坏死性结节病样肉芽肿病的临床与病理分析

Clinical and pathological analysis of 3 necrotizing sarcoid granulomatosis cases

目的:提高对坏死性结节病样肉芽肿病的认识.方法:结合3例坏死性结节病样肉芽肿病患者的临床资料,探讨该病的临床表现、影像学、病理、治疗和预后.结果:坏死性结节病样肉芽肿病病因不明确,临床症状可有发热、胸痛、咳嗽等,影像学主要表现为胸膜下单发、多发的肺内结节或团块;主要病理特征为肺内上皮细胞性肉芽肿、肉芽肿性血管炎和坏死.坏死性结节病样肉芽肿病对糖皮质激素治疗有效,易复发.结论:坏死性结节病样肉芽肿病罕见,临床表现无特异性,糖皮质激素治疗有效,病情易反复.肉芽肿性血管炎和坏死的严重程度可能和该病的预后相关.

AIM：To facilitate further understanding of necrotizing sarcoid granulomatosis.METHODS：Through review and analysis of the clinical documents of 3 NSG patients,the present study discusses the clinical manifestations,radiological and pathological diagnosis,treatment and prognosis of the 3 cases.RESULTS：The etiology of this rare disease remains unknown.Symptoms are most frequently fever,chest pain and productive cough etc.The radiological manifestations include solitary nodule,multiple nodules or masses in all three patients.The pathologic findings of NSG revealed confluent noncaseating sarcoid-like granulomas,granulomatous vasculitis and necrosis.Treatment with steroids appears to hasten recovery but relapses are frequent.The prognosis seems to correlate with the severity level of granulomatous vasculitis and scope of necrosis.CONCLUSION：Necrotizing sarcoid granulomatosis is rare and there are no specific definitive clinical manifestations therefore it is often misdiagnosed as other pulmonary diseases.Steroids are effective but relapses are recurrent.The prognosis seems to correlate with the severity level of granulomatous vasculitis and scope of necrosis.