Pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis with cryptogenic organizing pneumonia in invasive breast cancer: a case report and review of literature

Amyopathic dermatomyositis (ADM) is recognized as a variant phenotype of dermatomyositis and characterized by typical skin manifestations without evidence of muscular inflammation. While interstitial lung disease (ILD) is occasionally found as one of the lung manifestations in ADM patients, the development of a pneumomediastinum and/or subcutaneous emphysema in this disease entity is one of the extremely rare pulmonary complications. These latter complicated pulmonary manifestations have been usually reported in idiopathic ADM with ILD without any other associated medical conditions. We report a case presented with the spontaneous pneumomediastinum and subcutaneous emphysema in both ADM and cryptogenic organizing pneumonia during adjuvant chemotherapy based on cyclophosphamide for breast cancer.     

Management of life-threatening subcutaneous emphysema using subcutaneous penrose drains and colostomy bags

Subcutaneous emphysema is a common complication post cardiothoracic surgery. Severe subcutaneous emphysema may cause respiratory obstruction and sometimes tracheostomy or intubation is required. We report a case of massive subcutaneous emphysema following aortic valve replacement. It was not relieved with initial bilateral chest tubes, but the subcutaneous Penrose drains produced a dramatic improvement and provided effective decompression of the subcutaneous emphysema. We also describe the colostomy bags, which covered the drains. These were useful for keeping the area sterile and measuring the amount of air through the individual drains.     

肺气肿的CT肺功能成像:CT技术与肺功能检查的对照研究

目的　比较高分辨CT(HRCT)、CT肺功能成像及肺功能检查 (PFT)对肺气肿及阻塞性肺疾病 (COPD)的诊断效用。方法　阻塞性通气障碍组 37例 ,正常对照组 2 0名。所有入选者均在 1周内完成CT检查和PFT。比较HRCT视觉评分、CT肺功能成像参数与PFT结果的相关性和差异性。结果　 (1)CT检查发现 ,阻塞性通气障碍组 37例中有 2 3例有肺气肿征象归属肺气肿亚组。 (2 )HRCT视觉评估仅与PFT参数中一氧化碳弥散量 (DLCO)占预计值百分比存在相关性 (r =- 0 731,P <0 0 0 1)。 (3)肺气肿亚组的视觉分数与最大吸气末、呼气末各像素指数 (PI)存在相关性 ,其中分别与PI 950in、PI 950ex相关性最好 (r分别 =0 5 0 6、0 6 10 ,P均 <0 0 0 1)。 (4)利用PI 910in、PI 910ex分别对两组的肺功能进行分级 ,与一秒钟用力呼气容积 /用力肺活量 (FEV1/FVC)比值的分级结果比较 ,差异无显著性 (χ2 分别 =0 5 2 7、3 6 4 5 ,P分别 =0 913、0 30 2 ) ,采用PI 910in对肺气肿亚组分级与FEV1/FVC的分级结果比较 ,差异无显著性 (χ2 =2 5 9,P =0 4 5 9)。结论　HRCT诊断肺气肿较普通CT敏感 ,但受层厚和照射剂量的限制 ,不能用于对全肺的评估。CT肺功能成像结果客观 ,操作简单 ,与HRCT视觉评分及PFT结果相关性好 ,对早期肺气肿的诊     

Bilateral pneumothorax and mediastinal emphysema in systemic lupus erythematosus

Summary The association of pneumothorax and mediastinal emphysema in systemic lupus erythematosus (SLE) has not been described extensively in the literature. We describe a 36 year-old man with SLE, complicated by bilateral pneumothorax, mediastinal emphysema and pneumoperitoneum. Despite the treatment received, he died of respiratory failure.     

Severe subcutaneous generalized edema in a patient with dermatomyositis

Abstract

Subcutaneous generalized edema associated with dermatomyositis (DM)/polymyositis (PM) is extremely rare. Herein we report a case of severe subcutaneous generalized edema complicating DM. A 78-year-old woman was hospitalized in our department because of massive edema in the four limbs. Elevated muscle enzymes, heliotrope rash, results of electromyography, and muscle biopsy confirmed the diagnosis of DM. The absence of other diseases that could cause the symptoms indicated that massive edema was correlated with the pathophysiology of DM. Although myopathy and edema responded well to oral prednisolone, dysphagia persisted. We conclude that subcutaneous generalized edema can occur during the course of DM/PM, and subcutaneous vasculopathy may be involved in the pathogenesis of DM/PM.     

Severe subcutaneous generalized edema in a patient with dermatomyositis

Subcutaneous generalized edema associated with dermatomyositis (DM)/polymyositis (PM) is extremely rare. Herein we report a case of severe subcutaneous generalized edema complicating DM. A 78-year-old woman was hospitalized in our department because of massive edema in the four limbs. Elevated muscle enzymes, heliotrope rash, results of electromyography, and muscle biopsy confirmed the diagnosis of DM. The absence of other diseases that could cause the symptoms indicated that massive edema was correlated with the pathophysiology of DM. Although myopathy and edema responded well to oral prednisolone, dysphagia persisted. We conclude that subcutaneous generalized edema can occur during the course of DM/PM, and subcutaneous vasculopathy may be involved in the pathogenesis of DM/PM.     

肺纤维化合并肺气肿患者的肺功能特征

目的 了解肺纤维化合并肺气肿（combined pulmonary fibrosis and emphysema,CPFE）患者肺功能特点,及是否能通过肺功能将CPFE、单纯肺气肿、未合并肺气肿的特发性肺纤维化（idiopathic pulmonary fibrosis,IPF）进行区分.方法 回顾性分析了48例CPFE患者利用肺量计及脉冲振荡法测量的肺功能参数,并与单纯肺气肿患者（50例）、未合并肺气肿的IPF患者（46例）的肺功能进行了比较.结果 肺量计参数显示：CPFE组肺总量（TLC）％ pred正常[（86.9±17.8）％],而未合并肺气肿的IPF组TLC％ pred明显降低[（70.4±17.0）％],CPFE组的FEV1/FVC大致正常[（72.0±9.4）％],而单纯肺气肿组明显降低[（56.5±14.4）％],CPFE组、单纯肺气肿组及未合并肺气肿的IPF组的弥散功能均降低,分别为（57.2±17.3）％、（62.8±25.7）％、（66.9±21.5）％.脉冲振荡法测量的参数显示：CPFE组与未合并肺气肿的IPF组总气道阻力（R5）正常,而单纯肺气肿组明显增高[（161.3±69.0）％],虽然CPFE组、单纯肺气肿组及未合并肺气肿的IPF组外周气道阻力（R5-R20）均增加,但单纯肺气肿组较CPFE组和未合并肺气肿的IPF组增加更为明显（P=0.006,P=0.013）,三组X5轻度减低,单纯肺气肿组较CPFE组和IPF组减低更为明显（P=0.004,P=0.047）.相关分析显示：CPFE组、单纯肺气肿组R5-R20和RV/TLC呈正相关（r=0.44,P=0.01;r=0.66,P=0.01）;CPFE组、单纯肺气肿组K与RV/TLC呈负相关（r=-0.51,P=0.01; r=-0.66,P=0.01）,未合并肺气肿的IPF组TLC％ pred与R5-R20和X5有相关性（r=-0.50,P=0.01; r=0.6,P=0.01）.结论 CPFE的肺功能特点为肺容积正常,通气功能正常,弥散功能明显降低,总气道阻力及中心气道阻力正常,周边气道阻力增加和弹性阻力增高,肺的顺应性降低.     

Sequential occurrence of combined pulmonary fibrosis and emphysema syndrome in a non‐smoker female patient

The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under‐recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non‐smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre‐existent interstitial fibrosis, female gender and absence of a history of smoking.     

肺纤维化合并肺气肿肺功能及高分辨CT的特征

目的 探讨肺纤维化合并肺气肿(CPFE)患者临床症状、体征、肺功能和高分辨CT(HRCT)的特征;分析肺纤维化和肺气肿的程度与肺功能之间的关系.方法 前瞻性、随机临床病例对照研究.CPFE患者40例,COPD患者80例.比较两组患者一般特征及肺功能等,探讨CPFE肺纤维化和肺气肿的程度与肺功能之间的相关性.结果 40例CPFE患者,年龄(71.8±8.1)岁,男33例,有吸烟史者36例.病程6个月至15年.临床主要症状为咳嗽(34例)、呼吸困难(32例)及双下肺部爆裂音(23例).与COPD患者比较,CPFE患者PaO2降低(f=-2.016,P＜0.05).与COPD患者比较,CPFE患者FEV1％pred[(72.7±20.0)％vs (53.8±15.6)％,t=5.687,P＜0.01]和FEV1/FVC[(70.4±15.8)％vs (54.3±10.7)％,t=6.590,P＜0.01]增高,RV％ pred[(106.8±51.8)％vs(177.6±72.9)％,t=-5.484,P＜0.01]、TLC％pred[(90.1±22.5)％vs (135.4±86.2)％,t=-3.228,P＜0.01]和DL CO％ pred[(43.6±19.4)％vs (63.5±16.1)％,t=-5.900,P＜0.01]降低;VC％ pred和FVC％ pred两组之间比较差异无统计学意义(t值分别为-0.876、0.450,P值均＞0.05).CPFE患者胸部HRCT的主要表现为同时存在以双上肺野为主的肺气肿和双下肺野为主的肺间质改变.肺气肿表现为小叶中心型肺气肿(87.5％)、旁间隔型肺气肿(27.5％)、全小叶型肺气肿(10.0％)和肺大疱(35.0％).肺间质改变为网格影97.5％,磨玻璃影55.0％,蜂窝肺37.5％.调整性别、年龄和吸烟混杂因素后,肺纤维化程度与DL CO％ pred相关(P＜0.01),肺气肿严重程度与DLCO％ pred和FEV1/FVC相关(P＜0.05).结论 CPFE多发生在男性吸烟者,肺通气功能受损较轻,而弥散功能显著下降.HRCT是CPFE诊断的主要依据.HRCT显示肺纤维化和肺气肿严重者肺弥散功能更差.     

肺纤维化合并肺气肿综合征肺功能及与高分辨率CT定量相关性分析

目的:分析肺纤维化合并肺气肿(CPFE)综合征患者肺功能及与高分辨率CT(HRCT)定量相关性。方法:本研究为病例对照研究,采用非随机对照的方法收集2011年10月至2021年10月于兰州大学第一医院住院治疗的CPFE综合征患者239例和同期收治的特发性肺纤维化(IPF)患者294例及肺气肿患者287例,根据肺部纤维化...     

多层螺旋CT肺密度测定与肺气肿患者肺功能的相关性研究

目的探讨多层螺旋CT肺密度测定与肺气肿患者肺功能的相互关系。方法对45例肺气肿患者及40例对照组人员的CT肺密度和肺功能进行测定,并将CT肺密度测定结果与肺功能结果进行对照研究。结果肺气肿患者CT呼、吸气相VD与TLC、FEV1及FEV1/FVC呈显著正相关（P〈0.01）,而与RV/TLC呈显著负相关（P〈0.01）。吸气相全肺MLD与PFT指标无相关性（P〉0.05）。结论对于肺气肿疾病多层螺旋CT肺密度测定与肺功能检测有着密切的关系。     

Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema

Background and objective

This study evaluated whether patients with combined pulmonary fibrosis and emphysema (CPFE) have an increased likelihood of pulmonary hypertension (PHT) when compared with idiopathic pulmonary fibrosis (IPF) patients without emphysema.

Methods

Two consecutive IPF populations having undergone transthoracic echocardiography were examined (n = 223 and n = 162). Emphysema and interstitial lung disease (ILD) extent were quantified visually; ILD extent was also quantified by a software tool, CALIPER. Echocardiographic criteria categorized PHT risk.

Results

The prevalence of an increased PHT likelihood was 29% and 31% in each CPFE cohort. Survival at 12 months was 60% across both CPFE cohorts with no significantly worsened outcome identified when compared with IPF patients without emphysema. Using logistic regression models in both cohorts, total computed tomography (CT) disease extent (ILD and emphysema) predicted the likelihood of PHT. After adjustment for total disease extent, CPFE had no stronger association with PHT likelihood than IPF patients without emphysema.

Conclusion

Our findings indicate that the reported association between CPFE and PHT is explained by the summed baseline CT extents of ILD and emphysema. Once baseline severity is taken into account, CPFE is not selectively associated with a malignant microvascular phenotype, when compared with IPF patients without emphysema.

     

Hemodynamics in dogs with pulmonary hypertension due to emphysema

We examined hemodynamic parameters in a canine model of pulmonary emphysema in which the lesion was severe enough to increase transmural mean pulmonary artery pressure ( ) to about 10 mmHg higher than a control group of dogs. Pulmonary emphysema was produced in 6 dogs (Group E) by the repeated weekly instillations of the enzyme papain administered into their lobar bronchi. In 5 control dogs (Group C), a normal saline solution was used. Pulmonary hemodynamics and gas exchange measurements were obtained after 7 doses of the enzyme were administered when, relative to the prepapain value, total lung capacity had increased by 40% in Group E. Hemodynamic measurements were performed before and after plasma volume expansion to elevate the mean transmural wedge pressure ( ) to approximately 15 mmHg. At similar cardiac outputs and , in Group E were higher than in Group C in both the pre (mean ±SD; 7.3 ± 1.4 mmHg vs 18.5 ± 5.5 mmHg,P < 0.01) and volume-loaded (21.7 ± 2.2 vs 27.9 ± 4.7 mmHg,P < 0.01) conditions. Compared with Group C, arterial blood oxygen tension and diffusing capacity for carbon monoxide were reduced in Group E. Left ventricular performance was normal in Group E, whereas right ventricular performance appeared slightly depressed. The latter finding was explained in terms of a relatively increased right ventricular afterload in Group E, which impeded right ventricular ejection.     

Subcutaneous cervical emphysema and pneumomediastinum due to a lower gastrointestinal tract perforation

This case report describes a 69-year-old man presenting with an extensive subcutaneous emphysema in his neck and generalized peritonitis caused by a lower gastrointestinal tract perforation. This case emphasizes that subcutaneous emphysema patients with negative thoracic findings should be scrutinized for signs of retroperitoneal hollow viscus perforation.     

Spontaneous pulmonary interstitial emphysema in a term unventilated infant

Pulmonary interstitial emphysema (PIE) is a form of air block most frequently seen in ventilated preterm infants with severe lung disease; it is rarely reported in spontaneously breathing term infants. We report on an infant previously diagnosed with laryngomalacia and congestive heart failure and with evidence of antenatal stroke before the onset of pulmonary disease. He presented at 6 weeks of age with spontaneous pneumothorax. Focal cystic changes were seen on imaging studies of the lungs. There was no prior history of mechanical ventilation. Prior chest X-rays did not show cystic changes. He subsequently underwent resection of the affected lung areas. Pathologic examination revealed persistent PIE with cystic expansion, pleural blebs, and reactive pleuritis, as well as subpleural air-space enlargement. The patient did well postoperatively and was discharged home without further problems. This case demonstrates that PIE can occur in an infant without any history of mechanical ventilation, suggesting the need for a high index of suspicion for PIE, even in nonventilated and spontaneously breathing term neonates. PIE should be included in the differential diagnosis of cystic lung lesions in all young infants.     

The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis

Background and objective: In this study the prevalence, lung function and prognosis of IPF combined with emphysema were evaluated. Methods: Consecutive patients with usual interstitial pneumonia (UIP) on high‐resolution computed tomography (HRCT), with or without emphysema, were assessed retrospectively. The area of fibrosis in the base of the lungs was assessed by HRCT as minimal (<2 cm from the subpleura), moderate (≥2 cm from the subpleura, <1/3 of the area of the base of the lungs) or severe (≥1/3 of the area of the base of the lungs). Results: Among 660 patients with UIP on HRCT, 221 showed upper‐lobe emphysema. Pulmonary function results for patients with UIP and UIP/emphysema, respectively, were: FVC, 71.8% and 87.1%; FEV1%, 86.7% and 87.9%; and DL_CO, 74.3% and 65.2% of predicted. The relationship between FVC, the extent of fibrosis and survival was investigated in 362 patients with records of pulmonary function tests and no lung cancer at the time of entry into the study. Although the extent of fibrosis was similar between the groups, 71.3% of UIP patients met the lung volume criteria for IPF (FVC <80% of predicted), whereas only 26.5% of UIP/emphysema patients met the lung volume criteria for IPF. Median survival was 7.5 years in the UIP group and 8.5 years in the UIP/emphysema group. Conclusions: Emphysema was a common finding in patients with UIP. Patients with UIP and emphysema had greater lung volumes and better survival compared with those with UIP alone.