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.     

慢性萎缩性多软骨炎

慢性萎缩性多软骨炎(PCA)为一少见疾患,1923报告第一例,至1976年已发表159例,英语作者命名为“复发性多软骨炎”。临床表现:PCA发生于所有年龄,而以40—50岁多见,无性别上的优势,且无遗传倾向,起病急剧,无诱发因素。常呈现以下几类症状。 1.软骨炎:选择性累及耳廓和鼻中隔软骨。 (1)耳廓软骨炎:1/3病例为边缘性,单侧或双侧受累,表现为红、肿、热、变粘及整个耳     

耳廓软骨膜炎治疗方法的选择

目的探讨耳廓软骨膜炎不同治疗方法的选择及效果。方法对29例耳廓软骨膜炎患者进行回顾性分析。治疗方法包括抗生素或糖皮质激素抗炎、切开引流及清创术。结果1例复发性多软骨炎患者通过糖皮质激素治疗症状控制，其他28例感染性软骨膜炎患者全部治愈，仅使用抗生素治疗5例，切开引流5例，清创术18例。铜绿假单胞菌为主要致病菌。耳廓外形无明显畸形17例，耳廓轻度畸形7例，患者耳廓明显缩小畸形5例。结论根据病因、致病菌、发病时机选择正确的方法是治愈耳廓软骨膜炎，减轻耳廓畸形的关键。     

以突发听力下降为首发表现的复发性多软骨炎

目的提高对复发性多软骨炎(relapsing polychondritis,RP)临床特征的认识。方法分析一例以突发听力下降为首发表现的复发性多软骨炎患者的临床资料,复习相关文献,对该病的流行病学、发病机制、诊断及治疗进行分析讨论。结果该病例为女性,62岁,首发症状为突发双耳听力下降,继之出现耳廓增厚,声带固定及左下支气管开口狭窄等表现。文献报道RP发病男女之比约为1:1~3,发病年龄为40~60岁,可能是自身免疫性疾病,病变常累及耳、鼻、咽喉、气管及关节软骨,内耳受累少见,但首发症状可表现为突发听力下降。临床表现是该病的主要诊断依据,血中Ⅱ型胶原抗体水平检测有助于诊断。结论RP是一种全身性免疫性疾病,可以突发听力下降为首发症状,对突聋患者的诊断要注意排除RP,以避免误诊。尤其是曾有免疫系统疾病史或多系统同时发病的患者更要警惕。     

复发性多软骨炎的耳鼻咽喉科表现

复发性多软骨炎（ｒｅｌａｐｓｉｎｇｐｏｌｙｃｈｏｎｄｒｉｔｉｓ，ＲＰＣ）是较少见的自身免疫性疾病，病变可累及全身软骨结构，包括耳、鼻、眼、喉、气管、支气管等。早期诊作者单位：５３００２１南宁广西医科大学附属第一医院耳鼻咽喉科治，及时系统的治疗则预后良好；延误诊治，病变进入晚期则为不可逆性，可造成患者毁容致残，甚至死亡。现将我科１９９０～１９９７年间收治的１１例ＲＰＣ的耳鼻咽喉科表现报道如下。本组男９例，女２例，年龄２０～６７岁。病程１０个月～５年。１．临床表现：①耳廓软骨炎１０例次，急性期耳廓红…     

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(2例报告及文献复习)

目的探讨复发性多软骨炎的临床特征及诊治方法。方法总结分析2例复发性多软骨炎患者的临床资料并复习相关文献。结果2例患者均有耳鼻喉、气管和关节等多器官软骨侵犯,予以气管切开及糖皮质激素治疗,症状控制。结论复发性多软骨炎是一种少见的软骨组织进行性炎症,病变部位和临床表现多种多样,糖皮质激素可有效控制本病,对于出现呼吸困难者需气管切开。     

伴气管狭窄的复发性多软骨炎3例

目的 探讨复发性多软骨炎（RP）的临床特点及诊治方法。方法 分析3例复发性多软骨炎患者的临床资料、相关检查结果及治疗方法,并结合文献复习。结果 3例病理检查确诊的RP患者均有典型临床表现,内镜、CT三维重建等检查可明确病变范围和严重程度,均予糖皮质激素及免疫抑制剂治疗,症状早期均有不同程度改善,但症状反复。1例复发后接受喉气管成型支架扩张术,随访至今病情稳定;1例于诊断后2年死亡;1例5年后死亡。结论 RP是一种少见的原因不明的累及全身系统的疾病,诊断需结合临床表现、CT、气管镜检查及病理检查等。糖皮质激素及免疫抑制剂能在早期部分缓解患者的症状,气管切开及狭窄气道重建能较好解除呼吸道梗柤,延长生存时间,但气道受累的RP预后较差。     

累及呼吸道的复发性多软骨炎临床分析

目的 探讨累及呼吸道的复发性多软骨炎的呼吸道的临床症状和影像特点.方法 回顾性分析3例累及呼吸道的复发性多软骨炎的病例的临床资料.结果 复发性多软骨炎的呼吸道症状有声嘶、咽痛、咳嗽、咳痰、喘息、呼吸困难等,CT检查可有喉软骨、气管、支气管软骨的增厚、钙化、软化、气道的狭窄,不同程度的声带、室带水肿.激素联合免疫抑制剂治疗效果尚可.结论 对于反复发作的声嘶以及症状不典型的咳嗽、咳痰、渐进性的呼吸困难等应及时行颈胸部CT检查,并注意系统查体,早期诊断,提高对复发性多软骨炎累及气道的认识.     

复发性多软骨炎的临床特征及处理特点(附2例报告及文献复习)

目的总结复发性多软骨炎的临床特征及处理特点.方法分析复发性多软骨炎2例的临床资料并复习文献.结果本组2例经治疗好转,经随访无复发.结论复发性多软骨炎是一种累及全身多处软骨的发作性和进行性炎症,是较少见的综合征.临床有多种表现,常见的主要损害是耳、鼻、咽喉、眼球和全身软骨.     

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。     

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。     

Large vessel arteritis in relapsing polychondritis

A healthy 58-year-old woman presented with recurrent swelling and pain of the nose and both auricules. Bruits were heard over both carotid arteries. Magnetic resonance angiography revealed stenosis of both internal carotid arteries. Relapsing polychondritis was diagnosed. These symptoms improved after treatment with prednisolone and azathioprine. Although relapsing polychondritis is sometimes associated with systemic vasculitis, large vessel arteritis is rare and can negatively affect prognosis. We conclude that the detection of systemic vascular lesions, including those involving the central nervous system, can play an important role in the diagnosis of relapsing polychondritis and that early treatment is essential for a good outcome.     

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.     

Rationale for Selective Neck Dissection in Tumors of the Upper Aerodigestive Tract

Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant –SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP – including the inner ear dysfunction – were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.     

Endolymphatic hydrops as a cause of audio-vestibular manifestations in relapsing polychondritis

Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant -SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP -- including the inner ear dysfunction -- were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.     

侵犯喉气管支气管的复发性多软骨炎

目的　分析喉、气管和支气管受侵的复发性多软骨炎 (relapsingpolychondritis,RP)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～1998年间我院收治 2 0例RP中 ,13例 (65 % )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 (46 % ) ,气管 (69% ) ,支气管 (69% )。治疗 :用皮质激素、免疫抑制剂和氨苯砜控制症状 ,4例因呼吸道梗阻行气管切开术 ,2例气管塌陷的患者用T型管或金属支架治疗。 13例中 2例治疗无效 ,死于呼吸道并发症 ,5年生存率 78 6 %。结论　呼吸道受累是RP病情严重的临床表现及主要死亡原因 ,经皮质激素、免疫抑制剂、氨苯砜治疗或气管切开术后可控制症状 ,但无法控制病程发展。     

Deterioration of hearing in a cochlear implantee with relapsing polychondritis

We report on a rare case of cochlear implantation in a patient affected by relapsing polychondritis (RP), which over time induced cochlear fibrosis/ossification and deterioration of previously excellent hearing performance. The clinical course was determined by CT scan, electrophysiology, and speech perception data. We conclude that RP is a severe autoimmune connective disorder that can cause profound sensorineural hearing loss. Cochlear implantation in these patients can provide excellent performance. Continuation of therapy may improve prognosis, but relapses involving inner ear structures can determine fibrosis/ossification of the modiolus and interfere with cochlear implant use.     

Relapsing polychondritis: a rare etiology of dysphonia and novel approach to treatment

Relapsing polychondritis affects the airway in half of all diagnosed patients. The traditional approach to the treatment of relapsing polychondritis in all sites is oral steroids and other immune-modulating drugs. We present a case of laryngeal relapsing polychondritis treated with local injections of steroid in the face of oral therapy failure. The novel approach is discussed and the disease process is reviewed.     

Relapsing polychondritis--a study of four cases.

Four case reports of relapsing polychondritis, (RP), are presented, together with a literature review and management suggestions. There are approximately 211 reported cases in world literature making RP an uncommon condition associated with high morbidity and mortality rates. The key to the management of RP is based on accurate and early diagnosis though the ideal medical regimen has yet to be elucidated.