美國胸科協會(ATS)和歐洲呼吸學會(ERS)聯合發表的共識中，將特發性肺纖維化(IPF)定義為原因不明并以普通型間質性肺炎(UIP)為特征性病理改變的一種慢性纖維化性間質性肺疾病。在2000年ATS／ERS的IPF共識意見 及2003年中華醫學會呼吸病學分會IPF的診斷和治療指南(草案) 中均推薦的治療方案為糖皮質激素，或與細胞毒制劑(環磷酰胺及硫唑嘌呤)聯合使用。但目前尚缺乏循證醫學證據支持該治療方案能夠提高IPF患者生活質量或生存率 。近年來隨著對IPF的發病機制認識的深入，越來越多的臨床醫師和研究者對IPF患者是否需要用糖皮質激素等藥物的治療提出了質疑。
2000 年美國胸科學會/ 歐洲呼吸學會( ATS/ERS) 發表了特發性肺纖維化( idiopathic pulmonary fibrosis, IPF) 診斷和治療的共識。歷經11 年, IPF 的臨床和基礎研究均取得了許多重要進展。2011 年來自美國胸科學會( American Thoracic Society,ATS) 、歐洲呼吸學會( European Respiratory Society, ERS) 、日本呼吸學會( Japanese Respiratory Society,JRS) 和拉丁美洲胸科學會( Latin American Thoracic Association, ALAT) 的間質性肺疾病( ILD) 、特發性間質性肺炎( IIP) 和IPF領域的著名專家, 系統回顧了2010 年5 月前有關IPF的文獻, 共同制定了第一部以循證為基礎的IPF診斷和治療指南( 簡稱2011 指南) , 于2011 年3 月正式頒布 。2011 指南涵蓋當今對IPF 的最新知識, 囊括IPF 定義、流行病學、危險因素、診斷、自然病史、分期與預后、治療和疾病過程監控、將來的研究方向等多方面的內容。限于篇幅, 本文對2011 指南中的主要內容作一簡介, 以饗國內同道。
骨化性氣管支氣管病( tracheobronchopathia osteochondroplastica,TPO 或TO) , 是一種罕見的氣管內膜系統的良性病變, 主要以氣管和主支氣管黏膜下多發性骨和軟骨組織結節狀增生為特征, 通常引起氣道狹窄[ 1] 。大多數患者沒有臨床癥狀, 因此易被誤診和漏診。本文將TO 的臨床特征做一綜述, 以提高對該病的認識。
ObjectiveTo explore the differential diagnosis value of airspace consolidation in thoracic CT between organizing pneumonia (OP) and acquired community pneumonia (CAP).MethodsA retrospective study was taken by retrieving the patients CT database from October 2010 to August 2016. Fifty-six consecutive patients with OP and 99 consecutive patients with CAP whose CT showed airspace consolidation were enrolled and their clinical characteristics and radiological characteristics were analyzed.ResultsThe percentage of patients whose CT image showed various amount of air bronchogram (ABG) with different shapes is higher in OP group than that in CAP group (87.5% and 72.7% respectively, χ2=4.558, P=0.033). The median and interquartile range amount of ABG in the OP patients were significantly higher than those in CAP group [4 (ranged from 2 to 8) and 2 (ranged from 0 to 4) respectively, z=3.640, P=0.000]. Morphologically, 58.9% of the OP patients showed entire air bronchogram (EABG) on the thoracic CT, significantly higher than that in CAP group (21.2%) (χ2=22.413, P=0.000). Interrupted ABG was found in 26.3% of CAP patients, while 16.1% of OP patients shared same features and the difference was not statistically significant (χ2=2.125, P=0.148). Traction bronchiectasis and ground glass opacity (GGO) were more likely to be found in the OP patients rather than CAP patients with 26.8% and 39.3% respectively, while they were found in 1.0% and 11.1% in the CAP patients (P<0.05). Reversed halo sign was found only 1.0% of the CAP patients, significantly lower than that in OP group, 26.8% (χ2=25.671, P=0.000). Pleural effusion and bronchial wall thickening were more commonly found in the CAP group with 56.6% and 35.4% respectively. By multivariate logistic analysis, EABG (OR=5.526, P=0.000), traction bronchiectasis (OR=21.564, P=0.010), GGO (OR=4.657, P=0.007) and reversed halo sign (OR=13.304, P=0.023) were significantly associated with OP, while pleural effusion (OR=0.380, P=0.049) and bronchial wall thickening (OR=0.073, P=0.008) were significantly associated with CAP. Other features in thoracic CT coexisting with ABG all reach significance statistically between the OP and CAP group (all P<0.05).ConclusionsAirspace consolidation in thoracic CT may be valuable for the differential diagnosis between OP and CAP. EABG is more commonly found in OP patients than in CAP patients. When EABG exists or ABG coexists with traction bronchiectasis, GGO and reversed halo sign, a diagnose of OP should be considered.
Objective To analyze the clinical presentations and radiological characteristics of pathologically proved cases of cryptogenic organizing pneumonia(COP).Methods The clinical and radiological features of 8 patients with COP confirmed by open lung biopsy were analyzed.Treatment and follow-up data were also recorded.Results There were 5 male an 3 female patients aged 37 to 68 years.Dyspnea，cough and inspiratory crackles were the most common symptoms and signs.Various computed tomography findings including ground glass opacities,pathy consolidation with air bronchograms，nodules and reticulation were simultaneously observed in the same patient.The diagnostic imaging features of COP were patchy or lobar consolidation，often by a predominantly subpleural distribution，and irregular band-like opacities distributed along the bronchovascular bundle or located in the subpleural area.All patients were treated with corticosteroids and yielded significant improvement in seven cases.Conclusions COP could be diagnosed by clinical and radiological findings and histopathological examination was needed for confirmed diagnosis.In general，COP responds well to glucocorticoid therapy and has a benign prognosis.
ObjectiveTo evaluate the role of rapid on-site evaluation (ROSE) in the diagnosis of lung space-occupying lesions.MethodsFrom June 1, 2017 to December 30, 2017, transbronchial biopsies were performed in patients with lung space-occupying lesions on chest CT, and biopsies were taken for ROSE and subsequent routine pathological examination. ROSE interpretation was performed by a pulmonologist who had been trained in cytopathology for 3 months. The interpretation was correlated with the follow-up routine pathological examination. The contemporary cases with lung space-occupying lesions who underwent transbronchial biopsies without ROSE were retrospectively reviewed. The quality assessment of biopsy specimens recorded in pathological reports were compared between cases with and without ROSE.ResultsA total of 101 patients underwent transbronchial biopsies in parallel with ROSE. The interpretation results of the pulmonologist were compared with the follow-up routine pathology, which showed that the consistency rate of malignant lesions was 84.1% and the consistency rate of benign lesions was 93.8%. Consistency test showed good agreement between the ROSE conducted by the pulmonologist and the routine pathological examination by pathologists (κ=0.66, P<0.01). The quality assessment of biopsy specimens showed that there was no significant difference on rate of satisfied biopsy specimens between cases with and without ROSE (98.0%vs 94.5%, P=0.14).ConclusionsThe use of ROSE combining with bronchoscopy allows good preliminary assessment of lung space-occupying lesions. Pulmonologists trained in short-term formal cytopathology are fully capable of performing ROSE, thereby obviating the need for cytopathologists to participate in on-site evaluation.
Objective To explore the prognostic significance of baseline clinical and pulmonary physiological variables on idiopathic pulmonary fibrosis ( IPF) . Methods Patients diagnosed with IPF according to 2011 ATS/ERS/JRS/ALAT statementwere selected from Nanjing DrumTower Hospital between January 1, 2002 and July 31, 2010. The baseline characteristics were abstracted, including age, gender, smoking history, corticosteroid, delay before diagnosis, body mass index, finger clubbing, oxygenation index ( PaO2 /FiO2 ) , C-reaction protein, erythrocyte sedimentation rate ( ESR) , serum lactate dehydrogenase ( LDH) , albumin, vital capacity ( VC) , forced vital capacity ( FVC) , total lung capacity ( TLC) , and singlebreath diffusing capacity of the lung for carbon monoxide ( DLCO) . The relationships between all factors and survival were examined with a univariate Cox proportional-hazard model. Kaplan-Meier method was used to assess the survival probabilities between groups with different baseline characteristics. Results Eighty-four patients were included in this study, with the median survival time of 34. 7 months. PaO2 /FiO2 , FVC% pred, VC% pred, TLC% pred, and DLCO% pred showed significant associations with the mortality of IPF ( hazard ratios 0. 940-0. 994, P lt; 0. 01) . The Kaplan-Meier analyses for above variables also showed significant differences ( P lt;0. 05) . Besides, the statistical difference of survival probability could be found between the patients with elevated serumLDH and those with normal LDH ( 27. 0 months vs. 43. 1 months, P =0. 014) . Conclusions Baseline oxygenation and pulmonary function parameters may indicate the prognosis of IPF patients. Serum LDH may provide clinicians with additional prognostic information.
Objective To highlight the characteristics of giant cell interstitial pneumonia ( GIP) . Methods The clinical, radiological, and pathological data of two patients with GIP pathologically proven by open lung and TBLB biopsy were presented respectively, and relevant literatures were reviewed. Results Patients with GIP usually had a history of exposure to metal dust. Clinical presentations included cough and dyspnea on exertion, and pulmonary function testing showed a restrictive abnormality. On chest radiography and high-resolution CT scans, it presented as bilateral areas of ground-glass attenuation, areas of consolidation, diffuse small nodules, extensive reticular opacities, and traction bronchiectasis. The main pathological findings included a desquamative interstitial pneumonia ( DIP) -like reaction with intra alveolar macrophages and numerous large multinucleated histiocytes that ingested inflammatory cells were admixed with macrophages. The finding of GIP was almost pathognomonic for hard metal pneumoconiosis. Conclusions GIP is a very rare chronic interstitial pneumonia, and has no characteristic clinical manifestations. Radiographic findings are similar to other idiopathic interstitial pneumonias. Careful collection of the occupational history can help to minimize misdiagnosis.
ObjectiveTo compare the expressive differences of plasma Kerbs von den lungen-6 (KL-6) in patients with idiopathic interstitial pneumonia (IIP) and connective tissue disease associated secondary interstitial pneumonia (CTD-SIP), and analyze the clinical significances.MethodsThe clinical data and peripheral blood of 399 inpatients with interstitial pneumonia and 50 healthy controls were collected from January 2011 to December 2014 in Nanjing Drum Tower Hospital. The level of plasma KL-6 was measured by chemiluminescence immunoassay method. The subjects were divided into IIP (n=233) group and CTD-SIP (n=166) group, usual interstitial pneumonia (UIP) pattern and non-UIP pattern, and stable (S) UIP group and acute exacerbation (AE) UIP group. Statistical analyses were performed by using IBM SPSS 19.0 (SPSS, Inc., Chicago IL, USA) to compare the differences of plasma KL-6 in groups.ResultsThere were more male subjects (61.8%) in the IIP group, and the average age of (62.3±12.5) years was significantly older (both P<0.01). Plasma KL-6 levels in the IIP ［(1 822.7±1 505.2) U/ml) and the CTD-SIP group ［(1 846.7±1 625.3) U/ml］ were significantly higher than the healthy control group ［(190.2±88.7) U/ml］ (both P<0.001). However, there was no any difference of KL-6, white blood cell count (WBC), lactate dehydrogenase (LDH), C-reactive protein (CRP) and erythrocyte sedimentation rate between the IIP and the CTD-SIP group. The level of plasma KL-6 was positively correlated to WBC, LDH and CRP in the IIP group (r=0.159, P=0.016; r=0.380, P<0.001; r=0.158, P=0.015, respectively); and it was positively correlated to LDH and CRP in the IIP group (r=0.187, P=0.016 and r=0.068, P=0.032) in the CTD-SIP group. There was no significant difference of plasma KL-6 between the UIP and non-UIP subgroups (P>0.05). The difference of plasma KL-6 between the S-UIP and AE-UIP subgroup was significant (P<0.001 and P=0.023). There was no any significant difference of plasma KL-6 among the subgroups with CTD patients (primary Sj?gren’s syndrome, n=90; rheumatoid arthritis, n=20; polymyositis/dermatomyositis, n=26; undifferentiated connective tissue disease, n=10; anti-neutrophil cytoplasmic antibody associated vasculitis, n=15 and systemic sclerosis, n=5) (P=0.785 2).ConclusionsPlasma KL-6 may be a useful biomarker for interstitial pneumonia. It can show the disease activities, but is not able to distinguish IIP from SIP.
Objective By analyzing and summarizing the clinical characteristics of pulmonary capillary hemangiomatosis (PCH), to enhance clinical physicians’ recognition, diagnosis and treatment capabilities for PCH. Methods Clinical data of two cases of PCH, who were diagnosed in Nanjing Tower Hospital, were reported retrospectively. Simultaneously, by using “Pulmonary Capillary Hemangiomatosis” as keywords, a total of 227 relevant articles was retrieved from domestic and international databases and 113 articles of case report were analyzed. Results Two cases of PCH patients were diagnosed eventually in our hospital, which were initially misdiagnosed as other diseases. After follow-up, the first case underwent lung transplantation four months after diagnosis, and the second case had been regularly taking medication to decrease pulmonary arterial pressure. Currently, two patients were in stable condition with pulmonary artery pressure significantly reduced. In literature review, 93 patients’ medical records were included in this study. We summarized the diagnostic methods, clinical manifestations, genetic testing, function check results, imaging characteristics, histopathology, treatment and prognosis of these patients. Conclusions PCH is very rare. The extremely low incidence rate and nonspecific clinical manifestations lead to high risk of misdiagnosis and underdiagnosis. Therefore, fully understanding and grasping its clinical characteristics are crucial for reducing misdiagnosis and underdiagnosis in the future.