Lung disease is a frequent clinical manifestation in people living with primary immunodeficiency diseases, the most prevalent of which are common variable immunodeficiency disorders (CVID). CVID is primarily characterised by antibody deficiency, but recent definitions and diagnostic criteria recognise a much more complex pattern of immunological defects [1]. CVID can be classified into two major clinical phenotypes. One group experiences infection as the only major clinical manifestation, whilst the other present a variety of lymphoproliferative, inflammatory and/or autoimmune complications. The ERS eGLILDnet CRC is working for better care and research for people affected by GLILD http://bit.ly/37WZGdd

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原发性免疫缺陷的间质性肺病：迈向更光明的未来

肺部疾病是原发性免疫缺陷病患者的常见临床表现，其中最常见的是常见变异免疫缺陷病 (CVID)。CVID 的主要特征是抗体缺乏，但最近的定义和诊断标准认识到了一种更为复杂的免疫缺陷模式 [1]。CVID 可分为两种主要的临床表型。一组将感染作为唯一的主要临床表现，而另一组则出现各种淋巴组织增生、炎症和/或自身免疫并发症。ERS eGLILDnet CRC 正在努力为受 GLILD 影响的人们提供更好的护理和研究 http://bit.ly/37WZGdd