• 作者：Emma Garcí ; a-Melchor ; ^{emmitagm@gmail.com} ; Sonia Mí ; nguez Blasco ; Anna Moltó ; Revilla ; Lourdes Mateo Soria ; Susana Holgado Pé ; rez ; Alejandro Olivé ; Marqué ; s
• 关键词：Vasculitis ; Sí ; ndrome Churg-Strauss ; Anticuerpos anticitoplasma de neutró ; filo
• 刊名：Reumatolog铆a Cl铆nica
• 出版年：2010
• 期刊代码：pca303_1699258X
• 类别：med
• 出版时间：March-April 2010
• 卷：6
• 期：2
• 页码：76-81
• 文件大小：181 K

Introduction

Churg-Strauss S铆ndrome (SCS) is a necrotizing vasculitis affecting small to medium-sized vessels, characterized by lung involvement, asthma and peripheral blood eosinophilia, and pathologically by the presence of granulomas and eosinophilic infiltrates.

Objectives

This report analizes the characteristics of 9 patients with SCS diagnosed in an university referral center.

Patients and methods

Retrospective study. Between 1984 and 2007 nine patients with SCS were diagnosed in our center. Epidemiological, clinical, laboratory test as well as pathologic studies and treatment required were retrospectively analyzed.

Results

Nine patients (7 males). The mean age at the time of diagnosis was 51 years (range 23鈥?6 years). Eight of these patients had history of asthma. The more frequent organs involved were the skin (66%), musculoesqueletical system (66%), peripheral nervous system (55%) and the lung (55%). All patients presented peripheral eosinophilia. ANCA positivity was demonstrated in 6 patients (66%), most of the patients with the p-ANCA pattern. All patients were treated with corticosteroids, and in 8 immunosupressant treatment was required, mainly cyclophosphamide.

Conclusions

In this report, 9 patients with SCS are presented. Clinical characteristics are similar with the observed in other reports. We observed a major positivity of ANCA. Most of the patients were treated with corticosteroids and inmunosupresants, but the treatment should be tailored depending on the involvement of the patient.