Conen David, Leuppi Jörg, Bubendorf Lukas, Ronsdorf Anke, Tamm Michael, Hauser Thomas
Department of Internal Medicine, University Hospital Basel, Switzerland.
Swiss Med Wkly. 2004 Jun 26;134(25-26):377-80. doi: 10.4414/smw.2004.10661.
The association of leukotriene receptor antagonists and Churg-Strauss Syndrome (CSS) has been recognised for several years. However, whether these drugs have a direct pathogenic role remains controversial. The present case describes an asthmatic patient, who developed severe obstructive symptoms and progressive heart failure after two sequential exposures to montelukast. As the patient exhibited a markedly raised blood eosinophil count with diffuse infiltrates on chest x-ray and signs of myocarditis, CSS was suspected. The disease was confirmed by open lung biopsy. The symptoms improved rapidly after administration of high dose immunosuppression with methylprednisolone and cyclophosphamide. This case is noteworthy because the time course of events strongly suggests a direct aetiological role for montelukast in the development of CSS. The pathophysiological mechanism of the association remains unknown.
白三烯受体拮抗剂与变应性肉芽肿性血管炎(CSS)之间的关联已被认识多年。然而,这些药物是否具有直接致病作用仍存在争议。本病例描述了一名哮喘患者,在先后两次服用孟鲁司特后出现严重的阻塞性症状和进行性心力衰竭。由于患者血液嗜酸性粒细胞计数显著升高,胸部X线显示弥漫性浸润以及心肌炎体征,怀疑为CSS。经开放性肺活检确诊该病。给予大剂量甲基泼尼松龙和环磷酰胺免疫抑制治疗后症状迅速改善。该病例值得关注,因为事件的时间进程强烈提示孟鲁司特在CSS发生过程中具有直接病因学作用。两者关联的病理生理机制尚不清楚。