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皮肤结节性多动脉炎的临床表现、临床病程及预后标志物

Manifestations, clinical course and prognostic markers in cutaneous polyarteritis nodosa.

作者信息

Munera-Campos Mónica, Bielsa Isabel, Martínez-Morillo Melania, Aparicio Gloria, Olivé Alejandro, Ferrándiz Carlos

机构信息

Department of Dermatology, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, Barcelona, Spain.

Department of Rheumatology, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, Barcelona, Spain.

出版信息

J Dtsch Dermatol Ges. 2020 Nov;18(11):1250-1259. doi: 10.1111/ddg.14271. Epub 2020 Oct 4.

Abstract

BACKGROUND AND OBJECTIVES

Cutaneous polyarteritis nodosa (CPAN) is a comparatively rare form of vasculitis that affects small arteries and arterioles in the panniculus and dermo-subcutaneous junction. Limited information is available regarding its course in the European population. The aim of this study is to characterize the manifestations and prognostic markers of recurrence in CPAN.

PATIENTS AND METHODS

We report a retrospective study of patients with clinical and histopathologic evidence of CPAN, which was treated at two tertiary referral centers in Spain between 1989 and 2019.

RESULTS

31 patients were included. The most frequent manifestation was subcutaneous nodules (90.3 %); ulcers were frequent at diagnosis (35.5 %). Two thirds of the patients had at least one extracutaneous manifestation. Seventeen patients (54.8 %) experienced relapse. The strongest predictor of recurrence was ulceration in the initial episode (OR 18.6; 95 % CI 2.73-38; p < 0.01). The pre-treatment results of laboratory parameters associated with inflammation (such as C-reactive protein and neutrophil-to-lymphocyte ratio) were significantly higher in the relapsing group. There were no disease-related deaths and none of the patients developed systemic PAN.

CONCLUSIONS

Although CPAN is a vasculitis limited to the skin, symptoms may involve adjacent skeletal muscle or peripheral nerves. While the condition is not life-threatening, the presence of ulceration and elevation of certain laboratory parameters predicts a worse prognosis.

摘要

背景与目的

皮肤型结节性多动脉炎(CPAN)是一种相对罕见的血管炎形式,累及皮下组织和真皮 - 皮下交界处的小动脉和小静脉。关于其在欧洲人群中的病程信息有限。本研究的目的是描述CPAN复发的表现和预后标志物。

患者与方法

我们报告了一项对有CPAN临床和组织病理学证据患者的回顾性研究,这些患者于1989年至2019年在西班牙的两个三级转诊中心接受治疗。

结果

纳入31例患者。最常见的表现是皮下结节(90.3%);诊断时溃疡很常见(35.5%)。三分之二的患者至少有一种皮肤外表现。17例患者(54.8%)复发。复发的最强预测因素是初次发作时的溃疡(OR 18.6;95% CI 2.73 - 38;p < 0.01)。复发组中与炎症相关的实验室参数(如C反应蛋白和中性粒细胞与淋巴细胞比值)的治疗前结果显著更高。没有与疾病相关的死亡,且没有患者发展为系统性结节性多动脉炎(PAN)。

结论

虽然CPAN是一种局限于皮肤的血管炎,但症状可能累及相邻的骨骼肌或周围神经。虽然该病不会危及生命,但溃疡的存在和某些实验室参数的升高预示着预后较差。

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