Maarschalk-Ellerbroek L J, de Jong P A, van Montfrans J M, Lammers J W J, Bloem A C, Hoepelman A I M, Ellerbroek P M
Department of Internal Medicine and Infectious Diseases, University Medical Centre of Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands,
J Clin Immunol. 2014 Aug;34(6):642-54. doi: 10.1007/s10875-014-0068-6. Epub 2014 Jun 21.
Pulmonary disease is common in patients with common variable immunodeficiency disorders (CVID) and involves infections, chronic airway disease and interstitial lung disease. Chronic pulmonary disease is associated with excess morbidity and early mortality and therefore early detection and monitoring of progression is essential.
Thin slice CT scan and pulmonary function were used to determine the prevalence and spectrum of chronic (pre-clinical) pulmonary disease in adult CVID patients regardless of symptoms. CT Scans were scored for airway abnormalities (AD) and interstitial lung disease (ILD). Other CVID related complications and B and T lymphocyte subsets were analyzed to identify patients at risk for pulmonary disease.
Significant pulmonary abnormalities were detected in 24 of the 47 patients (51%) consisting of AD in 30% and ILD in 34% of cases. In only 7 (29%) of these 24 patients pulmonary function test proved abnormal. The presence of AD was correlated to (recurrent) lower respiratory tract infections despite IgG therapy. The presence of ILD was correlated to autoimmune disease and a reduction in the numbers of CD4 + T cells, naïve CD4 + T cells, naïve CD8 + T cells and memory B cells and lower IgG through levels over time.
Preclinical signs of AD and ILD are common in CVID patients despite Ig therapy and do not correlate to pulmonary function testing. Patients at risk for ILD might be identified by the presence of autoimmunity or a deranged T cell pattern. Larger studies are needed to confirm these findings and to determine thresholds for the T lymphocyte subsets.
肺部疾病在普通可变免疫缺陷疾病（CVID）患者中很常见，包括感染、慢性气道疾病和间质性肺病。慢性肺部疾病与发病率过高和早期死亡相关，因此早期检测和病情进展监测至关重要。
使用薄层CT扫描和肺功能检查来确定成年CVID患者中慢性（临床前）肺部疾病的患病率和范围，无论其有无症状。对CT扫描结果进行气道异常（AD）和间质性肺病（ILD）评分。分析其他与CVID相关的并发症以及B和T淋巴细胞亚群，以识别肺部疾病风险患者。
47例患者中有24例（51%）检测到明显的肺部异常，其中30%为AD，34%为ILD。在这24例患者中，只有7例（29%）肺功能测试结果异常。尽管进行了IgG治疗，但AD的存在与（反复）下呼吸道感染相关。ILD的存在与自身免疫性疾病以及CD4⁺T细胞、初始CD4⁺T细胞、初始CD8⁺T细胞和记忆B细胞数量减少以及随着时间推移IgG水平降低相关。
尽管进行了Ig治疗，但AD和ILD的临床前体征在CVID患者中很常见，且与肺功能测试无关。存在自身免疫或T细胞模式紊乱的患者可能存在ILD风险。需要更大规模的研究来证实这些发现，并确定T淋巴细胞亚群的阈值。
