis, a comparatively rare condition systemically affecting organs containing collagen, such as cartilage tissue, is diagnosed based on clinical manifestations and pathological findings. Our case report involves a respiratory symptom alone. A 65-year-old man seen for fever and dyspnea was found in computed tomography and endoscopy to have subglottic and tracheal stenosis, necessitating tracheostomy and tracheal cartilage biopsy. Pathologically bony cartilage change was seen but no definite auricular inflammation findings. Additional auricular cartilage biopsy, showed no inflammation. Residual auricular cartilage scarring, however, indicated cartilage destruction by previous inflammation or trauma, yielding a final diagnosis of relapsing polychondritis. Steroid therapy quickly relieved symptoms. Auricular cartilage biopsy thus proved useful in definitively diagnosing this condition.     

病变累及呼吸道的复发性多软骨炎临床特征分析

目的　研究病变累及呼吸道的复发性多软骨炎临床特点。方法　回顾性分析2007年1月～2014年12月我院56例病变累及呼吸道复发性多软骨炎患者临床资料,喉部、气管病变内镜及影像学特征和治疗。结果　吸气性呼吸困难51例（91.1%）,声音嘶哑49例（87.5%）,咳嗽咳痰15例（26.8%）,发热5例（8.9%）,咽部异物感1例（1.8%）。综合喉镜及CT结果评估病变累及范围：喉部28例,喉及颈段气管7例,喉及气管全长的15例,累及气管及支气管的6例。28例行气管切开（1例伴支气管支架）者,8例拔气管套管,4例患者后期放置T管支撑,3例拔管。2例患者放置气管支架治疗。结论　复发性多软骨炎患者早期临床表现隐匿,容易漏诊误诊,喉镜检查、颈部及肺部CT有临床特异性,疾病后期出现的声门下和气管上段狭窄是导致不能拔除气管套管的重要原因,降低患者生活质量。     

A case of relapsing polychondritis preceded by inner ear involvement

Relapsing polychondritis is a rare disease of unknown etiology causing systematic inflammation of the whole cartilaginous tissues. We report a patient with relapsing polychondritis preceded by inner ear involvement. A 68-year-old female visited our hospital for a chief complaint of sudden bilateral hearing loss. On pure-tone audiometry, sensorineural hearing loss, 47dB on the right and 51dB on the left, was observed. Later, bilateral auricular chondritis and uveitis developed. On biopsy of the auricular cartilage, perichondritis was observed. The patient was diagnosed with relapsing polychondritis in the early stage, 2 weeks after the onset. Oral predonine administration was initiated, and the auricular inflammation was improved within 10 days. The hearing loss disorder was gradually improved. The steroid dose was reduced, and no decrease in hearing has been observed so far.     

Total hearing loss and blindness caused by relapsing polychondritis and myelodysplastic syndrome

Relapsing polychondritis is an autoimmune disease that primarily manifests as cartilaginous tissue destruction. However, the immune impairment may also involve noncartilaginous tissues such as kidneys, blood vessels, etc. The disease may occur as a primary disorder or in association with other diseases. The case of a female patient with concurrent manifestation of myelodysplastic syndrome—refractory anemia with excess blasts, and relapsing polychondritis, is presented. The diagnosis of relapsing polychondritis was established ten months after the diagnosis of myelodysplastic syndrome, when the criteria for the former were met. Total hearing loss and blindness developed soon thereafter.