九例继发性淋巴细胞性间质性肺炎临床及预后分析

Clinical Features and Prognosis of Secondary Lymphocytic Interstitial Pneumonia:An Analysis of Nine Cases

目的探讨继发性淋巴细胞性间质性肺炎的临床特点及预后。方法回顾性调查北京协和医院1990年至2010年病理证实的9例继发性淋巴细胞性间质性肺炎的临床及随访资料。结果 9例患者中,男3例,女6例;年龄7～64岁;6例成人患者中5例女性。3例未成年患者中,2例EB病毒感染,1例考虑继发于反复肺部感染。6例成人患者中,1例诊断为干燥综合征,1例为重叠综合征,2例为原发性胆汁性肝硬化,1例可能继发于感染,1例合并高嗜酸粒细胞血症。呼吸系统症状以咳嗽、咳痰及呼吸困难为主。全身表现以乏力、发热、体重减轻及关节疼痛为常见。影像学主要征象为双肺弥漫病变或间质改变,以双肺弥漫或双下肺为主。病理学特点为弥漫的多克隆的淋巴细胞浸润气道周围并扩展到肺间质。给予糖皮质激素及糖皮质激素联合免疫抑制剂等治疗。2例死于继发感染,存活者未发现恶性病变。结论淋巴细胞性间质性肺炎临床表现有一定特点,但特异性不强,确诊需要病理诊断。针对原发病治疗,预后尚佳。

Objective To investigate the clinical characteristics and prognosis of secondary lymphocytic interstitial pneumonia（LIP）.Methods Clinical data of 9 cases with secondary LIP diagnosed from 1990 to 2010 were retrospectively analyzed.Results Of 9 patients there were 3 males and 6 females,the range of age was 7-64 years.In the 6 adult patients there were 5 females.2 cases were infected by EB virus and 1 by recurrent pulmonary infection in 3 non-adult patients.In the adult patients,1 case was diagnosed with Sjgren＇s syndrome,1 case with overlapping syndrome,2 cases with primary biliary cirrhosis,1 case was probably caused by infection,and 1 case was complicated with eosinophilia.Dominant symptoms of pulmonary system were cough,expectoration,and shortness of breath on exertion.Dominant systematic symptoms were asthenia,pyrexia,weight lose,and arthralgia.CT revealed diffuse ground glass opacities with a lower lung zone predominance.Pathologic feature of LIP was a diffuse,polyclonal lymphoid cell infiltration surrounding airways and extending to the lung interstitium.The patients were treated by glucocorticoid and immunosuppressants.Two cases died with secondary infection.Follow-up did not comfirm malignant tumors in the survivors.Conclusions The clinical features of LIP are characteristic,but lacking of specificity.The final diagnosis depends on pathological examination.Treatment targeted on primary diseases can probably have a good efficacy,and the clinical outcome is favorable.