PDF(Pubmed)
Abstract：
BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic clonal plasma cell disorder. Pulmonary involvement is frequently found in patients with POEMS syndrome, manifesting various clinical features. Therefore, to improve diagnostic accuracy and provide treatment strategies, a comprehensive analysis of pulmonary manifestations of POEMS syndrome is needed.
METHODS: This retrospective study included patients with POEMS syndrome at Peking Union Medical College Hospital, a major referral medical center in China, between June 1, 2013, and June 1, 2023. Demographic data, laboratory findings, pulmonary function test results, echocardiograms, and chest imaging data were extracted. Continuous variables were compared using the t-test or Mann-Whitney method. Pearson\'s chi-square test or Fisher\'s exact test was conducted to compare categorical data.
RESULTS: Overall, 282 individuals diagnosed with POEMS syndrome were included in this study, of which 56% were male with an average age of 48.7 years. Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). Chest computed tomography and echocardiography findings showed that 56.4% of patients exhibited pleural effusion, 62.8% displayed mediastinal or hilar lymphadenopathy, 46.5% presented pleural thickening, 27.3% demonstrated bone lesions of the ribs or thoracic vertebra, 7.8% showed lung interstitial abnormalities, and 35.5% had pulmonary hypertension. Decreased diffuse capacity and restrictive ventilatory patterns were identified in 85.2% (115 cases) and 47.4% (64 cases) of patients, respectively. Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities.
CONCLUSIONS: Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem.
METHODS: This retrospective study included patients with POEMS syndrome at Peking Union Medical College Hospital, a major referral medical center in China, between June 1, 2013, and June 1, 2023. Demographic data, laboratory findings, pulmonary function test results, echocardiograms, and chest imaging data were extracted. Continuous variables were compared using the t-test or Mann-Whitney method. Pearson\'s chi-square test or Fisher\'s exact test was conducted to compare categorical data.
RESULTS: Overall, 282 individuals diagnosed with POEMS syndrome were included in this study, of which 56% were male with an average age of 48.7 years. Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). Chest computed tomography and echocardiography findings showed that 56.4% of patients exhibited pleural effusion, 62.8% displayed mediastinal or hilar lymphadenopathy, 46.5% presented pleural thickening, 27.3% demonstrated bone lesions of the ribs or thoracic vertebra, 7.8% showed lung interstitial abnormalities, and 35.5% had pulmonary hypertension. Decreased diffuse capacity and restrictive ventilatory patterns were identified in 85.2% (115 cases) and 47.4% (64 cases) of patients, respectively. Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities.
CONCLUSIONS: Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem.
摘要：
背景：多发性神经病，器官肿大,内分泌病,单克隆丙种球蛋白病，皮肤改变（POEMS）综合征是一种罕见的多系统克隆浆细胞疾病。肺部受累常见于POEMS综合征患者，表现出各种临床特征。因此,为了提高诊断准确性并提供治疗策略，需要全面分析POEMS综合征的肺部表现.
方法：这项回顾性研究包括北京协和医院POEMS综合征患者，中国主要的转诊医疗中心，2013年6月1日至2023年6月1日。人口统计数据,实验室发现,肺功能检查结果,超声心动图,并提取胸部影像学数据。使用t检验或Mann-Whitney方法比较连续变量。进行Pearson卡方检验或Fisher精确检验以比较分类数据。
结果：总体而言，282名被诊断患有POEMS综合征的人被纳入这项研究，其中56%为男性，平均年龄48.7岁。40.1%的病人出现呼吸道症状,以呼吸困难为最常见症状（34.4%）。胸部计算机断层扫描和超声心动图检查结果显示，56.4%的患者出现胸腔积液，62.8%显示纵隔或肺门淋巴结病，46.5%的患者出现胸膜增厚，27.3%显示肋骨或胸椎的骨病变，7.8%显示肺间质异常，35.5%有肺动脉高压。在85.2％（115例）和47.4％（64例）的患者中，弥散能力和限制性通气模式降低。分别。有呼吸道症状的患者表现出比没有呼吸道症状的患者更高的肺功能下降指标。预后不良的高危患者表现出较多的肺功能异常。
结论：肺部表现异常是POEMS综合征的重要特征。一些POEMS综合征患者在初步评估时出现呼吸道症状。这些发现强调了临床医生早期识别和准确诊断POEMS综合征的重要性，特别是在涉及肺和多系统的情况下。
方法：这项回顾性研究包括北京协和医院POEMS综合征患者，中国主要的转诊医疗中心，2013年6月1日至2023年6月1日。人口统计数据,实验室发现,肺功能检查结果,超声心动图,并提取胸部影像学数据。使用t检验或Mann-Whitney方法比较连续变量。进行Pearson卡方检验或Fisher精确检验以比较分类数据。
结果：总体而言，282名被诊断患有POEMS综合征的人被纳入这项研究，其中56%为男性，平均年龄48.7岁。40.1%的病人出现呼吸道症状,以呼吸困难为最常见症状（34.4%）。胸部计算机断层扫描和超声心动图检查结果显示，56.4%的患者出现胸腔积液，62.8%显示纵隔或肺门淋巴结病，46.5%的患者出现胸膜增厚，27.3%显示肋骨或胸椎的骨病变，7.8%显示肺间质异常，35.5%有肺动脉高压。在85.2％（115例）和47.4％（64例）的患者中，弥散能力和限制性通气模式降低。分别。有呼吸道症状的患者表现出比没有呼吸道症状的患者更高的肺功能下降指标。预后不良的高危患者表现出较多的肺功能异常。
结论：肺部表现异常是POEMS综合征的重要特征。一些POEMS综合征患者在初步评估时出现呼吸道症状。这些发现强调了临床医生早期识别和准确诊断POEMS综合征的重要性，特别是在涉及肺和多系统的情况下。
