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.
【Abstract】 Objective To investigate the clinical characteristics and prognosis of secondary lymphocytic interstitial pneumonia ( LIP) . Methods Clinical data of 9 cases with secondary LIP diagnosed from1990 to 2010 were retrospectively analyzed. Results Of 9 patients there were 3 males and 6 females,the range of age was 7-64 years. In the 6 adult patients there were 5 females. 2 cases were infected by EB virus and 1 by recurrent pulmonary infection in 3 non-adult patients. In the adult patients, 1 case was diagnosed with Sjogren’s syndrome, 1 case with overlapping syndrome, 2 cases with primary biliary cirrhosis,1 case was probably caused by infection, and 1 case was complicated with eosinophilia. Dominant symptoms of pulmonary system were cough, expectoration, and shortness of breath on exertion. Dominant systematic symptoms were asthenia, pyrexia, weight lose, and arthralgia. CT revealed diffuse ground glass opacities with a lower lung zone predominance. Pathologic feature of LIP was a diffuse, polyclonal lymphoid cell infiltration surrounding airways and extending to the lung interstitium. The patients were treated by glucocorticoid and immunosuppressants. Two cases died with secondary infection. Follow-up did not comfirm malignant tumors in the survivors. Conclusions The clinical features of LIP are characteristic, but lacking of specificity. The final diagnosis depends on pathological examination. Treatment targeted on primary diseases can probably have a good efficacy, and the clinical outcome is favorable.
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.
背景: 吸烟与肺气肿及放射学所见肺间质性异常有关。肺间质性异常与肺总量减少、肺气肿严重程度之间相关的程度尚不了解。方法: 我们从一个吸烟者队列获得了2508 张肺部高分辨率计算机体层摄影( HRCT) 扫描影像, 并在其中的2416 张( 96% ) 中研究了肺间质性异常。我们用线性和逻辑回归( 分析) 评价了肺间质性异常与HRCT 测量的肺总量、肺气肿之间的相关性。结果: 在被评价的2416 张HRCT扫描影像中, 194 张( 8% ) 有肺间质性改变。在校正了相关协变量的统计学模型中, 肺间质性改变与肺总量减少[ - 0. 444 L, 95% 可信区间( CI) - 0. 596 ～- 0. 292, P lt;0. 001] 以及肺气肿相关。此肺气肿发生率相对较低, 标准为肺衰减阈值为- 950 个亨斯菲尔德单位( - 3% , 95% CI - 4 ～- 2, P lt;0. 001) 和- 910 个亨斯菲尔德单位( - 10% , 95% CI - 12 ～ - 8, P lt;0. 001) 定义。与无肺间质性异常者相比, 有异常者更多表现为限制性肺功能损害( 肺总量lt; 预测值的80% , 比值比为2. 3, 95% CI 1. 4 ～3. 7,P lt;0. 001) , 而较少符合慢性阻塞性肺疾病( COPD) 的诊断标准( 比值比为0. 53,95% CI 0. 37 ～0. 76, P lt;0. 001) 。肺间质性异常对肺总量和肺气肿的影响取决于COPD 的状态( 相互作用P lt;0. 02) 。肺间质性改变与较多暴露于烟草烟雾以及当前的吸烟状况呈正相关。结论: 在吸烟者中, 肺间质性异常———大约每12 张HRCT扫描影像就有1 例———主要与肺总量以相关, 并也与肺气肿相关。【述评】间质性肺病肺功能改变以限制性通气功能障碍和弥散功能降低为主要特征, COPD 以阻塞性通气功能障碍为主要表现, 常伴有肺总量和残气容积增加。COPD 合并间质性肺病患者的肺功能改变情况缺乏研究。本研究采用HRCT 扫描评价了吸烟患者合并肺间质性异常时肺气肿和肺容积的改变, 结果发现有肺间质性改变吸烟患者常表现为限制性通气功能障碍。研究者主要通过HRCT 测量研究肺气肿和肺容积改变, 并未通过肺功能仪测定患者的通气功能和弥散功能改变, 如能将影像学改变与肺功能测定结合则更准确反映吸烟合并肺间质性异常者肺通气功能及肺容积改变。