Pulmonary abnormalities in patients with primary hypogammaglobulinemia
Pulmonary abnormalities in patients with primary hypogammaglobulinemia
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DOI：
10.1016/s0091-6749(99)70085-0
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发表时间：
1999-11-01
影响因子：
14.2
通讯作者：
Ruuskanen, O
中科院分区：
文献类型：
--
作者：
Kainulainen, L;Varpula, M;Ruuskanen, O
背景：肺部并发症在原发性低丙种球蛋白血症患者中很常见。目的：本研究的目的是评估22例原发性低丙种球蛋白血症患者的肺部异常情况，其中18例为常见变量免疫缺陷，4例为X连锁无丙种球蛋白血症，并进行3年的前瞻性随访研究，以评估肺部异常的可能进展。方法：使用肺部影像(胸片、高分辨率计算机断层扫描)和肺功能测试来评估肺部改变。16例患者存在支气管扩张症，而胸片显示仅3例患者出现支气管扩张症。肺功能检查显示5例梗阻。对IJ患者进行ZI前瞻性3年随访。14例患者中有5例无症状进展，均接受静脉免疫球蛋白替代治疗，且输注前血清免疫球蛋白浓度均在5gN或以上。结论：大多数原发性低丙种球蛋白血症患者存在肺部异常。一项新的发现是，尽管接受了足够的免疫球蛋白替代治疗，患者仍可能发生静止性和无症状的肺部变化进展。高分辨率CT是监测肺部改变的首选方法。
Background: Pulmonary complications are common in patients with primary hypogammaglobulinemia. Intravenous immunoglobulin replacement therapy has been thought to reduce the occurrence of pulmonary complications, yet they do occur.Objective: The purpose of this study was to evaluate pulmonary abnormalities in 22 patients with primary hypogammaglobulinemia (18 with common variable immunodeficiency; 4 with X-linked agammaglobulinemia) and to conduct a prospective 3-year follow-up study to assess the possible progression of pulmonary abnormalities.Methods: Pulmonary changes were evaluated with use of pulmonary imaging (chest radiographs, high-resolution computed tomography), and pulmonary function testing.Results: High-resolution computed tomography revealed pulmonary abnormalities in 21 patients. Bronchiectasis was present in 16 patients, whereas chest radiographs revealed bronchiectasis in only 3 patients. Pulmonary function testing showed obstruction in 5 patients. zi prospective 3-year follow-up was conducted in IJ patients. It showed silent progression of bronchiectasis in 5 of the 14 patients, all of whom were receiving intravenous immunoglobulin replacement therapy and had preinfusion serum IgG concentrations of 5 gn or more.Conclusions: Pulmonary abnormalities develop in most patients with primary hypogammaglobulinemia. A new finding is that silent and asymptomatic progression of pulmonary changes may occur in patients despite an adequate immunoglobulin replacement therapy. High-resolution computed tomography is the method of choice in monitoring pulmonary changes.