急性间质性肺炎

急性间质性肺炎（ＡＩＰ）是８０年代中期提出的间质性肺疾病中一个新的分类,其病理特点和临床表现与慢性型的特发性肺纤维化（ＩＰＦ）不同,与成人呼吸窘迫综合征（ＡＲＤＳ）相似,但病因不明。本文就近十余年的文献资料对ＡＩＰ的病理、临床、诊治等方面作一介绍。     

关于特发性肺纤维化病理分类的商榷

编辑同志 :我们近日比较了美国胸科学会[1] 和贵刊发表的 3篇文章[2 4 ] ,发现前文有一些不同的提法 ,主要为 :(1)特发性肺纤维化 (ＩＰＦ) [或隐源性致纤维化性肺泡炎 (ＣＦＡ) ]的定义 :ＩＰＦ是一些特发性间质性肺炎的一种 ,是一个独特的临床疾病 ;(2 )普通型间质性肺炎 (ＵＩＰ)的病理组织学类型与ＩＰＦ是一致的 ;(3)脱屑型间质性肺炎 (ＤＩＰ)、呼吸性细支气管炎伴间质性肺病 (ＲＢＩＬＤ)、急性间质性肺炎 (ＡＩＰ)、非特异性间质性肺炎 (ＮＳＩＰ)、淋巴细胞性间质性肺炎 (ＬＩＰ)以及特发性闭塞性细支气管炎伴间质性肺炎 (特发性…     

急性心肌梗死合并急性呼吸窘迫综合征的诊断和治疗

目的探讨急性心肌梗死（ＡＭＩ）合并急性呼吸窘迫综合征（ＡＲＤＳ）的临床特点和误诊原因，以提高对其认识和诊断水平。方法结合文献资料回顾性分析３例临床病例。结果２例死亡，１例经溶栓，扩张冠状动脉和ＢｉＰＡＰ通气治疗好转。结论ＡＭＩ合并ＡＲＤＳ虽少见，但确定存在，多数情况下易被误诊为左心衰竭性肺水肿，遇不易鉴别病例需行心导管检查。     

急性呼吸窘迫综合征两种诊断标准的比较研究

比较两种ＡＲＤＳ（急性呼吸窘迫综合征）诊断标准的临床应用。符合国内标准者１２９例，病死率４５．０％。符合美欧ＡＬＩ（急性肺损伤）和ＡＲＤＳ诊断标准者分别为４９和７５例，ＡＲＤＳ者的病死率显著高于ＡＬＩ者。ＡＲＤＳ预后与合并ＭＯＤＳ（多器官功能障碍综合征）显著相关。Ｘ线胸片有双肺浸润影而呼吸频率（ＲＲ）小于２８次／分者２１例，其平均年龄（６１．０±７．２）显著高于ＲＲ大于２８次／分者。８例生前符合两种诊断标准者，尸检证明４例无ＡＲＤＳ病理特征。结果表明：①ＡＲＤＳ合并ＭＯＤＳ者病死率明显增高；②老年患者ＲＲ明显增快的程度低于年轻者；③两种诊断标准均存在局限性，对于合并肺部并发症的危重者，诊断ＡＲＤＳ应持之慎重。     

急性呼吸窘迫综合征(ARDS)

急性呼吸窘迫综合征（ＡＲＤＳ）ＡＲＤＳ的基本概念与高危因素中国医科大学呼吸疾病研究所（沈阳，１１０００１）康健于润江ＡＲＤＳ（急性呼吸窘迫综合征）其病因繁多，发病机制错综复杂，病死率高。实际上，对于医学界来说，它并不陌生。早在１９４５年第一次世界大战...     

闭塞性细支气管炎伴机化性肺炎

闭塞性细支气管炎伴机化性肺炎是 Epler 等于1985年提出的一个新病名,但它不是一个新疾病。本文就其概念、病理特点、临床表现、胸部 X 线特点、支气管-肺泡灌洗液的细胞学检查、诊断和鉴别诊断、治疗、预后和病因进行了论述。此外,本文介绍了本病与普通型间质性肺炎在病理和临床上的区别。     

急性间质性肺炎

急性间质性肺炎中国医科大学呼吸疾病研究所（沈阳，１１０００１）于润江急性间质性肺炎（ａｃｕｔｅｉｎｔｅｒｓｔｉｔｉａｌｐｎｅｕｍｏｎｉａＡＩＰ）是急性病程、预后极为不佳、原因未明的间质性肺炎。是由Ｋａｔｚｅｎｓｔｅｉｎ１９８６年首次提出的一个新的疾病...     

流行性出血热成人呼吸窘迫综合征的发病机理与治疗

流行性出血热成人呼吸窘迫综合征的发病机理与治疗江苏省人民医院传染科黄湘虎，孙志坚，唐季和流行性出血热（ＥＨＦ）的发病机理及发病过程中出现的许多病理生理变化都可能使本病容易发生成人呼吸窘迫综合征（ＡＲＤＳ），且临床上常易与急性左心衰竭肺水肿相混淆，及时...     

老年脱屑性间质性肺炎

脱屑性间质性肺炎 (ＤＩＰ)是一种少见的特发性弥漫性间质疾病 ,早期被划在特发性肺纤维化 (ＩＰＦ)的范畴内。 1998年Ｋａｔｇｅｎｓｔｅｉｎ和Ｍｇｅｒｓ因ＤＩＰ的临床症状、组织病理、治疗和预后方面和呼吸性细支气管炎伴间质性肺病 (ＲＢＩＬＤ)有很多相似之处 ,且 2种疾病与长期吸烟有密切关系 ,而将其视为一类 ,即ＤＩＰ/ＲＢＩＬＤ ,从ＩＰＦ中分离出来。直到 1999年美国胸科学会和欧洲呼吸病学会的新分类法中才将ＤＩＰ列为一种独立性疾病。肺间质疾病尤其ＩＰＦ的发病率在世界范围内日渐提高 ,已成为常见病。这类疾病的早中期在临…     

慢性再生障碍性贫血与难治性贫血鉴别的探讨

慢性再生障碍性贫血与难治性贫血鉴别的探讨王秀瑛，刘晓华，夏明玉慢性再生障碍性贫血（ＣＡＡ）是临床上常见的血液系统疾病，典型ＣＡＡ不难确诊。在临床实践中ＣＡＡ与骨髓增生异常综合征（ＭＤＳ）中难治性贫血（ＲＡＲＡ）的鉴别有时很困难。正确的诊断对治疗与预后...     

自身免疫性胰腺炎诊治进展

自身免疫性胰腺炎(AIP)是一种由自身免疫介导、以胰腺肿大及胰管不规则狭窄为特征的胰腺慢性炎症性疾病,病理以胰管纤维化伴有淋巴浆细胞浸润为特征,其治疗尚缺乏统一标准。本文就自身免疫性胰腺炎的流行病学、发病机制、临床特点、影像学检查、病理学特点、诊断标准、治疗及预后七个方面的相关研究进展进行综述,以加深对其认识及了解。     

Clinically occult subpleural fibrosis and acute interstitial pneumonia a precursor to idiopathic pulmonary fibrosis?

Background and objective:   The clinical features and acute component of the pathological findings in patients with acute exacerbation of IPF are similar to those of acute interstitial pneumonia (AIP). A growing body of research suggests that acute exacerbation of subclinical IPF is similar to AIP. This study tested the hypothesis that subpleural fibrosis (resembling localized usual interstitial pneumonia) in patients with diffuse alveolar damage can mimic patients with AIP alone.
Methods:   The clinical, radiological and histopathological characteristics of patients with AIP with or without subpleural fibrosis, as identified at post-mortem examination, were assessed.
Results:   Fourteen patients with AIP met the inclusion criteria. These comprised nine men and five women ranging in age from 65 to 90 years. The mean duration from onset of symptoms to admission was 12.3 days, and from admission to death was 50.5 days. All patients presented with severe respiratory failure with bilateral pulmonary infiltrates on CXR. CT scans revealed bilateral ground glass opacity (100.0%), air space consolidation (69.2%) and no honeycombing. In seven patients (50.0%) histological examination showed subpleural fibrosis. There were no differences in the clinicopathological or CT scan findings between patients with AIP only compared with those with AIP and subpleural fibrosis.
Conclusions:   Subpleural fibrosis resembling localized usual interstitial pneumonia is often present in patients diagnosed as having AIP. These patients were not distinguishable from those with AIP only, based on the clinicopathological and radiological findings.     

Sorafenib-induced acute interstitial pneumonia in patients with advanced hepatocellular carcinoma: report of three cases

Little is known about acute interstitial pneumonia (AIP) induced by sorafenib therapy in patients with advanced hepatocellular carcinoma (HCC). Here, we present three patients with advanced HCC who developed AIP during sorafenib therapy, with fatal complications in two cases. Case 1 was a 76-year-old man who developed dyspnea. Chest CT showed interstitial pneumonia. Sorafenib was discontinued immediately, and prednisolone was started. His pneumonia resolved. A drug-induced lymphocyte stimulation test for sorafenib was positive. Case 2 was a 75-year-old man and case 3 was a 77-year-old man, both of whom developed high-grade fever and hypoxemia during sorafenib therapy, and were diagnosed with AIP. In spite of high-dose steroid therapy, their respiratory failure worsened and both patients died. In all three cases, serum KL-6 or surfactant protein D concentrations were elevated, and blood and sputum cultures did not grow pathogens. All three patients were smokers with restrictive lung disease on preoperative respiratory function testing, but did not have respiratory symptoms before sorafenib therapy. The clinical features of these three cases suggest that male gender, older age, smoking history, and lung disease are associated with acute sorafenib-induced AIP in patients with advanced HCC.     

Low hospital mortality in patients with acute interstitial pneumonia

STUDY OBJECTIVES: To compare the presenting features and outcome of patients with acute interstitial pneumonia (AIP) with other patients with diffuse alveolar damage (DAD) and with historical control subjects. DESIGN: Retrospective chart review. SETTING: A large, urban, teaching hospital. INTERVENTIONS: Patients were classified into idiopathic (AIP group) and secondary causes of DAD (ARDS group) according to available clinical and microbiology data. AIP and ARDS cases were compared, and ARDS cases were analyzed for long-term outcome. Measurements and results: Twenty patients with pathologic diagnosis of DAD were identified. Four cases were excluded; eight cases of ARDS due to known etiologies were identified. These etiologies included pneumonia and sepsis (n = 6), cocaine use (n = 1), and carmustine chemotherapy (n = 1). Eight cases of AIP were found. When compared with the ARDS group, patients in the AIP group had a longer time from the onset of symptoms until hospital admission (16.8 +/- 15.7 days vs 2.2 +/- 1.0 days, p = 0.0015) and a shorter time from hospital admission to open-lung biopsy (8.3 +/- 3.0 days vs 15.5 +/- 9.5 days, p = 0.02) [mean +/- SD]. Seven of eight patients with AIP and four of eight patients with ARDS survived to hospital discharge (p = not significant). The 12.5% mortality rate for patients with AIP reported in this series was significantly lower than the previously reported cumulative rate of 69.5% (p = 0.0025). Follow-up in five AIP survivors for a mean of 7.6 +/- 3.5 years (range, 3 to 11 years) showed all to be without shortness of breath or relapse despite mild residual fibrosis on chest radiograph and mild-to-moderate restriction on pulmonary function tests (mean total lung capacity, 68.5 +/- 6.2% predicted). CONCLUSIONS: Our data support a favorable hospital and long-term outcome for patients with AIP, with no evidence of recurrence or progression to chronic interstitial lung disease.     

Acute interstitial pneumonia.

The term "acute interstitial pneumonia" (AIP) describes an idiopathic clinicopathological condition, characterized clinically by an interstitial lung disease causing rapid onset of respiratory failure, which is distinguishable from the other more chronic forms of interstitial pneumonia. It is synonymous with Hamman-Rich syndrome, occurring in patients without pre-existing lung disease. The histopathological findings are those of diffuse alveolar damage. AIP radiologically and physiologically resembles acute respiratory distress syndrome (ARDS) and is considered to represent the small subset of patients with idiopathic ARDS. It is frequently confused with other clinical entities characterized by rapidly progressive interstitial pneumonia, especially secondary acute interstitial pneumonia, acute exacerbations and accelerated forms of cryptogenic fibrosing alveolitis . Furthermore, many authors use the above terms, both erroneously and interchangeably. It has a grave prognosis with >70% mortality in 3 months, despite mechanical ventilation. This review aims to clarify the relative clinical and pathological issues and terminology.     

开胸肺活检对肺间质疾病的诊断价值

目的探讨开胸肺活检对肺间质疾病的诊断作用。方法对１９９３～１９９８年６月２４例开胸肺活检的肺间质疾病患者进行回顾性分析。结果２４例患者均获病理确诊,其中普通型间质性肺炎（ＵＩＰ）７例,闭塞性细支气管炎伴机化性肺炎（ＢＯＯＰ）３例,结节病３例,弥漫性泛细支气管炎（ＤＰＢ）２例,肺结核２例。其他急性间质性肺炎（ＡＩＰ）、呼吸性细支气管炎伴肺间质病（ＲＢＩＬＤ）、肺组织细胞增生症Ｘ、炎性结节、多发性肺脓肿、肺组织炎症和肺泡细胞癌各１例。结论开胸肺活检作为一种诊断方法,能获得足够的肺组织,具有很高的敏感性和特异性,能明确病变的部位和程度。对常规和纤维支气管镜未能确诊的病例,尤其是一些罕见病和不典型的病例,具有较大的价值。     

Elevation of cytokeratin 19 fragment in patients with interstitial pneumonia associated with polymyositis/dermatomyositis.

OBJECTIVE: Cytokeratin 19 fragment (CK19) levels in serum have been documented as a useful tumor marker for lung cancer. We hypothesize that CK19 may increase in serum in patients with interstitial pneumonia associated with polymyositis/dermatomyositis (PM/DM) and CK19 might be a useful variable to evaluate the activity of lung injury. METHODS: 1. We measured CK19 in sera in 15 patients diagnosed with PM/DM; 6 had nonspecific interstitial pneumonia (NIP), 4 had acute interstitial pneumonia (AIP), and 5 had no pulmonary involvement. We also measured CK19 in 10 healthy nonsmokers serving as a control group. 2. We evaluated the correlation between CK19 level and individual clinical course in patients with pulmonary involvement associated with PM/DM. RESULTS: CK19 levels in sera of patients with NIP associated with PM/DM were significantly higher versus patients with PM/DM without interstitial pneumonia and healthy nonsmokers. CK19 levels in sera in patients with AIP associated with PM/DM were significantly higher compared with the other groups. CK19 values in sera changed according to the progression or improvement of interstitial pneumonia. Immunohistochemical studies using pulmonary tissues obtained at autopsy from patients with AIP associated with PM/DM revealed that the hyaline membrane was mostly stained by anti-human cytokeratin 19 monoclonal antibody as well as the strong positivity of proliferating type II pneumocytes. CONCLUSION: These results suggest that the measurement of CK19 was a useful variable to evaluate the activity of lung injury in interstitial pneumonia associated with PM/DM.     

Desquamative interstitial pneumonia-like changes in idiopathic pulmonary fibrosis

A 61-year-old man was admitted to our hospital with fever, cough and dyspnea on exertion. The chest X-ray showed diffuse reticulo-granular infiltrates. Deterioration of clinical features and remarkable elevation of BALF lymphocytes (64.3%) suggested active interstitial pneumonia. The open lung biopsy specimen showed chronic interstitial pneumonia with DIP-like pathologic change. There was a remarkable clinical, physiological and roentgenographic improvement associated with decrease of BALF lymphocytes in response to steroid therapy. BAL is useful for monitoring disease activity and tapering steroids in patients with interstitial pneumonia who respond to steroid therapy.     

Acute interstitial pneumonia: report of a series.

Four cases of acute interstitial pneumonia (AIP) are described with special emphasis on clinical background, lung imaging and bronchoalveolar lavage findings. A retrospective chart review of four patients with histologically-proven AIP, diagnosed between 1998 and 2000, was carried out. Clinical data, bronchoalveolar lavage (BAL) findings, high-resolution computed tomography (HRCT) and histological features were analysed. Three patients died and only one is in follow-up. HRCT showed areas of ground glass attenuation and alveolar consolidation in all patients. Histology, documented by open lung biopsy or autopsy specimens, was consistent with the organising form of diffuse alveolar damage pattern. BAL findings were characteristic, with a huge neutrophilia associated with scattered atypical type II pneumocytes collected in clusters with extracellular amorphous material (fragments of hyaline membranes) observed in two out of three cases. In this paper, four cases of acute interstitial pneumonia are reported in detail. The poor prognosis associated with this entity has been confirmed and the possible diagnostic role of the bronchoalveolar lavage is emphasised.