Hasegawa Mizue, Sakai Fumikazu, Okabayashi Asako, Sato Akitoshi, Yokohori Naoko, Katsura Hideki, Asano Chihiro, Kamata Toshiko, Koh Eitetsu, Sekine Yasuo, Hiroshima Kenzo, Ogura Takashi, Takemura Tamiko
Division of Respiratory Medicine, Tokyo Women's Medical University Yachiyo Medical Center, Japan.
Department of Diagnostic Radiology, Saitama Medical University International Medical Center, Japan.
Intern Med. 2017 Nov 1;56(21):2899-2902. doi: 10.2169/internalmedicine.7757-16. Epub 2017 Sep 15.
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease. Interestingly, the lung lesions of this case resolved immediately after the initiation of intravenous immunoglobulin monotherapy.
普通可变型免疫缺陷(CVID)是免疫缺陷疾病的一个异质性亚组。复发性细菌感染是CVID的主要特征,但也可能出现各种非感染性并发症。一名42岁女性出现咳嗽和胸部X线阴影异常。实验室检查显示显著的低丙种球蛋白血症。计算机断层扫描显示双侧肺野有多个实变和结节、全身淋巴结肿大以及脾肿大。一份手术肺活检标本最终诊断为CVID中的淋巴增殖性疾病,归类为肉芽肿性淋巴细胞间质性肺病。有趣的是,该病例在开始静脉注射免疫球蛋白单一疗法后,肺部病变立即消退。