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韩国结节性多动脉炎的临床特征。

Clinical features of polyarteritis nodosa in Korea.

作者信息

Bae Young Deok, Choi Hyo Jin, Lee Jung Chan, Park Jeong Jin, Lee Yun Jong, Lee Eun Bong, Song Yeong Wook

机构信息

Division of Rheumatology, Department of Internal Medicine, Hallym University Kangdong Sacred Heart Hospital, Seoul, Korea.

出版信息

J Korean Med Sci. 2006 Aug;21(4):591-5. doi: 10.3346/jkms.2006.21.4.591.

Abstract

Polyarteritis nodosa (PAN) is a systemic vasculitis characterized by multi-organ involvement with protean manifestations. We evaluated the clinical features of PAN in Korea. Twenty-seven patients were diagnosed as PAN at Seoul National University Hospital between January 1990 and July 2003. The male-to-female ratio was 1.7:1 and mean age at onset (+/-SD) was 47.4+/-20 yr. Their presenting features at diagnosis were similar to those reported previously, i.e., myalgia, muscle weakness or leg tenderness (70%), fever (52%), weight loss >4 kg (44%), skin rash (44%), peripheral edema (33%), abdominal pain (33%), and arthralgia/arthritis (30%). However, the prevalence of testicular pain or tenderness was higher (24%) than reported previously and only three (11.5%) had HBsAg positivity without liver enzyme elevation. Nine patients (33%) had a five-factor score (FFS) of 2. Fourteen patients (52%) responded to treatment, 2 patients relapsed and 4 died within 1 yr of diagnosis. During a median follow-up of 55.5 months, three of the four PAN-related deaths had an initial FFS of 2. The clinical features of PAN were not significantly different from those reported previously. However, testicular pain or tenderness was more frequent and patients with a high FFS tended to have a poorer prognosis.

摘要

结节性多动脉炎(PAN)是一种系统性血管炎,其特征为多器官受累,临床表现多样。我们评估了韩国PAN的临床特征。1990年1月至2003年7月期间,27例患者在首尔国立大学医院被诊断为PAN。男女比例为1.7:1,发病时的平均年龄(±标准差)为47.4±20岁。他们在诊断时的表现与先前报道的相似,即肌痛、肌肉无力或腿部压痛(70%)、发热(52%)、体重减轻>4kg(44%)、皮疹(44%)、外周水肿(33%)、腹痛(33%)以及关节痛/关节炎(30%)。然而,睾丸疼痛或压痛的发生率(24%)高于先前报道,仅有3例(11.5%)HBsAg阳性但肝酶无升高。9例患者(33%)的五因素评分(FFS)为2分。14例患者(52%)治疗有效,2例复发,4例在诊断后1年内死亡。在中位随访55.5个月期间,4例与PAN相关死亡患者中有3例初始FFS为2分。PAN的临床特征与先前报道的无显著差异。然而,睾丸疼痛或压痛更为常见,FFS高的患者预后往往较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/608c/2729876/0e844f99234c/jkms-21-591-g001.jpg

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