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肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)是一种病因未明的罕见多系统自身免疫性疾病.其特征为坏死性肉芽肿性炎和免疫性中小血管炎,大部分患者以呼吸道病变为首发症状,容易误诊为肺结核.笔者报道2015年1月本院收治的1例以“发热、肺部阴影、单侧胸腔积液”为主要临床表现的患者,诊断为肺结核及结核性胸膜炎,经抗结核治疗无效,为明确诊断,笔者组织临床专家对患者的诊断及治疗进行了讨论.患者经肺活组织检查及检测c-ANCA阳性后,最终诊断为肉芽肿性多血管炎.“,”Granulomatosis with polyangiitis (GPA) is a rare multisystem autoimmune disease of unknown etiology.Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small-and mediumsized blood.Respiratory lesions is as common as the first manifestation of GPA.It is easily misdiagnosed as pulmonary tuberculosis.We reported a patient who presented with “fever,lung shadow,unilateral pleural effusion”.He was diagnosed with tuberculosis and tuberculous pleurisy in our hospital in January 2015.The anti-TB treatment was ineffective.In order to make a definite diagnosis,the author organized the clinical experts to discuss the diagnosis and treatment of the patient,and the final diagnosis of the disease was GPA after lung biopsy and c-ANCA detection positive.