Ananthakrishna Rajiv, Goel Ruchika, Padhan Prasanta, Mathew John, Danda Debashish
Department of Medicine, Christian Medical College and Hospital, Vellore, 632004, India.
Clin Rheumatol. 2009 Jun;28 Suppl 1:S7-10. doi: 10.1007/s10067-008-1016-8. Epub 2008 Oct 23.
Relapsing polychondritis (RP) is a rare recurring inflammatory disorder with variable clinical course. It has been described mainly in Caucasian population. Reports from other ethnic groups are few. We report seven cases of relapsing polychondritis in south Indian population. In between 1995 and 2008, seven patients fulfilling the McAdam-Damiani-Levine criteria for diagnosis of relapsing polychondritis were identified. Records pertaining to these patients were studied and clinical presentation, course, and treatment offered were analyzed retrospectively. The female-to-male ratio in our series was 2.5:1. The age of onset of symptoms ranged from 28 to 54 years, with a mean of 40.2 years. An average of 20 months, ranging from 3 months to 6 years, elapsed before the patient presented to us seeking a diagnosis. Various structural involvement in our series were as follows: pinna in four (57%), nasal cartilage in five (71%), joints in three (43%), eyes in three (43%), laryngotracheal tree in three (43%), inner ear in one (14.3%), skin in one (14.3%), and heart in one (14.3%). Associated autoimmune diseases were present in four (57%) patients in the form of one of the following in each: vasculitis, autoimmune hemolytic anemia, hypothyroidism, and rheumatoid arthritis. All seven patients received prednisolone with three of them requiring additional immunosuppressants. There was no mortality amongst the four patients who had remained on follow-up at the time of this report. Although RP is an uncommon disorder, clinicians should be aware of the manifestations so as to initiate prompt treatment and prevent complications. Our series reports less frequent auricular cartilage and skin involvement and an exceptional case of basal cell carcinoma, although the other manifestations were similar to that seen in Caucasian and other Asian populations.
复发性多软骨炎(RP)是一种罕见的复发性炎症性疾病,临床病程多变。该病主要在白种人群中被描述,其他种族的报道较少。我们报告了印度南部人群中的7例复发性多软骨炎病例。在1995年至2008年期间,确定了7例符合复发性多软骨炎诊断的McAdam - Damiani - Levine标准的患者。研究了这些患者的记录,并对临床表现、病程和所提供的治疗进行了回顾性分析。我们系列中的女性与男性比例为2.5:1。症状出现的年龄范围为28至54岁,平均为40.2岁。患者在向我们寻求诊断之前平均经过了20个月,范围从3个月到6年。我们系列中各种结构受累情况如下:耳廓4例(57%),鼻软骨5例(71%),关节3例(43%),眼睛3例(43%),喉气管树3例(43%),内耳1例(14.3%),皮肤1例(14.3%),心脏1例(14.3%)。4例(57%)患者伴有以下自身免疫性疾病之一:血管炎、自身免疫性溶血性贫血、甲状腺功能减退和类风湿关节炎。所有7例患者均接受了泼尼松龙治疗,其中3例需要额外的免疫抑制剂。在撰写本报告时,4例接受随访的患者中无死亡病例。尽管RP是一种罕见疾病,但临床医生应了解其表现,以便及时开始治疗并预防并发症。我们的系列报告显示耳廓软骨和皮肤受累较少,还有一例基底细胞癌的特殊病例,尽管其他表现与白种人和其他亚洲人群中所见相似。