NEJM:淋巴细胞性间质性肺炎

A previously healthy 37-year-old man presented to the pulmonary clinic with a 10-month history of dry cough, fatigue, and dry mouth. He was a lifelong nonsmoker. On physical examination, crackles were heard at both lung bases. The oral mucosa and conjunctivae were dry, and dental caries were present. A chest radiograph showed reticular opacities at the lung bases and cystic lucencies in both lungs (Panel A, asterisks). Laboratory testing was negative for human immunodeficiency virus infection. Results were otherwise notable for normal immunoglobulin levels and an autoantibody profile consistent with Sjögren’s syndrome, which was confirmed with a minor salivary gland biopsy. Computed tomography of the chest showed well-demarcated, thin-walled peribronchovascular cysts, predominantly in the lower lungs, as well as mild ground-glass opacities and subpleural reticulation (Panel B). A surgical lung biopsy showed dense interstitial lymphocytic infiltration with marked alveolar septal thickening. Immunohistochemical analysis was negative for CD1a-positive Langerhans’ cells. A diagnosis of lymphoid interstitial pneumonia with Sjögren’s syndrome was made. Treatment with prednisone was started. Three months later, mycophenolate mofetil was started, and the dose of prednisone was slowly tapered and then stopped after 6 months. At the 1-year follow-up, the patient’s symptoms and radiographic findings were stable.

一名既往健康的37岁男性因持续10个月的干咳、乏力和口干症状到肺科诊所就诊。他一生从未吸烟。体格检查时，双肺底可闻及爆裂音。口腔黏膜和结膜干燥，并存在龋齿。胸部X线片显示肺底有网状阴影，双肺有囊状透亮区（图A，星号所示）。实验室检测人类免疫缺陷病毒（HIV）感染结果为阴性。其他检查结果中，免疫球蛋白水平正常，自身抗体谱符合干燥综合征表现，经小唾液腺活检确诊。胸部计算机断层扫描显示以双肺下叶为主的边界清晰、壁薄的支气管血管周围囊肿，以及轻度磨玻璃样阴影和胸膜下网状改变（图B）。外科肺活检显示致密的间质淋巴细胞浸润，伴有明显的肺泡隔增厚。免疫组织化学分析显示CD1a阳性的朗格汉斯细胞呈阴性。诊断为伴有干燥综合征的淋巴细胞性间质性肺炎。开始使用泼尼松治疗。3个月后，开始使用吗替麦考酚酯，并逐渐缓慢减少泼尼松剂量，6个月后停用。在1年随访时，患者的症状和影像学表现均稳定。