Diagnostic approach to pulmonary lymphangioleiomyomatosis

Abstract. Pulmonary lymphangioleiomyomatosis is an interstitial lung disease with distinctive clinical, radio-logic, physiological and pathological findings. We report an additional case of this rare condition, diagnosed on first medical evaluation by trans-bronchial lung biopsy and discuss the diagnostic approach.     

Extrapulmonary presentation of asymptomatic pulmonary lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare parenchymal lung disease, which affects young women of childbearing age and is characterized pathologically by proliferation of interstitial smooth muscle and formation of cysts in the lung. While LAM is usually predominantly a respiratory disorder, it can also initially involve other extrapulmonary organs. We report the case of a 35-year-old Chinese woman, who presented with a 4-week history of left thigh swelling which was found to be secondary to compression of pelvic veins by a mass lesion. The latter was found histologically to show LAM. Despite the patient being asymptomatic and displaying normal lung function, a thoracic high resolution CT scan showed typical features of early LAM. This case further illustrates that LAM can have multisystem involvement, and demonstrates the importance of being aware of the diagnosis in cases presenting with extrapulmonary manifestation, in order that patients are diagnosed and managed appropriately.     

Quantitative assessment of pulmonary function in lymphangioleiomyomatosis patients using high-resolution computed tomography and pulmonary function tests

BackgroundTo explore the feasibility of using quantitative high-resolution computed tomography (HRCT) to evaluate pulmonary function in patients with pulmonary lymphangioleiomyomatosis (PLAM).MethodsPulmonary function tests (PFTs) were performed in 30 patients with pathologically confirmed PLAM with the use of HRCT. These results were correlated with quantitative HRCT in 21 patients.ResultsThere were significant correlations between the HRCT parameters for lung function and PFT parameters. Among these parameters, emphysema volume (EV), pulmonary volume with a pixel index less than the trigger threshold (−950 HU) to account for a proportion of total lung volume [PI-950 (%)] and forced expiratory volume in 1 second/forced vital capacity [FEV1/FVC (%)] had the strongest correlations, reaching values between −0.71 and −0.68. HRCT lung function might therefore also be helpful for predicting changes in lung function before and after treatment.ConclusionsHRCT is helpful for the assessment of pulmonary function in PLAM patients and can assist in the clinical evaluation of lung function and treatment response in patients with this disease.     

When and how to treat pulmonary non-tuberculous mycobacterial diseases

Non-tuberculous mycobacteria are ubiquitous environmental organisms that have been recognized as a cause of pulmonary infection for over 50 years. Traditionally patients have had underlying risk factors for development of disease; however, the proportion of apparently immunocompetent patients involved appears to be rising. Not all patients culture-positive for mycobacteria will have progressive disease, making the diagnosis difficult, though criteria to aid in this process are available. The two main forms of disease are cavitary disease (usually involving the upper lobes) and fibronodular bronchiectasis (predominantly middle and lingular lobes). For patients with disease, combination antibiotic therapy for 12–24 months is generally required for successful treatment, and this may be accompanied by drug intolerances and side-effects. Published success rates range from 30% to 82%. As the progression of disease is variable, for some patients, attention to pulmonary hygiene and underlying diseases without immediate antimycobacterial therapy may be more appropriate. Surgery can be a useful adjunct, though is associated with risks. Randomized controlled trials in well-described patients would provide stronger evidence-based data to guide therapy of non-tuberculous mycobacteria lung diseases, and thus are much needed.     

肺隐球菌病的CT影像表现

目的探讨肺隐球菌病(pulmonary cryptococcosis,PC)的CT影像表现,加强对本病认识,提高诊断水平,减少误诊。方法搜集本院2008至2015年间经病理确诊的26例PC患者的CT影像表现进行分析。结果 26例PC患者中,男14例,女12例,年龄31-65岁,中位年龄48岁。CT表现形态多样:(1)孤立结节或肿块型7例;(2)多发结节或肿块型7例;(3)实变型1例;(4)弥漫混合型11例。所有结节或实变均位于肺外带,呈聚集分布,结节或肿块边缘不规则,所有病例未见钙化,有20例病灶内见支气管充气征,11例出现厚壁空洞,11例呈宽基底状紧贴胸膜,4例周边出现"晕征",2例小结节出现"胸膜凹陷征"。增强扫描:3例出现不均一强化,内见散在低密度坏死灶;1例孤立结节呈周边强化,中间密度稍低;2例实性结节呈均一强化。结论 PC的CT表现形态多样,病灶位于肺外带,呈聚集分布,病变内部多见支气管充气征,呈宽基底向外紧贴胸膜,厚壁空洞。     

Radiological and pulmonary function outcomes of children with SARS

We examined the radiological and pulmonary function outcomes of children affected with severe acute respiratory syndrome (SARS) at 6 months from diagnosis. Twenty-one female and 26 male Chinese patients (median age, 13.6 years; interquartile range, 9.9-16.0) were studied. In each subject, high-resolution computed tomography (HRCT) of the thorax and pulmonary function were assessed. All children were asymptomatic and had a normal clinical examination. Mild pulmonary abnormalities were detected on HRCT in 16 (34.0%) subjects, including residual ground-glass opacification (n = 5), air trapping (n = 8), and a combination of ground-glass changes and air trapping (n = 3). The need for oxygen supplementation (P = 0.02) and lymphopenia during the course of illness (P = 0.012) were significant risk factors in predicting abnormal HRCT. There were no significant lung function differences between those with and without HRCT abnormalities. Despite complete clinical resolution, a considerable proportion of children affected with SARS had abnormal HRCT findings at 6 months. These abnormalities were more prevalent in those with severe disease. It is important that careful follow-up be carried out to assess the clinical significance and persistence of such abnormalities.     

Association between pulmonary artery to aorta diameter ratio with pulmonary hypertension and outcomes in diffuse cystic lung diseases

To investigate the importance of pulmonary vascular measurements on computed tomography (CT) in predicting pulmonary hypertension (PH) and worse outcomes in diffuse cystic lung diseases (DCLDs).We conducted a cross-sectional study of patients with DCLDs. Patients underwent pulmonary function tests, a six-minute walk test (6MWT), chest CT, transthoracic echocardiography, and right heart catheterization. Pulmonary artery (PA) diameter and PA-ascending aorta ratio (PA-Ao ratio) were obtained from CT. Mean pulmonary artery pressure (mPAP) from right heart catheterization was correlated with tomographic, functional, and echocardiographic variables. The association between the PA-Ao ratio with outcomes was determined by Kaplan–Meier curves.Thirty-four patients were included (18 with pulmonary Langerhans cell histiocytosis and 16 with lymphangioleiomyomatosis, mean age 46 ± 9 years). Forced expiratory volume in the first second and lung diffusing capacity for carbon monoxide were 47 ± 20% and 38 ± 21% predicted, respectively. PA diameter and PA-Ao ratio were 29 ± 6 mm and 0.95 ± 0.24, respectively. PA-Ao ratio > 1 occurred in 38.2% of patients. PA-Ao ratio was a good predictor of PH. mPAP correlated best with PA-Ao ratio, PA diameter, oxygen desaturation during six-minute walk test, and echocardiographic variables. Patients with PA-Ao ratio > 1 had greater mPAP, and a higher risk of death or lung transplantation (log-rank, P < .001) than those with PA-Ao ratio ≤ 1.The PA-Ao ratio measured on CT scan has a potential role as a non-invasive tool to predict the presence of PH and as a prognostic parameter in patients with DCLDs.     

肺切除术对肺癌患者左心功能的影响

目的观察肺切除术对肺癌患者左心功能的影响。方法 37例行肺切除术的肺癌患者,包括肺叶切除组23例、全肺切除组14例,术前及术后10 d采用单光子发射计算机体层扫描仪(SPECT)进行心肌灌注显像,测定患者左室射血分数(LVEF)、左室舒张末期容积(LVEDV)、左室收缩末期容积(LVESV)。结果术后10 d,两组LVEF较术前下降(P均<0.05),LVESV、LVEDV较术前升高(P均<0.05),全肺切除组术后上述指标变化较肺叶切除组明显(P均<0.05)。结论行肺叶或全肺切除术后10 d肺癌患者左心功能较术前明显下降,且全肺切除者左心功能下降较肺叶切除者更明显。     

Pediatric bronchiectasis: correlation of HRCT, ventilation and perfusion scintigraphy, and pulmonary function testing

Bronchiectasis in children, although occurring with diminished frequency, continues as a major challenge for the pediatric pulmonologist. The method of choice for the diagnosis of the condition is high-resolution computed tomography (HRCT). The aim of the present study was to correlate the relationship(s) of HRCT, lung function, ventilation lung scintigraphy (VLS), and perfusion lung scintigraphy (PLS) in children with bronchiectasis. Sixteen children ranging in age from 4-18 years with clinical and chest X-ray evidence of bronchiectasis were enrolled in the study. The degree of bronchiectasis was assessed by HRCT scores, decrease in attenuation on expiratory scans, VLS, and PLS. HRCT scores for bronchiectasis and decreased lung attenuation showed a strong correlation with PLS (rho = 0.82; P < 0.001) and with VLS (rho = 0.72; P < 0.01). There was a moderate negative correlation between FEV(1) and HRCT bronchiectasis scores (rho = -0.53; P = 0.02), decreased lung attenuation score (rho = -0.64; P = 0.007), and atelectasis score (rho = -0.54; P = 0.03). In conclusion, HRCT provides a complete and precise assessment of children with bronchiectasis. Ventilation/perfusion scans and lung functions are additive tools to understand the complexity of the disease process and to improve diagnosis and therapeutic strategies.     

特发性肺纤维化患者的肺功能及CT影像学相关性分析

目的研究特发性肺纤维化(IPF)患者的肺功能与螺旋CT影像学诊断的相关性。方法分析40例临床诊断IPF患者的肺功能和螺旋CT检查结果。入选的所有病例均在1周之内行肺功能测定和胸部CT扫描。结果螺旋CT的磨玻璃样密度影与一秒末用力呼气率(FEV1/FVC)有负相关性(轻度);弥漫性线网状阴影与一氧化碳弥散量(DLco)和一氧化碳弥散率(DLco/VA)有显著负相关性;蜂窝影与FVC、FEV1、TLC、DLco和DLco/VA均具有一定的负相关性。结论不同程度的IPF患者肺功能参数与螺旋CT影像学征象存在一定的相关性。两种检查方法联合应用对于提高临床工作中IPF检出率及预测IPF的进展、预后及疗效有一定参考价值。     

The prevalence of acute response to bronchodilator in pulmonary lymphangioleiomyomatosis

OBJECTIVE: The objective of this study was to evaluate the prevalence of acute bronchodilator responses in patients who were evaluated for pulmonary lymphangioleiomyomatosis. METHODOLOGY: This study was a retrospective review of the medical records of 50 patients, who were assessed for lymphangioleiomyomatosis from 1978 to 2002 at Mayo Clinic in Rochester, Minnesota, and Jacksonville, Florida, USA. Each medical record was reviewed for pertinent clinical and pulmonary function test data, including results for lung volume and flow, diffusing capacity and response to bronchodilator. RESULTS: At initial testing, the mean FEV(1)/FVC ratio for the whole group was 60%; 35 patients (70%) had airflow obstruction; five patients (10%) had only restriction; and 10 (20%) had neither restriction nor obstruction. Three patients (6%) showed a positive response to bronchodilator at initial testing and only one patient showed a positive response at 1 year. Pulmonary function testing at 1-year follow up was available in a subgroup of this cohort (23 patients; 46%), and only one patient (2%) had a positive response to bronchodilator. Although there was a statistically significant decrease in the FEV(1)/FVC ratio (from 64% to 62%; P = 0.02), no other differences were found at 1-year follow up. CONCLUSIONS: In its advanced state, lymphangioleiomyomatosis produces irreversible airflow obstruction. A positive response to bronchodilator was unusual. Other measures of pulmonary function showed little or no change over a 12-month period.     

Effect of liuzijue qigong on patients with stable chronic obstructive pulmonary disease: A systematic review and meta-analysis

Backgrounds:Chronic obstructive pulmonary disease (COPD) is a common, preventable disease of airflow limitation that accounts for the third leading deaths of any disease process in the worldwide. Health benefits of liuzijue qigong (LQG) on patients with stable COPD has been assessed. This study was designed to perform a systemic review and meta-analysis of the effect of Liuzijue breathing exercise on patients with stable COPD.Methods:Published articles from 1970 to December 2020 were conducted using electronic searches. Two independents reviewers conducted data extraction. The Cochrane risk of bias assessment tool was used to evaluate the quality of the included studies.Results:A total of 16 eligible trials with 1039 patients with stable COPD were identified. Compared with control group, the pool meta-analysis of LQG showed a significant improvement in forced expiratory volume in one second (FEV1) (MD = −0.16, 95% CI [0.09, 0.23], P < .00001), FEV1% (MD = 9.71, 95% CI [8.44, 10.98], P < .00001), the ratio of forced expiratory volume to forced vital capacity in the first second (FEV1/FVC [%]) (MD = 4.81, 95% CI [2.12, 7.51], P = .0005), 6 minutes walking distance (6MWD) (MD = 21.89, 95% CI [14.67, 29.11], P < .00001), health-related quality of life (SMD = −0.84, 95% CI [−1.12,-0.55], P < .00001) and modified medical research council dyspnea scale (mMRC) (MD = −0.73, 95% CI [−0.96, −0.50], P < .00001). The observed effect was more pronounced for short term and medium-term duration interventions of study. It also showed improvements in the secondary outcome measures by LQG.Conclusions:In this systematic review and meta-analysis, LQG can improve lung ventilation function, exercise endurance and health-related quality of life of patients with stable COPD.Ethic and dissemination:This study is a systematic review and it does not involve harming to the rights of participants. Ethical approval will not be require for this study. The research results may be published in a peer-reviewed journals.     

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.     

Effect of pulmonary hypertension on lung compliance in newborn lambs

We studied the effects of increased pulmonary arterial pressure and vascular resistance on lung compliance in 12 anesthetized newborn lambs. Lung compliance was unaffected by large changes in pulmonary artery pressure and pulmonary vascular resistance (80% increase) that were induced by either hypoxia or acidosis. We conclude that contraction of the pulmonary vascular smooth muscle in normal newborn lambs does not affect the stiffness of the lung in the parenchymal regions undergoing volume change during ventilation.     

CT引导下经皮肺穿刺的临床应用

目的讨论CT引导下经皮肺穿刺的临床应用。方法分析42例在CT引导下经皮肺穿刺的病例资料。结果 42例患者中,经皮肺穿刺病理诊断40例,准确率达95.24%,其中恶性肿痛24例;良性病变16例。并发症:气胸4例,发生率9.5%,少量咳血8例,发生率19.04%。结论 CT引导下的经皮肺穿刺是一种操作简便、阳性率高、并发症少、创伤小的获得病理的临床诊断方法,值得推广。     

多索茶碱治疗急性加重期慢性阻塞性肺疾病的临床观察

目的观察多索茶碱治疗慢性阻塞性肺疾病急性加重期（AECOPD）的临床疗效。方法选择AECOPD患者85例,随机分为多索茶碱组（治疗组）44例和氨茶碱组（对照组）41例,观察两组患者治疗前后症状、体征及肺功能变化。结果两组治疗前后临床症状、呼吸困难评分和肺功能等比较,差异有统计学意义（P〈0.05）。结论多索茶碱治疗AECOPD能迅速缓解症状,畅通气道,改善肺功能,是治疗AECOPD的有效选择。     

Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a multisystem disorder of women, characterized by cystic degeneration of the lungs, renal angiomyolipomas (AML), and lymphatic abnormalities. LAM lesions result from the proliferation of benign-appearing, smooth muscle-like LAM cells, which are characterized by loss of heterozygosity (LOH) of one of the tuberous sclerosis complex (TSC) genes. LAM cells are believed to migrate among the involved organs. Because of the apparently metastatic behavior of LAM, we tried to isolate LAM cells from body fluids. A cell fraction separated by density gradient centrifugation from blood had TSC2 LOH in 33 of 60 (55%) LAM patients. Cells with TSC2 LOH were also found in urine from 11 of 14 (79%) patients with AML and in chylous fluid from 1 of 3 (33%) patients. Identification of LAM cells with TSC2 LOH in body fluids was not correlated with severity of lung disease or extrapulmonary involvement and was found in one patient after double lung transplantation. These studies are compatible with a multisite origin for LAM cells. They establish the existence of disseminated, potentially metastatic LAM cells through a relatively simple, noninvasive procedure that should be valuable for molecular and genetic studies of somatic mutations in LAM and perhaps other metastatic diseases.