Sharma A, Bambery P, Wanchu A, Sharma Y P, Panda N K, Gupta A, Singh S
Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Scand J Rheumatol. 2007 Nov-Dec;36(6):462-5. doi: 10.1080/03009740701406502.
Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by recurrent episodes of inflammation and destruction of cartilaginous tissues. We describe the outcome of 10 patients followed up at the Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, over the past 10 years.
All the patients fulfilling the diagnostic criteria suggested by McAdam et al (Medicine (Baltimore) 1976;55:193-215) and modified by Damiani and Levine (Laryngoscope 1979;89;929-46) were included in the study. Detailed clinical features, investigations, treatment given, and outcome were recorded on file.
Six women and four men, mean age 48.1 years (range 26-65 years), met the criteria for diagnosis. The mean duration of symptoms, before diagnosis, was 27 months (range 1-72 months). Clinical features included auricular chondritis (100%), arthritis (80%), fever (50%), constitutional symptoms (50%), eye involvement (50%), hearing loss (40%), collapsed bridge of nose (30%), laryngotracheal involvement (20%), aortic dilatation (10%), and nephrotic syndrome (10%). Two patients had the MAGIC (mouth and genital ulcers with inflamed cartilage) syndrome. The number of episodes of cartilaginous inflammation varied from one to eight. Treatment included oral prednisolone (n = 9), intermittent 'pulse' cyclophosphamide (n = 2), and azathioprine (n = 2). One patient required tracheostomy and died later. The others are doing well. Mean duration of follow-up was 35.5 months (range 1-79 months).
The diagnosis of this potentially lethal condition is frequently delayed. Our series suggests that clinical manifestations of RP are similar in Caucasian, Oriental, and Asian populations. Laryngotracheal involvement was seen less frequently in our patients.
复发性多软骨炎（RP）是一种罕见的自身免疫性疾病，其特征为软骨组织反复出现炎症和破坏。我们描述了过去10年在昌迪加尔医学教育与研究研究生院（PGIMER）随访的10例患者的情况。
所有符合McAdam等人（《医学（巴尔的摩）》1976年；55:193 - 215）提出并经Damiani和Levine修改（《喉镜》1979年；89:929 - 46）的诊断标准的患者均纳入本研究。详细的临床特征、检查、所给予的治疗及结果均记录在案。
6名女性和4名男性，平均年龄48.1岁（范围26 - 65岁），符合诊断标准。诊断前症状的平均持续时间为27个月（范围1 - 72个月）。临床特征包括耳软骨炎（100%）、关节炎（80%）、发热（50%）、全身症状（50%）、眼部受累（50%）、听力丧失（40%）、鼻梁塌陷（30%）、喉气管受累（20%）、主动脉扩张（10%）和肾病综合征（10%）。2例患者患有MAGIC（口和生殖器溃疡伴软骨炎）综合征。软骨炎症发作次数从1次到8次不等。治疗包括口服泼尼松龙（n = 9）、间歇性“脉冲”环磷酰胺（n = 2）和硫唑嘌呤（n = 2）。1例患者需要气管切开术，随后死亡。其他患者情况良好。平均随访时间为35.5个月（范围1 - 79个月）。
这种潜在致命疾病的诊断常常延迟。我们的系列研究表明，RP在白种人、东方人和亚洲人群中的临床表现相似。我们的患者中喉气管受累较少见。
