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1、Case Study,Chief Complaint: 62-year-old man with progressive shortness of breath over the past 2 yearsHistory of Present Illness: Two years before patient began having shortness of breath (SOB). The SOB had become progressively worse in the past 12 months. A month prior to presentation, he developed
2、 severe SOB requiring admission to a local hospital. The patient reported no exposures related to hypersensitivity pneumonitis including birds, mold. His only chemical exposure was to malathion he sprayed in his backyard garden.,Past Medical & Surgical History: unremarkable Allergies: PenicillinMedi
3、cines:Nifedipine ,Furosemide ,Statin Family History: Negative for lung disease or rheumatologic processesSocial History: 30 pack-years cigarette consumption and stopped 15 years earlierTravel History: Negative,Physical Examination: General: well appearing Vital Signs: BP: 110/70 ,Pulse: 85 RR ,26 br
4、eaths/minute HEENT: No skin tightness around his mouth Neck: No jugular vein distention Cardiovascular System: No findings of pulmonary hypertension Respiratory System: Inspiratory crackles over lower half of chest Extremities: clubbingMusculoskeletal System: no arthritis or synovitis,Laboratory Wor
5、kup: ANA negative, speckled pattern with a negative Sm antibody, negative Scl-70 antibody; an echocardiogram revealed an estimated mean pulmonary artery pressure of 55 mmHg. Initial PFT Data: FVC 63% of predicted ,FEV1/FVC 85%DLCO 30% of predicted TLC 54% of predicted,HRCT FINDINGS,Slide courtesy of
6、 G Raghu, MD.,间质性肺疾病（Interstitial Lung Disease,ILD),北京医院呼吸科与危重症医学科 许小毛,什么是肺间质,肺泡间及终末气道上皮以外的支持组织，包括血管及淋巴管组织。肺实质指各级支气管及肺泡结构。,概述,以肺泡壁为主并包括肺泡周围组织及其相邻支持结构病变的一组疾病群,病因近200种。由于病变不仅局限于肺泡间质,还可累及肺泡上皮细胞、肺毛细血管内皮细胞和细支气管,并常伴有肺实质受累如肺泡炎、肺泡腔内蛋白渗出等改变,故也称为弥漫性肺实质疾病(Diffuse Parenchymal Lung Disease,DPLD),Classification o
7、f DPLD,DPLD,感染,全身性疾病,家族史,暴露史,ILD,不同的病因所致的ILD可以出现相同的病理表现，如RF和SLE可引起相同病理表现的ILD。同一种疾病可以表现为不同的病理表现，如干燥综合症可以表现为UIP，也可为NSIP。,不同的ILD在病因、发病机制、病理改变、自然病程、治疗方法及预后方面都不完全相同。诊断的目的不仅限于ILD，而是要尽可能的明确病因和病理类型。,临床表现,呼吸困难咳嗽、咯血与结缔组织并相关的症状：发热、脱发、皮疹、关节痛、眼干、口干。,体格检查,肺部听诊爆裂音或Velcro罗音杵状指紫绀肺动脉高压征象,实验室检查,一般检查：免疫学指标、ANCA,SACE肺功能
8、影像学支气管镜检查（BALF,TBLB,TBNA)肺组织活检（开胸肺活检，VATS),诊断思路,病史、体格检查、胸部X线检查(特别是HRCT)和肺功能测定来进行综合分析。诊断步骤包括1、明确是否是ILD/DPLD2、明确属于哪一类ILD/DPLD3、如何对IIP进行鉴别诊断。,是否为ILD,病史中最重要的症状是进行性气短、干咳和乏力。多数ILD患者体格检查可在双侧肺底闻及Velcro啰音。晚期病人缺氧严重者可见紫绀。 胸部X线对的诊断有重要作用。磨玻璃样改变, 小结节影、线状(网状)影或二者混合的网状结节状阴影。肺泡充填性疾病表现为弥漫性边界不清的肺泡性小结节影,有时可见含气支气管征,晚期肺容
9、积缩小可出现蜂窝样改变。 肺功能检查主要表现为限制性通气功能障碍和弥散功能(DLCO)下降。动脉血气分析可显示不同程度的低氧血症,而二氧化碳潴留罕见。,属于哪一类ILD/DPLD,(1)详实的病史是基础:包括环境接触史、职业史、个人史、治疗史、用药史、家族史及基础疾病情况。 (2)胸部X线影像(特别是HRCT)特点可提供线索:根据影像学的特点、病变分布、有无淋巴结和胸膜的受累等,可对ILD/DPLD进行鉴别诊断。,(3)BALF检查有确诊价值或者有助于诊断:找到感染原,如卡氏肺孢子虫;找到癌细胞;肺泡蛋白沉积症:呈牛乳样,过碘酸-希夫染色阳性;含铁血黄素沉着症:呈铁锈色并找到含铁血黄素细胞;石
10、棉小体计数超过1/ml:提示石棉接触。(4)某些实验室检查包括:抗中性粒细胞胞浆抗体:见于韦格纳肉芽肿;抗肾小球基底膜抗体:见于肺出血肾炎综合征;针对有机抗原测定血清沉淀抗体:见于外源性过敏性肺泡炎;特异性自身抗体检测:提示相应的结缔组织疾病,如何对IIP进行鉴别诊断,如经上述详实地检查及分析,仍不能确定为何种ILD/DPLD,就应归为IIP。其中IPF/UIP最常见,占所有IIP的60%以上,NSIP次之,而其余类型的特发性间质性肺炎相对少见。IIP的最后确诊,除了IPF可以根据病史、体征、支气管肺泡灌洗检查及胸部HRCT作出临床诊断外,其余确诊均需依靠病理诊断,Final diagnosi
11、s,CRP C- Clinical R- Radiologist P- Pathologist,Idiopathic Pulmonary Fibrosis(IPF),CLASSIFICATION OF IIP,CLASSIFICATION OF IIP,Major IIPs IPF INSIP RB-ILD DIP AIP,Rare IIPs ILIP Idiopathic pleuroparenchymal fibroelastosis Unclassifiable IIPs,ATS/ERS 2013,特发性间质性肺炎(Idiopathic Interstitial Pneumonia,II
12、P)属于ILD/DPLD中的一种。而特发性肺纤维化(Idiopathic Pulmonary Fibrosis,IPF)属于IIP中的一种,病理学上称为寻常性间质性肺炎（usual interstitial pneumonia,UIP )。,OVERVIEW,Prevalence: 1320/100,000 in US (approximately 35,000-55,000 cases)Onset: Usually between 50 and 70 yrClinical presentationProgressive dyspnea on exertionParoxysmal cough,
13、 usually nonproductiveAbnormal breath sounds on chest auscultation Abnormal chest x-ray or HRCTRestrictive pulmonary physiology with reduced lung volumes and DLCO and widened AaPO2,the mean length of survival from the time of diagnosis varied between 3.2 and 5 yrIn another study, the median survival
14、 was 28.2 mo from the onset of respiratory symptoms,POTENTIAL RISK FACTORS,Cigarette SmokingExposure to Commonly Prescribed DrugsChronic AspirationEnvironmental FactorsInfectious AgentsGenetic Predisposition to IPF,What is the cause of IPF?,Old idea - inflammation causes fibrosisNew idea -epithelial
15、 injury with abnormal healing cause fibrosisLimitation -patients present late in course of disease,Old idea,Inflammation causes fibrosis - BAL of lungs showed inflammatory cells in the lungTreatment with antiinflammatory medications -prednisone -imuran -cytoxanEffective in 15-30% of patient,New idea
16、,IPF is a consequence of ongoing alveolar epithelial injury and cell death.Epithelial cell injury probably leads to activation of TGF-, activation or induction through epithelial to mesenchymal transformation (EMT).,Symptoms,IPF usually presents insidiously, with the gradual onset of a nonproductive
17、 cough and dyspnea. Dyspnea is usually the most prominent and disabling symptom. It is usually progressive and in most patients it is reported to have been present for 6 mo before presentation. paroxysmal dry cough that is refractory to antitussive agents.,Physical examination,crackles are detected
18、on chest auscultation in more than 80% of patients. These are typically “dry,” end-inspiratory, and “Velcro” in quality, and are most prevalent in the lung bases. Clubbing is noted in 25 to 50% of patients .Cyanosis, cor pulmonale, an accentuated pulmonic second sound, right ventricular heave, and p
19、eripheral edema may be observed in the late phases of the disease,Laboratory and Serological Tests,The routine laboratory evaluation of a patient suspected of having IPF is often not helpful except to “rule out” other causes of ILD.Positive circulating anti-nuclear antibodies (ANAs) or rheumatoid fa
20、ctor occur in 10 to 20% of patients with IPF, but rarely are titers high. The presence of high titers ( 1:160) would suggest the presence of a connective tissue disease,High Resolution CT Scanning,patchy, predominantly peripheral, subpleural, bibasal reticular abnormalities. There may also be a vari
21、able amount of ground glass opacity that is usually limited in extent.In areas of more severe involvement there is often traction bronchiectasis and bronchiolectasis and/or subpleural honeycombing.,HRCT FINDINGS,Pulmonary Function Testing,The typical findings of pulmonary function tests are consiste
22、nt with restrictive impairment (reduced vital capacity VC and total lung capacity TLC)The DLC is reduced and may actually precede the reduction of lung volume.,Lung Biopsy,Usual interstitial pneumonia (UIP) is the histopathological pattern that identifies patients with IPF. Surgical lung biopsy reco
23、mmended in patients with suspected IPF, especially those with atypical clinical or radiographic featuresMajor purpose of histologic examination is to distinguish UIP from other histologic subsets of IIP,The potential risks and cost associated with surgical lung biopsy need to be balanced against the
24、 accuracy of a clinical diagnosis, the likelihood of identifying a more treatable form of ILD, and the efficacy of the treatment .,lung biopsy may be outweighed by increased risk for surgical complications (e.g., age 70 yr, extreme obesity, concomitant cardiac disease, extreme impairment in pulmonar
25、y function).,Histopathological assessment.,The histologic hallmark and chief diagnostic criterion is a heterogeneous appearance at low magnification with alternating areas of normal lung, interstitial inflammation, fibrosis, and honeycomb change.,Slide courtesy of KO Leslie, MD.,HISTOPATHOLOGIC ELEM
26、ENTS OF UIP,IPF诊断标准,排除其它已知原因导致的ILD (如：环境和职业导致的肺病，CTD-ILD，和药物性肺病等)。在没有外科肺活检资料条件下，胸部HRCT呈现典型的UIP表现。有外科肺活检资料条件下，胸部HRCT和病理均符合UIP表现。,Subjected to external review,HRCT 诊断标准,UIP 的病理学标准,明显纤维化 / 结构破坏,伴或不伴有胸膜下/间隔周围蜂窝样改变肺实质呈现斑片状纤维化出现成纤维母细胞灶缺乏不支持UIP诊断的特征,病理诊断标准,HRCT 与肺活检结果联合诊断IPF,Subjected to external review,HR
27、CT 与肺活检结果联合诊断IPF,Subjected to external review, Multidisciplinary discussion: sampling error on SLB? adequate HRCT?,HRCT 与肺活检结果联合诊断IPF,Subjected to external review, Multidisciplinary discussion: sampling error on SLB? adequate HRCT?,Treatment,Drugs: Corticosteroids, cytoxan, imuran, High-dose N-acetylcysteineLung Transplantation,Survival in treated vs untreated patients.,Collard H R et al. Chest 2004;125:2169-2174,IPF患者死亡率增高相关的特征,基础因素 呼吸困难程度 DLCO预计值的40% 6分钟步行实验中氧饱和度88% HRCT蜂窝肺的范围 肺动脉高压纵向因素 呼吸困难加重 FVC绝对值下降10% DLCO绝对值下降15% HRCT纤维化加重,Thank you ！,
