Man Milena Adina, Alexandrescu Dana, Pop Monica, Trofor Antigona
Iuliu Haţieganu University of Medicine and Pharmacy, Department of Pneumology, Cluj Napoca, Romania.
Pneumologia. 2012 Apr-Jun;61(2):113-6.
Churg-Strauss Syndrome (allergic granulomatous angiitis) is a rare systemic and pulmonary vasculitis. We report the case of a 62 years old female, non-smoker, with a 20 years history of moderate persistent asthma treated with Salmeterol/Fluticasone 50/500 microg bid for 5 years and supplemental Montelukast in the past 5 months. The patient was admitted in our hospital with fever, malaise, sensory deficits in the lower extremities, diffuse musculoskeletal and thoracic pain. Blood eosinophil was 38% of her total WBC, thoracic computed tomography evidenced ill-defined groundglass attenuation predominantly involving the lateral segment of the middle lobe. Pulmonary infiltrates with eosinophilia can be used to define eosinophilic lung diseases. We made the differential diagnosis of eosinophilic lung disease: acute or chronic eosinophilic pneumonias, allergic bronchopulmonary aspergillosis, Loffler syndrome, Churg-Strauss syndrome, bronchocentric granulomatousis, idiopathic hypereosinophilic syndromes. Bronchoalveolar lavage showed 14.6% eosinophils. Few days after hospital admission patient experienced nausea, vomiting and diarrhea. She underwent a digestive endoscopy, which showed eosinophilic enteritis according to colon biopsy. Nasal mucosa biopsy found granulomas. Anti-neutrophil cytoplasmatic antibody (ANCA) was positive at 1:20. She displayed more than four American College of Rheumatology (ACR) criteria for Churg-Strauss Syndrome (developed while she was receiving montelukast therapy). Discontinuation of Montelukast and association of oral prednisone (1 mg/kgc) induced rapid improvement of symptoms and rapid decrease of peripheric eosinophils (72 hours). This case report illustrates the importance of early diagnosis of Churg-Strauss syndrome and the possible pathogenic link between leukotriene receptor antagonist use and CSS development.
Churg-Strauss综合征（过敏性肉芽肿性血管炎）是一种罕见的系统性和肺部血管炎。我们报告一例62岁女性，不吸烟者，有20年中度持续性哮喘病史，使用沙美特罗/氟替卡松50/500微克每日两次治疗5年，并在过去5个月中加用孟鲁司特。患者因发热、乏力、下肢感觉障碍、弥漫性肌肉骨骼和胸部疼痛入院。血液嗜酸性粒细胞占白细胞总数的38%，胸部计算机断层扫描显示边界不清的磨玻璃影，主要累及中叶外侧段。伴有嗜酸性粒细胞增多的肺部浸润可用于定义嗜酸性粒细胞性肺病。我们对嗜酸性粒细胞性肺病进行了鉴别诊断：急性或慢性嗜酸性粒细胞性肺炎、变应性支气管肺曲霉病、吕弗勒综合征、Churg-Strauss综合征、支气管中心性肉芽肿病、特发性高嗜酸性粒细胞综合征。支气管肺泡灌洗显示嗜酸性粒细胞占14.6%。入院几天后患者出现恶心、呕吐和腹泻。她接受了消化内镜检查，根据结肠活检显示为嗜酸性粒细胞性肠炎。鼻黏膜活检发现肉芽肿。抗中性粒细胞胞浆抗体（ANCA）在1:20时呈阳性。她表现出超过四项美国风湿病学会（ACR）关于Churg-Strauss综合征的标准（在接受孟鲁司特治疗期间出现）。停用孟鲁司特并联合口服泼尼松（1毫克/千克体重）后症状迅速改善，外周嗜酸性粒细胞迅速减少（72小时）。本病例报告说明了Churg-Strauss综合征早期诊断的重要性以及白三烯受体拮抗剂使用与CSS发生之间可能的致病联系。
