Objective To investigate the constituent ratio and clinical features of diffuse interstitial lung disease（DILD） in Chongqing city.Methods Data was collected from all patients diagnosed as DILD in five comprehensive teaching hospitals in Chongqing from 2002 to 2006.The disease constituent ratio of DILD in the respiratory department or in the whole hospital was analysised.Meanwhile the clinical manifestation and the laboratory examination such as lung function and biopsy were also analysised.Results Mean age of DILD patients is （61.65±13.31）years with a ratio male to female of 1.83.The constituent ratio of DILD were 2.83‰ in respiratory department and 0.30‰ in hospital in 2002，and increased to 8.29‰ and 0.48‰ respectively in 2006.Graticule（62.20%）and honeycombing（24.50%） were predominant imaging manifestations.47.55% patients had restrictive lung function impairment，and 51.05% had mixed lung function impairment.Pathologic examination revealed UIP as the most common type，however，81.82% cases could not be classified to any category pathologically.Conclusions The prevalence of DILD increased progressively from 2002 to 2006.A definite diagnosis of DILD demands cooperation of physicians，radiologists and pathologists.
2000 年美国胸科学会/ 欧洲呼吸学会( ATS/ERS) 发表了特发性肺纤维化( idiopathic pulmonary fibrosis, IPF) 诊断和治疗的共识。历经11 年, IPF 的临床和基础研究均取得了许多重要进展。2011 年来自美国胸科学会( American Thoracic Society,ATS) 、欧洲呼吸学会( European Respiratory Society, ERS) 、日本呼吸学会( Japanese Respiratory Society,JRS) 和拉丁美洲胸科学会( Latin American Thoracic Association, ALAT) 的间质性肺疾病( ILD) 、特发性间质性肺炎( IIP) 和IPF领域的著名专家, 系统回顾了2010 年5 月前有关IPF的文献, 共同制定了第一部以循证为基础的IPF诊断和治疗指南( 简称2011 指南) , 于2011 年3 月正式颁布 。2011 指南涵盖当今对IPF 的最新知识, 囊括IPF 定义、流行病学、危险因素、诊断、自然病史、分期与预后、治疗和疾病过程监控、将来的研究方向等多方面的内容。限于篇幅, 本文对2011 指南中的主要内容作一简介, 以飨国内同道。
Objective To investigate the lung involvement in Chinese patients with systemic sclerosis ( SSc) and its functional impact. Methods 68 patients with SSc were enrolled in Scleroderma study of PUMCH ( Peking Union Medical College Hospital) . All the patients underwent high resolution computed tomography ( HRCT) , pulmonary function testing, 6-minute walk testing, and echocardiography. And 36 patients filled in the Saint George’s Respiratory Questionnaire ( SGRQ) for assessment of healthrelated quality of life. Results HRCT revealed interstitial lung disease ( ILD) in 52 ( 76. 5% ) patients, 20 out of them without respiratory symptoms. Reticular opacification, ground-glass opacity ( GGO) , traction bronchiectasis, and honeycomb were presented respectively in 80.8% , 73.1% , 59.6% , and 30.8% of patients with SSc-ILD. Fibrosis was the predominant HRCT finding, and pure GGO ( in the absence of reticulation or architectural distortion) was only present in 8 ( 15. 7% ) patients. 57 (83.8% ) patients presented with diffusion defect, with most of them having moderate to severe impairent. Reduced FVC or TLC presented in 20 ( 29.4% ) and 28 ( 41.2% ) of patients respectively. The significant correlation was observed between the DLCO and the extent of ILD on HRCT ( rs = -0.476, P =0.000) . DLCO showed significant correlations with all the four components of the SGRQ ( Plt;0.05) . Significant correlations were also observed among the SGRQ scores and SpO2 ( maximum desaturation) or Borg index. Stepwise multiple regression analysis confirmed that the DLCO, SpO2 , and Borg index contributed to the SGRQ. Conclusions Lung involvement in Chinese SSc patients is common and insidious. The HRCT features of SSc-ILD are predominant fibrosis plus GGO, indicating little reversibility. Thus HRCT should be performed routinely in newly diagnosed SSc patients for early screening of lung involvement. The lung function defect of SSc is characterized by reduced diffusing capacity, and DLCO show better correlations with HRCT abnormities or SGRQ than FVC or TLC. Thus DLCO is of great value for early detection or severity assessment of SSc-ILD. SGRQ can be used to examine the health related quality of life of SSc patients and may reflect severity of lung involvement.
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.
Objective To explore the effects of bone marrow mesenchymal stem cells ( BMMSCs) on pulmonary fibroblasts of patients with nonspecific interstitial pneumonitis ( NSIP) , and investigate the therapeutic mechanism of BMMSCs for interstitial pulmonary fibrosis. Methods Human BMMSCs, human pulmonry fibroblasts ( HPFs) from NSIP patients, and normal HPFs were primary cultured in vitro. Then HPFs fromNSIP patients were co-cultured with BMMSCs or normal HPFs using Transwell co-culture system. After 24 hours, levels of transforming growth factor β1 (TGF-β1) and interferon inducible protein 10 ( IP-10) in culture supernatants were detected by ELISA method. Meanwhile, interleukin-6 ( IL-6) , IL-8, and monocyte chemotactic protein-1 ( MCP-1) in co-culture supernatants were detected by liquid chip. After co-cultured for 48 hours, total protein of HPFs was extracted and the expression level of alpha smooth muscle actin ( α-SMA) secreted by HPFs were detected by Western blot.Results HPFs from NSIP patients secreted higher level of IL-6, IL-8, and MCP-1 than normal HPFs, and secreted high level of α-SMA. In the Transwell co-culture system, human BMMSCs significantly reduced the levels of IL-6, IL-8, and MCP-1 secreted from HPFs of NSIP patients, and reduced the high expression of α-SMA in HPFs of NSIP patients. Conclusion Human BMMSCs can significantly reduce the secretion of IL-6, IL-8, MCP-1, and the expression of α-SMA in HPFs from NSIP patients.
Objective To analyze the clinical symptoms, imaging, laboratory tests, efficacy and other indicators of patients diagnosed as diffuse parenchymal lung disease ( DPLD) , in order to provide a reference for differential diagnosis of secondary DPLD and idiopathic interstitial pneumonia ( IIP) .Methods Sixtytwo patients diagnosed as DPLD were retrospectively analyzed. Results In 62 patients with DPLD, 19 patients ( 30. 6% ) were diagnosed as secondary DPLD, 42 cases ( 67. 7% ) as IIP, 1 case ( 1. 6% ) as Langerhans cell histiocytosis. The smoking rate of the DPLD patient was 33. 8% , which was higher than general population ( 29. 7% ) . 94. 7% of the secondary DPLD patients had cough, which was higher than the IIP patients ( 71. 4%) . The average age of onset of the secondary DPLD and IIP was ( 45. 9 ±16. 8) years and ( 60. 5 ±7. 7) years respectively, without significant difference ( P gt; 0. 05) . Etiological factors of secondary DPLD were dust, pets, drugs, pesticides, decoration material, etc. The secondary DPLD patients had higher response rate to steroid therapy, but had no statistical difference compared with the IIP patients ( 46. 2% vs. 37. 5% , P gt;0. 05) . Conclusions As a group of diseases of known causes, history taking is very important for DPLD diagnosis and differential diagnosis. Clinical symptoms, imaging, and laboratory tests may provide reference for differential diagnosis of secondary DPLD and IIP.
ObjectiveTo improve clinicians' knowledge of hypersensitivity pneumonitis (HP). MethodsWe retrospectively analyzed the clinical data of 24 HP patients who were diagnosed in the Affiliated Drum Tower Hospital of Nanjing University Medical School during February 2005 to February 2013. The clinical,radiological and pathological features of those patients were summarized. ResultsAmong those 24 patients,15 were male and 9 were female,with mean age of (48±13) years. All patients had a history of environmental exposure. Two patients showed acute clinical manifestations,and there were 17 subacute and 5 chronic cases. The main clinical manifestations were dyspnea,cough,sputum,fever and weight loss with hypoxemia via blood gas analysis. Restrictive ventilatory impairment was the most frequent functional pattern,and the carbon monoxide diffusing capacity was decreased. Pulmonary function test showed restrictive ventilatory defect and gas interchange disturbance. The features of chest HRCT included diffuse ground-glass attenuation and/or patchy consolidation,centrilobular micronodules,mosaic sign,reticular and/or honeycombing lesions. Bronchoalveolar lavage fluid (BALF) demonstrated an increase of total cell counts with predominant lymphocytosis. The transbronchoscopic lung biopsy (TBLB) pathological examination revealed lymphocytic alveolitis,noncaseating granuloma,and interstial pneumonia. All patients were treated by corticosteroid and avoided antigen exposure and showed significant clinical and radiological improvement. ConclusionThe diagnosis of HP is difficult. In most cases (acute and subacute HP),a diagnosis can be made by combination history of exposure,chest HRCT manifestations,cell classification of BALF and pathological examination of TBLB. For atypical cases (chronic HP),a surgery lung biopsy is needed for multi-disciplinary diagnosis including pathologist,radiologist and pulmonologists.