肺坏死性结节病样肉芽肿病临床病理分析

Pulmonary necrotizing sarcoid granulomatosis: a clinicopathologic analysis

目的探讨肺坏死性结节病样肉芽肿病的临床病理学特征及其鉴别诊断。方法对2例发生在肺内的坏死性结节病样肉芽肿病进行光镜观察、PAS特殊染色和免疫组化标记。结果2例患者均为无明显诱因下胸痛,胸部X线平片及CT显示肺部多个结节状阴影,胸膜增厚。镜检肺组织内形成上皮样肉芽肿,肉芽肿中央有非干酪样凝固性坏死,多核巨细胞、淋巴浆细胞浸润,小血管炎,周围纤维组织增生。抗酸杆菌染色、PAS染色(-),肺肉芽肿区域组织内CD8(+),T淋巴细胞增多。经类固醇药物治疗后2例患者临床症状均好转。结论坏死性结节病样肉芽肿病是一种较少见的多发于肺内的良性肉芽肿性疾病,病理诊断应侧重于与结节病、Wegener肉芽肿以及结核、霉菌感染等引起的肉芽肿性疾病相鉴别。

Objective To study the clinical and pathological features and differential diagnosis of pulmonary necrotizing sarcoid granulomatosis （ NSG）. Methods Two cases of pulmonary necrotizing sarcoid granulomatosis were investigated by light microscopy, special staining of PAS and immunohistochemitry, respectively. Results Chest X-ray and computed tomography （CT） scan revealed multiple nodules as well as bilateral pleural thickening in these two cases. Microscopically, nodular aggregates of epitheIioid granulomas were present in the lesions of the lung. Infarct-like necrosis which was different from chaseous necrosis was an unique feature in cases of NSG. It was induced by granulomatous vasculitis leading ultimately to vascular obstruction. Areas of necrosis were surrounded by multinucleated giant cells, lymphocytes and plasma cells. Pulmonary fibrosis was seen around the nodules. PAS staining was negative and mycobacterium tuberculosis infection was not detected in the lung tissue. Immunohistochemically, the number of CDg positive T lymphocyte in the lesion was increased. The two patients were recovered after steroid treatment. Conclusion Pulmonary necrotizing sacoid granulomatosis is an uncommon benign granulomatous disease. Before pathological diagnosis, differential diagnosis should be made from other various types of pulmonary granulomatosis such as sarcoidosis and Wegener＇s granulomatosis and some infectious granulomatosis induced by tuberculosis or fungal infection.