Szczeklik Wojciech, Sokołowska Barbara Magdalena, Mastalerz Lucyna, Górka Jacek, Pacułt Karolina, Soja Jerzy, Musiał Jacek
II Katedra Chorób Wewnetrznych, Uniwersytet Jagielloński Collegium Medicum, Kraków.
Przegl Lek. 2010;67(7):439-42.
Churg-Strauss syndrome (CSS) is a rare, systemic, necrotizing, small- and middle-sized vessel vasculitis which is accompanied by blood eosinophilia, eosinophil infiltration of various tissues and bronchial asthma. Leukotriene receptor antagonists (LTRAs) may participate in the pathogenesis of the disease.
The aim of this study was a retrospective evaluation of the influence of LTRAs on the development of CSS and its later course.
Hospital charts of 24 patients from the southern Poland region with diagnosed CSS between 1999-2010 were retrospectively analyzed. Additional questionnaire concerning the LTRAs treatment was obtained from all patients. The diagnosis of the disease was reassessed and confirmed with the current guidelines.
In the studied group, before the development of CSS, 16 patients (66.7%) used LTRAs--namely montelukast. The average time from the beginning of treatment to the development of CSS symptoms was 14 months. Tapering down corticosteroid treatment was not observed. Patients treated with montelukast at the time of diagnosis had higher blood eosinopilia (7254 vs 2105, p<0.05), and more common nasal polyposis (68.75% vs 12.5%, p<0.05) with a need for polipectomy (62.5% vs 0%, p<0.05) when compared to patients not receiving the drug. Also the course of the disease was more severe in the LTRAs treated patients: more common exacerbations (5.93 vs 1.62, p<0.01) with the need for hospitalizations (1.6 vs 0.36/year, p<0.001) and need for higher base corticosteroids doses (9.75 vs 5 mg, p<0.05) and additional immunosuppressive therapy (81.25% vs 50%, p>0.05) to maintain the disease remission.
Patients receiving LTRAs before diagnosis of CSS had more severe course of the disease. Prescribing LTRAs for patients with asthma accompanied by nasal polyposis, and high blood eosinophilia needs special precautions. Further studies on the influence of LTRAs on CSS are needed.
变应性肉芽肿性血管炎（CSS）是一种罕见的系统性坏死性中小血管血管炎，伴有血液嗜酸性粒细胞增多、各种组织嗜酸性粒细胞浸润及支气管哮喘。白三烯受体拮抗剂（LTRAs）可能参与该病的发病机制。
本研究旨在回顾性评估LTRAs对CSS发生发展及其后续病程的影响。
对波兰南部地区1999年至2010年间确诊为CSS的24例患者的医院病历进行回顾性分析。从所有患者处获取关于LTRAs治疗的补充问卷。根据当前指南对疾病诊断进行重新评估并确认。
在研究组中，16例患者（66.7%）在CSS发病前使用过LTRAs，即孟鲁司特。从治疗开始到CSS症状出现的平均时间为14个月。未观察到糖皮质激素治疗减量情况。与未接受该药物治疗的患者相比，诊断时接受孟鲁司特治疗的患者血液嗜酸性粒细胞增多更明显（7254对2105，p<0.05），鼻息肉更常见（68.75%对12.5%，p<0.05），且需要息肉切除术的比例更高（62.5%对0%，p<0.05）。此外，接受LTRAs治疗的患者病程更严重：病情更频繁加重（5.93对1.62，p<0.01），需要住院治疗（1.6对0.36/年，p<0.001），需要更高剂量的基础糖皮质激素（9.75对5mg，p<0.05）以及额外的免疫抑制治疗（81.25%对50%，p>0.05）以维持疾病缓解。
在诊断CSS之前接受LTRAs治疗的患者病程更严重。为伴有鼻息肉和血液嗜酸性粒细胞增多的哮喘患者开具LTRAs时需要特别谨慎。需要进一步研究LTRAs对CSS的影响。
