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.     

Persistent pulmonary interstitial emphysema in an unventilated neonate

Persistent pulmonary interstitial emphysema (PPIE) is a chronic form of pulmonary interstitial emphysema. The disease is histologically distinguished by large cysts and giant cells. Our patient was a female twin who was born at 31 weeks of gestation with a birth weight of 1,450 g. A chest X-ray at 2 hr after delivery was normal. At 12 hr, respiratory distress developed, and nasal continuous positive airway pressure (CPAP) was initiated. A chest film revealed left-sided pneumothorax. A chest tube was inserted, and the baby continued on nasal CPAP for 5 days. Her chest X-ray on postnatal day 4 showed diffuse cystic changes in the left lung. Thoracic computed tomography revealed multiple thick-walled cysts, the largest measuring 3 cm in diameter. Our case confirms that localized PIE may occur in preterm infants who have been treated with nasal CPAP only. Since this method is being used increasingly to avoid mechanical ventilation and in the postextubation period, it is very important that clinicians be aware of its complications.     

皮肌炎合并纵隔、皮下气肿临床分析

目的:总结皮肌炎(DM)合并纵隔、皮下气肿的临床相关因素,提高对该并发症的认识。方法:回顾性分析7例皮肌炎患者并发纵隔、皮下气肿的临床特征、治疗及转归。结果:7例患者均存在不同程度的肺间质病变,较明显的皮肤血管炎表现,均合并感染;血肌酸激酶(CK)平均(88±30)U/L,而乳酸脱氢酶(LDH)平均(482±128)U/L、羟丁酸脱氢酶(HBDH)平均(417±164)U/L;2例男性、1例女性死亡,4例存活;均未行大剂量激素冲击治疗,均联合环孢素A或他克莫司等免疫抑制剂治疗。结论:DM患者中肺间质病变和皮肤血管炎可能是并发纵膈、皮下气肿的高危因素,感染亦是引发因素;男性患者较多,Gottron疹、甲周红斑、指端溃疡较突出,肌炎较轻,而LDH、HBDH升高;不推荐大剂量激素冲击治疗,联合钙调磷酸酶抑制剂(CNIs),预后较好。     

老年慢性阻塞性肺气肿并发自发性气胸52例分析

目的 探讨老年慢性阻塞性肺气肿并发自发性气胸的特点。方法 对1993—2002年我院收治的老年慢性阻塞性肺气肿并发自发性气胸52例的临床资料进行回顾性分析。结果 治愈45例(86．5％)．死亡5例(9．6％)。结论 老年慢阻肺并发自发性气胸起病隐匿．进展快．常危及生命，及时诊断和治疗．可降低死亡率。     

Clinical characteristics of combined pulmonary fibrosis and emphysema

Background and objective: Patients with combined pulmonary fibrosis and emphysema (CPFE) are sometimes seen, and we speculate that these patients have some different clinical characteristics from COPD patients. This study clarifies the clinical characteristics of CPFE patients. Methods: This was a retrospective study of 47 stable patients with concurrent emphysema and diffuse parenchymal lung disease with fibrosis, based on the findings of chest CT (CPFE patients). The clinical characteristics of CPFE patients were compared with those of emphysema‐dominant COPD patients without parenchymal lung disease (COPD patients). Results: Forty‐six of the 47 CPFE patients were male. Paraseptal emphysema was particularly common in the CPFE group. Honeycombing, ground‐glass opacities and reticular opacities were present in 75.6%, 62.2% and 84.4% of CPFE patients, respectively. Twenty‐two of the 47 CPFE patients (46.8%) had lung cancer. Pulmonary function tests showed that the CPFE group had milder airflow limitation and lower diffusing capacity than the COPD group. Desaturation during 6‐min walking test in CPFE patients tended to be more severe than in COPD patients, if the level of FEV₁ or 6MWD was equal. Conclusions: CPFE patients had some different clinical characteristics in comparison with COPD patients and may also have a high prevalence of lung cancer.     

肺间质纤维化合并肺气肿患者吸气功能训练的疗效观察

目的探讨吸气功能训练对肺间质纤维化合并肺气肿患者肺功能的影响。方法70例肺间质纤维化合并肺气肿患者随机分为治疗组（30例）和对照组（40例）,治疗组采用反馈式肺功能锻炼器训练3月;对照组仅接受肺功能康复宣教,自行锻炼。观察2组患者肺活量、气急气短分级以及肺功能改善情况。结果通过3月治疗后,在自觉气急气短、肺活量、第1秒用力呼气容积占肺活量比值（FEVl／FVC）及动脉血氧浓度方面,治疗组明显好于同时期的对照组,差异有统计学意义（P〈0．05）。结论吸气训练有助于肺间质纤维化合并肺气肿的患者肺功能的恢复。     

Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.     

Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone

Chronic obstructive pulmonary disease (COPD) and idiopathic interstitial pneumonias (IIP), with different radiological, pathological, functional and prognostic characteristics, have been regarded as separate entities for a long time. However, there is an increasing recognition of the coexistence of emphysema and pulmonary fibrosis in individuals. The association was first described as a syndrome by Cottin in 2005, named “combined pulmonary fibrosis and emphysema (CPFE)”, which is characterized by exertional dyspnea, upper-lobe emphysema and lower-lobe fibrosis, preserved lung volume and severely diminished capacity of gas exchange. CPFE is frequently complicated by pulmonary hypertension, acute lung injury and lung cancer and prognosis of it is poor. Treatments for CPFE patients with severe pulmonary hypertension are less effective other than lung transplantation. However, CPFE has not yet attracted wide attention of clinicians and there is no research systematically contrasting the differences among CPFE, emphysema/COPD and IIP at the same time. The authors will review the existing knowledge of CPFE and compare them to either entity alone for the first time, with the purpose of improving the awareness of this syndrome and exploring novel effective therapeutic strategies in clinical practice.     

The prevalence of pulmonary fibrosis combined with emphysema in patients with lung cancer

Background and objective: Combined pulmonary fibrosis and emphysema (CPFE) is a unique disorder of the upper lobe, whereas emphysema is usually associated with lower lobe fibrosis. Although CPFE might increase the risk of lung cancer, the prevalence of CPFE in patients with lung cancer and the incidence of lung cancer in patients with CPFE are unknown. The objective of this study was to determine the prevalence of CPFE in lung cancer patients and to assess the clinical features of these patients. Methods: A total of 1143 patients with lung cancer were reviewed. Based on HRCT performed at diagnosis of lung cancer, patients were categorized into four groups: normal, emphysema, fibrosis and CPFE. The clinical characteristics of patients with CPFE were compared with those of the other groups. Results: CPFE, emphysema and fibrosis were identified in 101 (8.9%), 404 (35.3%) and 15 (1.3%) patients with lung cancer, respectively. The median overall survival of CPFE patients (n = 101, 10.8 months) was significantly less than that of normal patients (n = 623, 53.0 months) or that of patients with emphysema alone (n = 404, 21.9 months). Acute lung injury occurred in 20 (19.8%) patients with CPFE. Conclusions: CPFE is more prevalent than fibrosis in patients with lung cancer, and patients with CPFE had a poorer prognosis in the present study. Further investigation is therefore necessary to elucidate whether CPFE is an independent risk factor for lung cancer.     

肺纤维化合并肺气肿的临床研究进展

特发性肺纤维化（IPF）和肺气肿在影像学、病理生理、治疗及预后等方面各不相同,是两种疾病。然而,部分患者影像学表现同时存在肺纤维化和肺气肿,被称为肺纤维化合并肺气肿（CPFE）。CPFE的表现与单纯的肺气肿或IPF不同,因此被认为是一种独立的疾病,且越来越受到关注。本文对CPFE的病因与发病机制、临床表现、诊断、并发症、治疗和预后等方面进行了综述。     

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...     

肺纤维化合并肺气肿

吸烟是肺气肿的重要危险因素,同时也与多种间质性肺病的发病密切相关.一部分吸烟者肺部影像可以同时存在下肺野纤维化和上肺野肺气肿,目前认为这种临床特征是一种独立的疾病,称作肺纤维化合并肺气肿.这部分患者肺功能表现为肺容积相对正常而弥散能力屁著下降,而且肺动脉高压发生率较高.但该病的发病机制不清楚,有效的治疗策略和预后判断有待进一步研究.     

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.

     

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.