如何从弥漫性间质性肺疾病中诊断特发性肺纤维化

弥漫性间质性肺病（interstitiallungdiseaseILD)是重要的呼吸疾病谱之一，国内外的一些资料显示，本病的发病率近年有增高趋势。ILD的病因近200种，种类庞杂，使得诊断有时非常棘手。因此，面对ILD这一病谱，建立正确的临床诊断思维程序，对于认识病因至关重要。首先应明确是否为弥漫性间质性肺病特发性肺纤维化（idiopathicpulmonaryfibrosis,IPF）属于特发性间质性肺炎（idiopathicinterstitialpneumonia,IIP）中的一种，而后者为原因不明的ILD。因此，首先应认清什么是ILD。通过询问病史、体格检查、胸部影像学检查、肺功能（…     

肺功能测定对几种间质性肺疾病的诊断价值

本文分析了22例特发性肺间质纤维化(IPF)、40例肺结节病(Sarc)和9例胶原病性肺炎(IP—CVD)的肺功能变化。IPF、IP—CVD肺功能改变较为明显,主要表现为严重的弥散功能障碍和限制性通气功能障碍。Sarc组近半数肺功能正常,其余主要为阻塞性通气功能障碍,限制性和弥散性肺功能障碍的程度较IPF和IP—CVD组明显为轻。Sarc三期间肺功能无明显差异。三组肺功能下降最为显著的是DLco,其次是FEV_1,FVC和FRC等,提出这些指标对间质性肺疾病可能有辅助诊断价值,但需结合其它检查才能确诊。     

间质性肺疾病的诊疗困境

间质性肺疾病是一类以肺间质炎症与纤维化为主要表现的临床异质性疾病总称。因间质性肺疾病临床 影像 病理特点之间存在交叉和重叠,其诊断和治疗仍面临较多困境。在诊断方面,间质性肺疾病早期诊断率低,根据影像学改变、支气管肺泡灌洗液标本,甚至肺部组织标本也难以明确疾病类型诊断;在治疗方面,除某些已知病因的继发性间质性肺疾病,大多间质性肺疾病只能通过免疫抑制和抗纤维化治疗延缓疾病进程,目前缺乏抗纤维化的特效药物。随着相关发病机制研究和新诊治技术手段的应用,期待间质性肺疾病的诊疗困境将逐步得到解决。     

间质性肺疾病的诊断思路

间质性肺疾病(interstitial lung disease,ILD)又称为弥漫性实质性肺疾病,是一类包括200多个病种的疾病。其病理改变主要发生于肺泡间质结构,也可累及细支气管和肺血管。ILD患者的临床、影像学及肺功能表现均相似,以进行性劳力性呼吸困难、胸部X线胸片示弥漫性浸润阴影、肺功能示限     

间质性肺疾病的高分辨CT诊断及临床意义

目的：评价胸部高分辨CT（HRCT）检查对间质性肺疾病的征象阳性检出率及与临床症状的一致性。材料与方法：回顾性分析结合临床症状行HRCT检查的190例患者,比较疑似间质性肺疾病及临床确诊间质性肺疾病患者的临床症状,与不同症状组的HRCT检查阳性率差异。结果：在不同组间间质性肺疾病的诊断率分别为：8.46%、29.31%和38.89%;在有进行性气短及胸部X线平片检查在短时间内（尤其是2周内）变化显著的患者中,HRCT检查可有29.31%的检出率,且58.9%的患者为轻中度的患者。结论：HRCT是诊断间质性肺疾病的重要检查手段,选择合适的切入点进行HRCT检查是早期发现间质性肺疾病,减少误诊、漏诊的有效途径,并可避免患者接受不必要的过度检查。     

间质性肺疾病治疗现状

间质性肺疾病(ILD)是一类以肺间质弥漫性渗出、浸润和纤维化为主要病变的呼吸系统疾病,发病率约5／10万。其病因复杂,临床上缺乏有效的治疗手段,预后差,1年生存率仅50％。因此,探索新的治疗方法和治疗途径,是目前亟待解决的临床问题。笔者复习文献,对现有的治疗药物和方法进行综合分析,为探讨新的治疗方法提供理论依据。     

间质性肺疾病的中医辨证治疗

间质性肺疾病由一组异质性疾病组成 ,是不同病因侵犯肺泡壁、肺泡腔以及整个下呼吸道 ,包括上皮细胞、内皮细胞、间质细胞和肺泡壁的结缔组织而致的疾病。它们在 X线、呼吸生理和病理形态诸方面具有相类似表现 ,最终可发展为弥漫性肺间质纤维化 ,导致呼吸衰竭。近几年来 ,本病的发生率呈逐渐上升趋势 ,现代医学的治疗方向是 :应尽量在肺泡炎阶段予以控制 ,此时肺泡结构尚未被破坏 ,上皮细胞和基底膜尚完整 ,积极彻底治疗可以阻止病变进一步发展 ,所选药物主要为皮质激素。皮质激素的应用时间要求长 ,用量要求足 ,但长时间用药有一定副作用 …     

Diagnosis of interstitial lung diseases

Interstitial lung diseases (ILDs), a broad heterogeneous group of parenchymal lung disorders, can be classified into those with known and unknown causes. The definitions and diagnostic criteria for several major forms of ILDs have been revised in recent years. Although well over 100 distinct entities of ILDs are recognized, a limited number of disorders, including idiopathic pulmonary fibrosis, sarcoidosis, and connective tissue disease-related ILDs, account for most ILDs encountered clinically. In evaluating patients with suspected ILD, the clinician should confirm the presence of the disease and then try to determine its underlying cause or recognized clinicopathologic syndrome. Clues from the medical history along with the clinical context and radiologic findings provide the initial basis for prioritizing diagnostic possibilities for a patient with ILD. High-resolution computed tomography of the chest has become an invaluable tool in the diagnostic process. A confident diagnosis can sometimes be made on the basis of high-resolution computed tomography and clinical context. Serologic testing can be helpful in selected cases. Histopathologic findings procured through bronchoscopic or surgical lung biopsy are often needed in deriving a specific diagnosis. An accurate prognosis and optimal treatment strategy for patients with ILDs depend on an accurate diagnosis, one guided by recent advances in our understanding of the causes and pathogenetic mechanisms of ILDs.     

胸痛相关肺血管病的诊治进展

胸痛是临床常见症状之一,本文针对以胸痛特点为临床表现的肺血管疾病进展予以综述,尤其是致死性肺栓塞的危险因素、临床表现、诊疗及预防等方面的进展,提高对肺栓塞的认识水平,为临床肺血管病的诊治提供参考,并提出了围绕肺血管病的诊治管理模式。     

Differential diagnosis of diffuse interstitial pulmonary diseases

Classification, differential diagnosis and treatment of diffuse interstitial pulmonary diseases are considered. Data on their X-ray (computed tomographic) and morphological manifestations are presented along with analysis of efficiency of pathogenetic (anti-inflammatory and antifibrotic) therapy.     

Diagnosis and treatment of pulmonary diseases caused by Mycobacterium avium complex

Recently, the clinical importance of non-tuberculous mycobacteria(especially, Mycobacterium avium complex [MAC] respiratory infection) has been increasing. In addition, an official ATS/IDSA statement about diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases has been published in February, 2007. In this review article, clinical features and radiological findings of pulmonary MAC diseases mainly i) primarily fibrocavitary disease, and ii) nodular/bronchiectatic disease are described. Primarily fibrocavitary disease is characterized by cavitary lesions in upper lung fields in elderly subjects, smoking patients, or patients with pneumoconiosis. Nodular/bronchiectatic disease is characterized by centrilobular nodules and diffuse bronchiectases in the right middle lobe and the left lingula in middle-aged women. In addition, diagnosis and treatment for pulmonary diseases caused by MAC are also described.     

Diagnosis and treatment of pulmonary hypertension

Primary pulmonary hypertension is a rare disease of unknown etiology, whereas secondary pulmonary hypertension is a complication of many pulmonary, cardiac and extrathoracic conditions. Chronic obstructive pulmonary disease, left ventricular dysfunction and disorders associated with hypoxemia frequently result in pulmonary hypertension. Regardless of the etiology, unrelieved pulmonary hypertension can lead to right-sided heart failure. Signs and symptoms of pulmonary hypertension are often subtle and nonspecific. The diagnosis should be suspected in patients with increasing dyspnea on exertion and a known cause of pulmonary hypertension. Two-dimensional echocardiography with Doppler flow studies is the most useful imaging modality in patients with suspected pulmonary hypertension. If pulmonary hypertension is present, further evaluation may include assessment of oxygenation, pulmonary function testing, high-resolution computed tomography of the chest, ventilation-perfusion lung scanning and cardiac catheterization. Treatment with a continuous intravenous infusion of prostacyclin improves exercise capacity, quality of life, hemodynamics and long-term survival in patients with primary pulmonary hypertension. Management of secondary pulmonary hypertension includes correction of the underlying cause and reversal of hypoxemia. Lung transplantation remains an option for selected patients with pulmonary hypertension that does not respond to medical management.     

不同肺活检方法对肺部弥漫性间质性疾病的诊断价值

目的 探讨不同肺活检方法在肺部弥漫性问质性疾病诊断中的价值.方法 回顾性分析因弥漫性肺问质性疾病接受经支气管镜肺活检(transbronchial lung biopsy,TBLB)、CT引导下经皮肺活检(CT-guided percutaneous needle aspiration biopsy,PCNA)、外科电视胸腔镜(lung biopsy by video-assisted thoracoscopic surgery,VATS)及小开胸肺活检(openlungbiopsy,OLB)患者的临床和病理诊断资料.结果 73例患者接受TBLB,36例病理诊断为肺间质纤维化,7例诊断UIP,8例诊断结核,2例诊断为肺泡蛋白沉着症.28例患者接受经皮肺活检,其中18例得到病理分型.外科电视胸腔镜肺活检28例,小开胸肺活检20例,除1例未明确外,其余全部病理分类.结论 在诊断弥漫性肺问质性疾病时,可首先考虑创伤性小的经纤维支气管镜肺活检,可除外感染、肿瘤及结节病.肺部弥漫性间质性疾病中特发性问质性肺炎的病理诊断特别是病理分型需要较大的活检组织,应考虑进行外科电视胸腔镜或小开胸肺活检.