两种遗传性结缔组织病与哮喘和气道塌陷
2007/08/01
目的是研究临床上的一种印象，在过度移动综合症（hypermobility syndrome，HMS）/良性关节过度移动综合症 (BJHS)和Ehlers Danlos综合症 (EDS)中，呼吸道疾病的发生率较正常人群高。509例受实者给予问卷调查，为221例健康对照，126例HMS和162例EDS，问卷的内容包括呼吸道症状，呼吸疾病和变态反应病史。其中157例给予临床和血清学检查，57例给予生理学检查。结果显示，和健康组比较，HMS和EDS人群中呼吸系统症状的表现形式和发生的频率明显增加，而运动耐量明显减少。尤其是哮喘和变态反应的发生率升高，临床的检查也证实了这一点。肺生理学检查揭示肺容积增加，气体交互功能受损和上下气道易于塌陷。
作者认为这是首次证实HMS/ BJHS和EDS患者表现为肺生理功能受损的呼吸系统症状。其哮喘的发病率升高可能是由于结缔组织功能受损或相关基因的连锁不平衡。在无哮喘的HMS/ BJHS和EDS患者中，支气管和肺间质的力学变化可能是气道易于塌陷的原因。
（韩福军 广州医学院第一附属医院 广州呼吸疾病研究所 510120 摘译）
(Ann Rheum Dis. 2007 Apr 5)
Morgan AW, Pearson SB, Davies S, Gooi HC, Bird HA.
Asthma and airways collapse in two heritable disorders of connective tissue.
Ann Rheum Dis. 2007 Apr 5
OBJECTIVES: This study investigated the clinical impression that there was an increased prevalence of respiratory disorders in both the hypermobility syndrome (HMS)/ benign joint hypermobility syndrome (BJHS) and Ehlers Danlos Syndrome (EDS), compared to the normal population.
METHODS: A questionnaire was distributed to 509 subjects (221 healthy controls, 126 HMS, 162 EDS) that documented respiratory symptoms and previously diagnosed respiratory and atopic disorders. A subgroup of 157 responders underwent full clinical and serological assessments and 57 subjects were assessed physiologically.
RESULTS: A significant increase in the frequency of a wide range of respiratory symptoms and reduced exercise tolerance was observed in subjects with both HMS and EDS compared to controls. In particular, there was an increased prevalence of asthmatic symptoms (HMS: OR 2.4, 95% CI 1.4-4.1, P=0.002; EDS: OR 3.1, 95% CI 1.8-5.2, P<0.001) and atopy (HMS: OR 2.7, 95% CI 1.6-4.5, P<0.001; EDS: OR 2.6, 95% CI 1.6-4.4, P<0.001), which was subsequently confirmed by clinical assessment. Pulmonary physiological studies revealed increased lung volumes, impaired gas exchange and an increased tendency of both the lower and upper airways to collapse.
CONCLUSIONS: We have demonstrated, for the first time, that individuals with HMS/ BJHS and EDS have respiratory symptoms in association with various pulmonary physiological abnormalities. The increased prevalence of asthma may be due to linkage disequilibrium between the genes causing these conditions or a function of the connective tissue defect itself. In the non-asthmatic population, changes in the mechanical properties of the bronchial airways and lung parenchyma may underlie the observed increased tendency of the airways to collapse.
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