以肺间质病变为首发表现的细支气管肺泡癌1例

目的 介绍1例以肺间质病变为首发表现的细支气管肺泡癌并复习相关文献,以提高临床医师对该病的认识.方法 对1例确诊为细支气管肺泡癌的患者诊治过程进行分析,结合文献讨论其临床特点、诊断.结果 胸部影像学表现为肺间质病变的细支气管肺泡癌易被误诊,肺穿刺活检是确诊的重要依据.结论 细支气管肺泡癌患者临床表现无特异性,提高认识并早期肺活检是降低细支气管肺泡癌误诊率的必由之路.     

对比呼气负压与传统方法检测慢性阻塞性肺疾病患者呼气气流受限

目的 对比呼气负压(NEP)与传统方法 检测慢性阻塞性肺疾病(COPD)患者呼气气流受限(EFL).方法 应用NEP与传统方法 (层流型流最计)检测72例COPD缓解期患者EFL;对20例不能完成传统测试的患者应用NEP检测其EFL;对比两种方法 的检测结果 .结果 72例COPD患者应用NEP 54例(75%)检测到EFL.第1秒用力呼气容积(FEV_1)占预计值百分比EFL评分2分者为(46.92±12.74)%,3分者为(33.35±8.96)%,显著低于EFL评分1分者[(63.60±16.65)%].ROC曲线分析NEP曲线下面积(AUC)为0.903,层流型流量计AUC为0.761.20例不能完成传统测试的患者均能耐受NEP测试,19例检测到EFL.结论 应用NEP检测COPD患者EFL,比应用传统方法 检测更能准确反映COPD患者呼吸困难的严重程度;NEP操作简单、无需患者主动配合,可在不能完成传统测试的患者中应用.     

呼气相气道内负压法检测呼气流速受限在慢性阻塞性肺疾病中的应用

目的探讨慢性阻塞性肺疾病(COPD)患者呼气流速受限(EFL)与呼吸困难严重程度的相关性,并观察吸入支气管扩张剂对 COPD 患者 EFL 的影响。方法采用呼气相气道内负压法(NEP)检测33例 COPD 患者支气管扩张试验前、后(吸入沙丁胺醇400μg)EFL 情况,其中男31例,女2例,年龄46～78岁,平均年龄(63±8)岁。结果 33例 COPD 患者中23例(70%)出现 EFL,其中11例(33%)仅仰卧位出现 EFL,12例(36%)仰卧位及坐位均出现 EFL。无 EFL 患者与 EFL 患者第一秒用力呼气容积占预计值百分比(FEV_1占预计值%)分别为(66±16)%和(31±10)%,差异有统计学意义(t=7.601、P<0.01),仰卧位及坐位均出现 EFL 患者的 FEV_1占预计值%最低[(24±7)%]。3分法和5分法 EFL 均与 FEV_1呈显著负相关(r=-0.836和-0.818,P 均<0.01)。3分法和5分法 EFL 均与医学研究委员会(MRC)推荐的呼吸困难严重程度分级评分标准(简称 MRC 呼吸困难评分)呈显著正相关(r=0.903和0.912,P均<0.01)。多元回归分析结果显示,5分法 EFL 和FEV_1对 MRC 呼吸困难评分的预测性均有统计学意义(标准化偏回归系数分别为0.679、-0.265,P分别为<0.01、0.029),但5分法 EFL 比 FEV_1对 MRC 呼吸困难评分的预测性更强。23例吸入沙丁胺醇前存在 EFL 患者,吸入后全部患者 EFL 仍然存在。结论与 FEV_1比较,EFL 对 COPD 患者呼吸困难严重程度预测性更强,可作为评价 COPD 患者呼吸困难严重程度更可靠的客观指标。COPD 患者的 EFL 不能被吸入支气管扩张剂逆转,即表现为 EFL 的不可逆性。     

Noninfectious interstitial lung disease during infliximab therapy: Case report and literature review

Pulmonary abnormalities are not frequently encountered in patients with inflammatory bowel diseases.However,lung toxicity can be induced by conventional medications used to maintain remission,and similar evidence is also emerging for biologics.We present the case of a young woman affected by colonic Crohn’s disease who was treated with oral mesalamine and became steroid-dependent and refractory to azathioprine and adalimumab.She was referred to our clinic with a severe relapse and was treated with infliximab,an antitumor necrosis factor α(TNF-α)antibody,to induce remission.After an initial benefit,with decreases in bowel movements,rectal bleeding and C-reactive protein levels,she experienced shortness of breath after the 5thinfusion.Noninfectious interstitial lung disease was diagnosed.Both mesalamine and infliximab were discontinued,and steroids were introduced with slow but progressive improvement of symptoms,radiology and functional tests.This represents a rare case of interstitial lung disease associated with infliximab therapy and the effect of drug withdrawal on these lung alterations.Given the increasing use of anti-TNF-α therapies and the increasing reports of pulmonary abnormalities in patients with inflammatory bowel diseases,this case underlines the importance of a careful evaluation of respiratory symptoms in patients undergoing infliximab therapy.     

Chronic obstructive pulmonary disease and interstitial lung disease in patients with lung cancer

Background and objective:   Although lung cancer is frequently accompanied by COPD and interstitial lung disease (ILD), the precise coincidence of these diseases with lung cancer is not well understood. The objectives of this study were to determine the prevalence of abnormal CT and spirometric findings suggestive of COPD or ILD in a population of patients with untreated lung cancer, and to estimate the lung cancer risk in this population.
Methods:   The study population consisted of 256 patients with untreated lung cancer and 947 subjects participating in a CT screening programme for lung cancer. Semi-quantitative analysis of low attenuation area (LAA), fibrosis and ground glass attenuation (GGA) on CT was performed by scoring. Gender- and age-matched subpopulations, with stratification by smoking status, were compared using the Mantel–Haenszel projection method.
Results:   Inter-observer consistency was excellent for LAA, but not as good for fibrosis or GGA scores. Pooled odds ratios for lung cancer risk using LAA, fibrosis, GGA scores and reduced FEV₁/FVC and %VC were 3.63, 5.10, 2.71, 7.17 and 4.73, respectively ( P  < 0.0001 for all parameters). Multivariate regression analyses confirmed these results.
Conclusion:   Abnormal CT and spirometric parameters suggestive of COPD and ILD were strong risk factors for lung cancer, even after adjusting for gender, age and smoking status.     

成人肺表面活性蛋白C基因突变相关家族性间质性肺疾病2例并文献复习

目的:提高对成人肺表面活性蛋白C（SFTPC）基因突变相关家族性间质性肺疾病的临床表现、影像表现、诊断和治疗的认识。方法:回顾分析宁波大学医学院附属医院诊断的2例成人SFTPC基因突变相关家族性间质性肺疾病,结合文献进行复习。以“familial interstitial lung disease”“SFTPC gen...     

无肌炎性皮肌炎引起急性肺间质病变2例并文献复习

目的提高对无肌炎性皮肌炎引起的急性肺间质病变（ADM—ILD）的临床表现诊断及治疗的认识。方法报道1例死亡及1例成功救治的无肌炎性皮肌炎引起的急性肺间质病变,并结合国内文献报道35例ADM—ILD患者的临床资料进行分析。结果临床上表现为胸闷、气促、干咳、发热、进行性呼吸困难、紫绀,并很快到严重的低氧血症和呼吸衰竭,高分辨率CT有很大的诊断价值。治疗效果欠佳。结论无肌炎性皮肌炎引起的急性肺间质病变死亡率很高,临床治疗过程中应该引起重视。     

Two‐year experience with mycophenolate mofetil in patients with scleroderma lung disease: a case series

To assess the effect of mycophenolate mofetil (MMF) on pulmonary functions in patients with systemic sclerosis‐associated lung disease (SSc‐ILD) who experienced an inadequate response to first line cyclophosphamide (CYC) therapy. Twelve consecutive SSc‐ILD patients who received MMF due to inadequate response to CYC as a first line agent, were retrospectively reviewed. Over the course of 2 years, pulmonary function tests (PFT) and high‐resolution computed tomography (HRCT) scans were performed. Following initial baseline tests, PFTs were continued at a frequency of every 6 months and HRCT scans were performed every 12 months. After MMF treatment, values of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) improved in three (25%) and two (16.6%) patients, respectively. It is also noted that the evaluation of serial HCRT scans showed no change in 54.5% of patients. Our case series suggested that PFT and imaging scores seemed to be stabilized by MMF in SSc‐ILD patients who were inadequate responders to CYC.     

Neurofibromatosis-associated lung disease: a case series and literature review.

An association of neurofibromatosis with diffuse lung disease (NF-DLD) has been described, but its true prevalence and characteristics remain unclear. The objective of the present study was to define diffuse lung disease in patients with neurofibromatosis. A retrospective case series and literature review in a tertiary care academic medical centre is reported in which medical records, chest radiographs and high-resolution computed tomography (HRCT) scans were reviewed. A total of 55 adult patients with neurofibromatosis were identified, three of whom had NF-DLD. A literature review revealed 16 articles reporting 61 additional cases, yielding a total of 64 NF-DLD cases. The mean age of patients was 50 yrs. Males outnumbered females; most reported dyspnoea. Of the 16 subjects with documented smoking histories, 12 were ever-smokers. Eight patients had HRCT scan results demonstrating ground-glass opacities (37%), bibasilar reticular opacities (50%), bullae (50%), cysts (25%) and emphysema (25%); none had honeycombing. A group of 14 patients had surgical biopsy results that showed findings of interstitial fibrosis (100%) and interstitial inflammation (93%). In conclusion, neurofibromatosis with diffuse lung disease is a definable clinical entity, characterised by upper lobe cystic and bullous disease and lower lobe fibrosis. Its relationship to smoking remains unclear.     

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.     

间质性肺病的治疗进展

间质性肺病是一组异源性肺部弥漫性疾病,其病因各异,故治疗方法及对药物的反应也各不相同。近年来,在对药物反应不佳的间质性肺疾病的治疗方面涌现了不少新的药物,本文将对各类间质性肺病的治疗以及近年来提出的可用于治疗间质性肺病的药物作一简要的概述。     

IgG4相关性肺疾病二例并文献复习

目的 总结IgG4相关性肺疾病的临床特点及诊治经验,提高临床对该病的认识,减少误诊误治.方法 分析第二军医大学附属长海医院呼吸内科收治的两例IgG4相关性肺疾病的一般情况、临床表现、实验室检查、组织病理学、治疗及预后,并进行相关文献复习.结果 两例患者均为男性,血清IgG4水平均显著升高(2.25 g/L;10 g/L).1例肺部影像学呈实性结节型改变,并有回盲部受累,糖皮质激素治疗有效.另1例肺部影像学呈支气管血管束型改变,对糖皮质激素治疗依赖.文献复习69例IgG4相关性肺疾病,因呼吸道症状就诊者39例(56.5％),伴有肺外受累者41例(59.4％).48例检测了血清IgG4水平,均显著升高(307 ～ 52 500 mg/L).胸部影像学表现实性结节型35例(50.7％).31例(44.9％)患者接受了糖皮质激素治疗,预后良好.结论 IgG4相关性肺疾病是临床少见的免疫性疾病,临床表现缺乏特异性,临床诊断应综合分析其血清免疫学检查、影像学表现及组织病理学结果,糖皮质激素治疗效果良好.     

Outcomes of patients with interstitial lung disease referred for lung transplant assessment

Background: Patients with interstitial lung disease (ILD) very frequently die before the opportunity to receive lung transplantation (LTx). This retrospective study describes the clinical course of 86 patients with ILD referred for LTx assessment between January 1999 and December 2002. Aims: (i) To describe the outcomes, (ii) to identify reasons of delay to transplantation, (iii) to describe the causes of death/complications and (iv) to assess the pathological diagnosis and concordance with explanted lung pathology. Methods: Data were collected from the case notes of all patients with ILD referred to the Alfred Hospital over a 4‐year period. Results: Twenty women and 66 men, mean age of 55 ± 8 years, were referred for LTx assessment. Forty‐five patients were deemed not suitable for LTx and 41 were listed. Twenty‐two patients underwent transplantation, 16 died on the waiting list and 7 are still on the waiting list. Complications were frequent (e.g. pulmonary embolism, malignancy and infection) and carried high mortality. Patients dying on the waiting list appeared generally to be in accelerated decline, dying shortly after listing, with no evidence in their lung function test assessment predicting them as a poor prognosis group. Conclusions: Serious complications and death on the waiting list of patients with idiopathic pulmonary fibrosis are high, not apparently because of delayed referral but usually in patients undergoing very rapid decline.     

Depression is a common and chronic comorbidity in patients with interstitial lung disease

Background and objective: Little is known about depression in interstitial lung disease (ILD). The aim of this study was to determine the prevalence of depression, characterize the association of depression with clinical variables and describe the natural history of depression in patients with ILD. Methods: In this prospective cohort study, clinical variables were recorded at baseline and 6 months. Depression was measured with the Centre for Epidemiologic Studies Depression scale. Depression prevalence was determined using the established threshold of >15 points. Multivariate linear regression was used to determine the baseline features that independently correlated with baseline depression score and that predicted depression severity at follow‐up. Results: Fifty‐two subjects were enrolled, and 45 returned for follow‐up (three deaths, one lung transplant). Prevalence of depression was 21% at baseline. Independent predictors of depressive symptoms at baseline included dyspnoea severity, pain severity, sleep quality and forced vital capacity (R² 0.67). The odds of clinically meaningful depression at follow‐up were 34‐fold higher for subjects who had clinically meaningful depression at baseline compared with those who were not (95% confidence interval 3.5–422, P < 0.0005). Baseline depression score was the strongest predictor of depression score at follow‐up (r 0.59, P < 0.00005). Conclusions: Depressive symptoms in ILD are common, persistent, and strongly and independently correlated with dyspnoea, pain, sleep quality and forced vital capacity. Clinically meaningful depression at baseline is the most important predictor of depressive symptoms at follow‐up. Patients with ILD should routinely be screened for depression.