Erosive arthritis in relapsing polychondritis

We report the case history of a 57 year old man who has suffered from typical deforming, relapsing polychondritis for 13 years. He has also developed erosive destructive seronegative polyarthritis involving some of his distal interphalangeal, proximal interphalangeal, metacarpophalangeal, intercarpal, wrist, intertarsal and metatarsophalangeal joints. The distribution of joint involvement in the small joints of the hands and feet is asymmetrical. Both hips and knee joints have also been involved necessitating bilateral total hip and right total knee replacement. The articular associations with relapsing polychondritis are discussed.     

Cryptogenic cirrhosis in relapsing polychondritis

A case of relapsing polychondritis with histologically proven cirrhosis is reported. This association has not been described before.     

Vascular involvement in relapsing polychondritis.

Review of four cases of relapsing polychondritis (RP) seen at one hospital in the 12-year period 1963 to 1974 revealed that one patient had aortic insufficiency with large artery involvement, two others had involvement of medium and large arteries and the fourth may have had mucocutaneous vasculitis. Valvular disease has occurred in 9% of all cases of RP reported in the literature and, if vasculitis beyong the aortic root is included, 25% of cases of RP manifested inflammatory vascular disease. The frequency of pseudotumour of the orbit and cochlear-labyrinthine dysfunction is also high and may be a manifestation of vasculitis.     

复发性多软骨炎呼吸道受累的特点

目的研究复发性多软骨炎(RP)呼吸道受累的特点。方法回顾性分析呼吸道受累和未受累的RP患者临床表现、呼吸功能试验、胸部CT、支气管镜检查的特点及其间相关性。结果38例呼吸道受累患者中男性20例、女性18例,发病年龄45岁±11岁(27~71岁),症状有咳嗽、咯痰、声嘶、憋气、喘息、呼吸困难等。18例行呼吸功能试验,其中阻塞性通气功能障碍10例,混合性通气功能障碍7例;与无呼吸道受累的RP患者相比,呼吸道受累者FEV1(1·4L±0·23Lvs2·5L±0·09L,P=0·001)、FEV1/FVC(46·0±4·86vs83·7±2·24,P=0·000)、PEF(3·3L/s±0·67L/svs6·9L/s±0·52L/s,P=0·006)、FEF50/FIF50(0·3±0·08vs1·3±0·51,P=0·010)、MMEF25~75(0·4L/s±0·18L/svs2·8L/s±0·73L/s,P=0·010)均明显下降;呼吸流速容量环显示呼气峰流速下降,呼气相平台形成。27例行胸部CT检查,气道狭窄11例、气管壁增厚8例。23例行支气管镜检查,黏膜炎症16例、软骨环消失6例、气道塌陷7例、正气道狭窄15例、左右主支气管狭窄分别为13和12例。结论呼吸功能试验是一项能早期发现RP呼吸道受累的敏感性高的检查,胸部CT和支气管镜检查对评价RP患者的气道受累情况有一定意义。     

Acute mastitis; a novel presentation of relapsing polychondritis

A 30 year old female with previous Crohn's disease presented with recurrent cutaneous vasculitis and polyarthritis. She subsequently developed recurrent transient bilateral mastitis with auricular and laryngotracheal chondritis typical of relapsing polychondritis. Acute mastitis is a previously unrecognized association of this disorder.     

Ear, nose and throat manifestations of relapsing polychondritis in a child

Relapsing polychondritis is a rare disorder, that can present initially to Otolaryngologists and can easily be overlooked due to its relative rarity and atypical initial symptoms and signs. Here we report on a 12-year-old schoolboy who presented initially with ear, nose and throat manifestations, including stridor, cough, hoarseness, sore throat and fever. The other clinical signs such as nasal tip, depression and softening of right auricle, developed subsequently. The clinical features, laboratory investigations, diagnostic criteria and treatment options were discussed with a brief review of literature.     

Anesthetic management of a child with relapsing polychondritis: a case report

Relapsing polychondritis （RP） is a rare disorder of unknown cause. It is characterized by recurrent inflammation of cartilage and connective tissue. Airway complications are the most serious manifestations. The anesthetic management of patients with RP is challenging Airway management is of primary importance because of the potential for collapse of supporting airway structures with resulting inability to intubate and ventilate the patient. Although it tends to occur in middle age （〉 40 years）, it has been reported in younger individuals. Herein we report a case of anesthetic management of a child with RP for tracheotomy combined with insertion of T tube stent and review several other cases.     

30例呼吸道受累的复发性多软骨炎临床观察

目的 研究复发性多软骨炎(RP)呼吸道受累的临床特点.方法 回顾性分析呼吸道受累RP患者临床表现、肺功能测定、胸部CT、支气管镜检查的特点.结果 30例呼吸道受累患者的症状有咳嗽、咳痰、声嘶、呼吸困难、胸痛、咯血和发热等.16例行肺功能测定,其中阻塞性通气功能障碍9例,混合性通气功能障碍5例;流速容量环显示呼气峰流速下降,呼气相平台形成.28例行胸部CT检查,气道狭窄23例、气管壁增厚18例.22例行支气管镜检查,正气道狭窄18例、左(右)主支气管狭窄14(13)例、软骨环消失10例、气道塌陷6例,气道内结节样新生物2例.结论 呼吸道受累是RP病情严重的临床表现,肺功能测定是一项能早期发现RP呼吸道受累的高敏感性的检查,胸部CT和支气管镜检查对评价RP患者的气道受累情况有一定价值.     

The value of 99mTc methylene diphosphonate bone scintigraphy in diagnosing relapsing polychondritis

Relapsing polychondritis （RP） is a recurrent disease involving cartilage mainly of the ear,nose, larynx, trachea, and bronchus. The typical manifestations of the disease in the ear and nose can be easily recognized, but the symptoms could be ignored or easily confused with those of other diseases when the cartilage of other sites is involved. Thus, it is necessary to develop a new technique for the diagnosis of this disease. Few cases of abnormal accumulation of radioactivity at cartilage shown by ^99mTc methylene diphosphonate （MDP） bone scintigraphy are described in the literature. In this report, we present 4 patients of whom 3 had positive findings on ^99mTc MDP bone scintigraphy with an assessment of ^99mTc MDP bone scintigraphy in the diagnosis of RP.     

复发性多软骨炎4例诊疗体会及文献复习

目的:分析4例复发性多软骨炎(RP)的诊疗经过,探讨RP的早期诊断及有效治疗方法,减少误诊误治。方法:回顾性复习2006年～2009年收治的4例RP病例,分析其主要临床表现及累及器官,总结治疗经验。结果:4例患者1例仅累、气管及支气管,1例累及眼、耳、鼻,1例同时累及气管、支气管及鼻、耳,1例以累及气管支气管为主,有轻度鼻骨累及。激素及免疫抑制剂对患者治疗有效。结论:临床医生需提高对RP的认识,掌握其诊断标准,RP的治疗原则,常规选用激素、免疫抑制剂,注意控制呼吸道感染。     

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我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。