Popa Valentin, Colby Thomas V, Reich Stanley B
Department of Medicine (Dr. Popa), Methodist Hospital, Sacramento, and University of California at Davis, Davis, CA 95823, USA.
Chest. 2002 Nov;122(5):1594-603. doi: 10.1378/chest.122.5.1594.
To determine the frequency and type of interstitial lung disease (ILD) in consecutive subjects with symptomatic Ig deficiency.
One hundred forty-eight consecutive subjects with repeated respiratory infections and Ig deficiency.
Ig classes and IgG subclasses (IgGSCs), the response to vaccination, pulmonary function tests, chest radiography, CT scan, Ga scan and, when possible, BAL and lung biopsy.
Twenty-nine of 148 subjects (19 men and 10 women aged 18 to 72 years) had evidence of ILD. In 20 subjects, no cause of ILD was apparent. The remaining nine cases were ostensibly due to identifiable causes. Twenty subjects had IgGSC deficiency, 8 subjects had common variable immunodeficiency, and 1 subject had combined IgM plus IgGSC deficiency. No isotype deficiency was consistently related to a specific ILD. After administration of IV Ig, eight subjects, all with IgGSC deficiency, improved clinically, physiologically, radiologically, and occasionally histologically, regardless of immunologic or radiologic features. In this selective sample, the prevalence of ILD in consecutive subjects with recurrent respiratory infections and Ig deficiency (29 of 148 subjects; 19.6%) was higher than in the general population (0.8%; p < 0.05).
ILD in Ig deficiency is frequent and usually involves IgGSC deficiency.
确定有症状的免疫球蛋白缺乏症连续患者间质性肺疾病（ILD）的发生率和类型。
148例有反复呼吸道感染和免疫球蛋白缺乏症的连续患者。
免疫球蛋白类别和IgG亚类（IgGSCs）、疫苗接种反应、肺功能测试、胸部X线摄影、CT扫描、镓扫描，以及在可能的情况下进行支气管肺泡灌洗（BAL）和肺活检。
148例患者中有29例（19名男性和10名女性，年龄18至72岁）有ILD证据。20例患者中，ILD的病因不明显。其余9例表面上是由可识别的病因引起。20例患者有IgGSC缺乏，8例患者有常见变异型免疫缺陷，1例患者有IgM加IgGSC联合缺乏。没有一种同种型缺乏与特定的ILD始终相关。静脉注射免疫球蛋白后，8例均有IgGSC缺乏的患者在临床、生理、放射学方面，偶尔在组织学方面均有改善，无论免疫或放射学特征如何。在这个选择性样本中，反复呼吸道感染和免疫球蛋白缺乏症连续患者中ILD的患病率（148例患者中有29例；19.6%）高于普通人群（0.8%；p<0.05）。
免疫球蛋白缺乏症中的ILD很常见，通常涉及IgGSC缺乏。
