Abstract：
BACKGROUND: Characteristics, practice patterns, and clinical outcomes of secondary spontaneous pneumothorax (SSP) with heritable connective tissue disorders (Marfan, Ehlers-Danlos, and Birt-Hogg-Dubé syndromes) are unclear.
METHODS: A nationwide Japanese inpatient database, which included data on 524 patients with SSP (884 hospitalizations) and 137821 with primary spontaneous pneumothorax (PSP) between July 2010 and March 2020, was used in this study. Hospitalization for SSP (n=884) was categorized into surgical (n=459) and non-surgical (n=425) groups, and the patient characteristics, treatment, and outcomes were compared between the groups. Multivariable analyses were performed to evaluate risk factors for pneumothorax recurrence. We also compared the patient characteristics among those with different underlying heritable connective tissue disorders.
RESULTS: Compared to the non-surgical group, the surgical group had less frequent readmission for pneumothorax (26% vs. 44%; hazard ratio, 0.47; 95% confidence interval, 0.38-0.58). Young patients (hazard ratio, 2.46; 95% confidence interval, 1.83-3.32) or those with Birt-Hogg-Dubé syndrome (2.53; 1.77-3.63) had a high risk of recurrence. Pneumothorax occurred frequently in teenagers with Marfan syndrome, in those aged 20-39 years with Ehlers-Danlos syndrome, and in those aged ≥40 years with Birt-Hogg-Dubé syndrome.
CONCLUSIONS: Detailed information on the characteristics and clinical course of SSP in heritable connective tissue disorders will aid the clinical decision-making process.
METHODS: A nationwide Japanese inpatient database, which included data on 524 patients with SSP (884 hospitalizations) and 137821 with primary spontaneous pneumothorax (PSP) between July 2010 and March 2020, was used in this study. Hospitalization for SSP (n=884) was categorized into surgical (n=459) and non-surgical (n=425) groups, and the patient characteristics, treatment, and outcomes were compared between the groups. Multivariable analyses were performed to evaluate risk factors for pneumothorax recurrence. We also compared the patient characteristics among those with different underlying heritable connective tissue disorders.
RESULTS: Compared to the non-surgical group, the surgical group had less frequent readmission for pneumothorax (26% vs. 44%; hazard ratio, 0.47; 95% confidence interval, 0.38-0.58). Young patients (hazard ratio, 2.46; 95% confidence interval, 1.83-3.32) or those with Birt-Hogg-Dubé syndrome (2.53; 1.77-3.63) had a high risk of recurrence. Pneumothorax occurred frequently in teenagers with Marfan syndrome, in those aged 20-39 years with Ehlers-Danlos syndrome, and in those aged ≥40 years with Birt-Hogg-Dubé syndrome.
CONCLUSIONS: Detailed information on the characteristics and clinical course of SSP in heritable connective tissue disorders will aid the clinical decision-making process.
摘要：
背景：特征，实践模式，继发性自发性气胸（SSP）伴遗传性结缔组织疾病的临床结局（Marfan，Ehlers-Danlos,和Birt-Hogg-Dubé综合征）尚不清楚。
方法：日本全国住院患者数据库，本研究使用了2010年7月至2020年3月524例SSP患者(884例住院)和137821例原发性自发性气胸(PSP)患者的数据.SSP住院（n=884）分为手术组（n=459）和非手术组（n=425），和病人的特征，治疗,并比较两组间的结局.采用多变量分析评价气胸复发的危险因素。我们还比较了患有不同潜在遗传性结缔组织疾病的患者的患者特征。
结果：与非手术组相比，手术组气胸的再入院频率较低(26%vs.44%；危险比，0.47；95%置信区间，0.38-0.58）。年轻患者(危险比，2.46；95%置信区间，1.83-3.32）或Birt-Hogg-Dubé综合征（2.53;1.77-3.63）的复发风险很高。气胸多发于青少年马凡氏综合征,在20-39岁的Ehlers-Danlos综合征患者中，以及年龄≥40岁的Birt-Hogg-Dubé综合征患者。
结论：关于遗传性结缔组织疾病中SSP的特征和临床过程的详细信息将有助于临床决策过程。
方法：日本全国住院患者数据库，本研究使用了2010年7月至2020年3月524例SSP患者(884例住院)和137821例原发性自发性气胸(PSP)患者的数据.SSP住院（n=884）分为手术组（n=459）和非手术组（n=425），和病人的特征，治疗,并比较两组间的结局.采用多变量分析评价气胸复发的危险因素。我们还比较了患有不同潜在遗传性结缔组织疾病的患者的患者特征。
结果：与非手术组相比，手术组气胸的再入院频率较低(26%vs.44%；危险比，0.47；95%置信区间，0.38-0.58）。年轻患者(危险比，2.46；95%置信区间，1.83-3.32）或Birt-Hogg-Dubé综合征（2.53;1.77-3.63）的复发风险很高。气胸多发于青少年马凡氏综合征,在20-39岁的Ehlers-Danlos综合征患者中，以及年龄≥40岁的Birt-Hogg-Dubé综合征患者。
结论：关于遗传性结缔组织疾病中SSP的特征和临床过程的详细信息将有助于临床决策过程。
