Evaluation of survival in mixed connective tissue disease (MCTD)

INTRODUCTION: 179 patients with mixed connective tissue disease (MCTD) were follow-up, and the cause of death was analyzed in 12 died patients. PATIENTS AND METHODS: The survival of 179 patients with MCTD was evaluated by using Kaplan-Meier's method. Clinically and immunological data of the patients were analyzed between 1 and 25 years follow-up period (mean: 13.1 +/- 5.5 years). RESULTS: The five-year survival rate was 96.4%, 10-year survival rate was 93.9%, and the 15-year survival rate was 89.6%. The cause of death was pulmonary hypertension in 5 patients, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in 3 cases, infection in 3 cases (hepatitis C virus induced hepatic coma in 2 patients, Staphylococcus sepsis in one patient), and on one patient myocarditis. The pulmonary hypertension was the most serious prognostic factor. CONCLUSION: In patients with MCTD the pulmonary hypertension with endothelial cells proliferation and microangiopathy developed very quickly, and there was progressive and therapy resistant statement. The secondary virus and bacterial infections may develop in the patients who were followed-up the long term period. Their survival rate was better than the data in the literature. This fact may cause genetic-demographic factors, and the sequential follow-up of the patients.     

肺功能及高分辨CT在间质性肺疾病中的相关性研究

目的 对间质性肺疾病患者影像学、肺功能及临床表现进行相关分析，评价肺功能特别是脉冲振荡（IOS）测定呼吸阻抗在该疾病诊断中的意义。方法 对56例间质性肺疾病患者的呼吸阻抗、常规肺功能、静息时动脉血氧分压（PaO2）及肺部高分辨CT评分和呼吸困难评分进行分析。结果 振荡频率在5Hz时的电抗（X5）、在20Hz时的电抗（X20）与肺活量（VC）呈正相关（r=0．699、0．537，P〈0．05），与用力肺活量（FVC）呈正相关（r=0．690、0．473，P〈0．05），与一氧化碳弥散量（DLCO）呈正相关（r=0．494、0．301，P〈0．05），与PaO2呈负相关（r=-0．621、-0．411，P〈0．05）；响应频率与VC、FVC、DLCO呈负相关（r=-0．658、-0．639、-0．352，P〈0．05），与PaO2呈正相关（r=0．277，P〈0．05）；特发性肺间质纤维化患者的病程较结缔组织病所致间质性肺疾病患者的病程明显延长（P〈0．05），其余观察指标差异无统计学意义（P〉0．05）。结论 常规肺功能指标VC、FVC、DLCO及呼吸阻抗指标响应频率、X5对肺间质纤维化的诊断有一定的意义；IOS测定呼吸阻抗对间质性肺疾病患者肺功能损害的动态监测和疗效评价具有一定的应用前景；应重视结缔组织病患者的影像学和肺功能检查以便及时发现肺部早期病变。     

慢性阻塞性肺疾病合并肺间质纤维化的临床研究

目的分析慢性阻塞性肺疾病合并肺间质纤维化（IPF—COPD）的特点并探讨其临床意义。方法分析6年来我院发现的28例PIF-COPD患者的病史、临床表现、X线胸片、肺高分辫率CT（HR—CT）、血气分析和肺功能检查结果。结果PIF—COPD的临床表现介于上述两种疾病,常有长期大量吸烟史。X线胸片、HRCT兼有两种疾病的特点。其肺功能多为混合性通气功能障碍,一氧化碳弥散量（DLCO）多为中重度下降。血气以低氧血症为主,部分合并有二氧化碳潴留。结论COPD和IPF是两种不同疾病,但可以同时存在,其临床表现具有独特性。肺功能检查、血气分析、X线胸片和肺CT．尤其肺HR—CT可为确诊提供依据。     

Human herpesvirus 8 and pulmonary hypertension

Human herpesvirus 8 (HHV-8) antibodies were detected in 1 of 33 patients with pulmonary hypertension (including in 1 of 16 with idiopathic pulmonary arterial hypertension), 5 of 29 with cystic fibrosis, and 3 of 13 with interstitial lung disease. No relationship between HHV-8 infection and pulmonary hypertension was found.     

Pneumoconiosis and Associated Medical Conditions

Hospital discharge data from Michigan for the years 1990 and 1991 were used to examine potential associations between pneumoconiosis and pulmonary hypertension, lung cancer, obstructive lung disease, and connective tissue disease among both men and women. Lung cancer, pulmonary hypertension, and obstructive lung disease were associated with coal workers' pneumoconiosis. Pulmonary hypertension and obstructive lung disease were associated with asbestosis and silicosis. Rheumatoid arthritis was associated with silicosis. The potential is suggested that misdiagnosis is the cause of the association between lung cancer and coal workers' pneumoconiosis.     

Significance of acute pulmonary embolism for the exacerbation of chronic obstructive pulmonary disease

The clinical diagnosis of acute pulmonary embolism in patients with chronic obstructive pulmonary disease is often difficult because there is no characteristics sign of acute pulmonary embolic event. The presentation of an acute embolic event may mimic the symptoms of acute airway obstruction which is caused by different reasons. The presence of chronic obstructive pulmonary disease was recorded on a clinical history, physical examination, chest roentgenogram, and the result of pulmonary function studies. Among the 178 patients with chronic obstructive pulmonary disease in 49 patients (27.5 percent) were suspected of having pulmonary embolism. Pulmonary embolism was excluded by normal or low probability V/Q scan in 19 patients. Among 23 patients who demonstrated a high probability lung scan there was a high positive predictive value for pulmonary embolism effectively avoiding the need for further studies. Among the 7 patients with nondiagnostic or intermediate probability lung scan contrast-enhanced helical computed tomography was detected the pulmonary embolism in 3 patients. The present study was undertaken to better define the noninvasive clinical, laboratory, roentgenographic, lung scan, and computed tomography characteristics of acute pulmonary embolism in patients with chronic obstructive pulmonary disease.     

Primary hemostasis in mixed connective tissue disease]

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease. High levels of Willebrand factor antigen and activity were detected in the presence of thrombocytopenia, previous thrombotic events, pulmonary vascular lesions and in the presence of circulating antiendothelial antibodies. Increased platelet activation was documented also in antibody positive cases and in thrombocytopenia. The alterations of endothelial and platelet functions may play important role in the development of vascular complications of mixed connective tissue disease.     

先天性心脏病合并重度肺动脉高压的外科治疗

目的 探讨先天性心脏病合并重度肺动脉高压的外科治疗.方法 选择先天性心脏病合并重度肺动脉高压患者32例.动脉血氧饱和度0.84一0.94,左向右分流20例,双向分流10例,无分流2例.测肺动脉压65～120(82±14) mm Hg(1 mm Hg=0.133 kPa).所有病例均在体外循环下行心内畸形矫治手术,术前给予吸氧、西地那非等治疗,术中应用乌司他丁及低温肺保护液肺动脉灌注等手段,术后常规应用血管活性药物.结果 32例手术顺利,术后肺动脉压下降20～40 mm Hg.30例治愈出院,2例术后早期死于严重低心排血量综合征及致命性心律失常,病死率为6.25％(2/32).结论 先天性心脏病合并肺动脉高压经围手术期综合治疗后再行手术根治可获得良好的临床效果.     

A new high resolution computed tomography scoring system for pulmonary fibrosis, pleural disease, and emphysema in patients with asbestos related disease.

The aim of this study was to describe a scoring system for high resolution computed tomographic (HRCT) scans analogous to the International Labour Office (ILO) scoring system for plain chest radiographs in patients with asbestos related disease. Interstitial fibrosis, pleural disease, and emphysema were scored, the reproducibility and the interobserver agreement using this scoring system were examined, and the extent of the various types of disease was correlated with measurements of lung function. Sixty asbestos workers (five women and 55 men) mean age 59 (range 34-78) were studied. The lungs were divided into upper, middle, and lower thirds. An HRCT score for the extent of pleural disease and pulmonary disease in each third was recorded in a way analogous to the International Labour Office (ILO) method of scoring pleural and parenchymal disease on chest radiographs. A CT score for the extent of emphysema was also recorded. Pleural disease and interstitial fibrosis on the plain chest radiographs were assessed according to the ILO scoring system. A chest radiographic score for emphysema analogous to that used for HRCT was also recorded. Two independent readers assigned HRCT scores that differed by two categories or less in 96%, 92%, and 85% compared with 90%, 78%, and 79% of cases for chest radiographs for fibrosis, emphysema, and pleural disease respectively. There was better intraobserver repeatability for the HRCT scores than for the chest radiograph scores for all disorders. Multiple regression analysis showed that scores for interstitial fibrosis, emphysema, and pleural disease on chest radiographs and HRCT correlated to a similar degree with impairment of lung function.     

A new high resolution computed tomography scoring system for pulmonary fibrosis, pleural disease, and emphysema in patients with asbestos related disease.

The aim of this study was to describe a scoring system for high resolution computed tomographic (HRCT) scans analogous to the International Labour Office (ILO) scoring system for plain chest radiographs in patients with asbestos related disease. Interstitial fibrosis, pleural disease, and emphysema were scored, the reproducibility and the interobserver agreement using this scoring system were examined, and the extent of the various types of disease was correlated with measurements of lung function. Sixty asbestos workers (five women and 55 men) mean age 59 (range 34-78) were studied. The lungs were divided into upper, middle, and lower thirds. An HRCT score for the extent of pleural disease and pulmonary disease in each third was recorded in a way analogous to the International Labour Office (ILO) method of scoring pleural and parenchymal disease on chest radiographs. A CT score for the extent of emphysema was also recorded. Pleural disease and interstitial fibrosis on the plain chest radiographs were assessed according to the ILO scoring system. A chest radiographic score for emphysema analogous to that used for HRCT was also recorded. Two independent readers assigned HRCT scores that differed by two categories or less in 96%, 92%, and 85% compared with 90%, 78%, and 79% of cases for chest radiographs for fibrosis, emphysema, and pleural disease respectively. There was better intraobserver repeatability for the HRCT scores than for the chest radiograph scores for all disorders. Multiple regression analysis showed that scores for interstitial fibrosis, emphysema, and pleural disease on chest radiographs and HRCT correlated to a similar degree with impairment of lung function.     

Undifferentiated connective tissue disease: clinical and serological profile of 578 patients followed for five years: disease course, prognosis and therapy

INTRODUCTION: Evolution of immunopathological diseases is usually slow and progressive. The term the undifferentiated connective tissue disease (UCTD) is used to describe the phase preceding a defined connective tissue diseases (CTD). AIMS: The objective of this work was evaluate the clinical and serological profile of patients with UCTD, who had been followed between 1994-1999. They have investigated the frequency and the type the developed autoimmune diseases from UCTD. PATIENTS: A total of 578 UCTD patients were evaluated. RESULTS: In 143/578 patients (24.7%) with the UCTD differentiated to systemic connective tissue diseases (28 systemic lupus erythematosus, 26 mixed connective tissue disease, 19 progressive systemic sclerosis, 3 polymyositis/dermatomyositis, 45 Sj?gren syndrome, and 22 systemic vasculitis). 86.7 percent (124/143) of the systemic connective disease developed in first two years of UCTD. The condition of 435/578 (75.2%) remained UCTD after 5 years, among them in 82 patients with UCTD was regression of the symptoms. The presence of the fever and anti-DNS antibodies correlated with SLE (P = 0.0104, Fisher exact test), arthritis/arthralgia and anti-RNP antibodies with MCTD (P = 0.0302), Raynaud phenomenon and ANA positivity with PSS (P = 0.0144), xerostomia/xerophtalmia and anti-SSA/SSB antibodies with Sj?gren syndromes (P = 0.0144). CONCLUSIONS: The UCTD in our patients seem to represents an dynamic phase, one part of the patients show progression to definite connective tissue diseases, one part show regression, and on part of the patients stay in UCTD phase.     

Nitrite plasma levels in chronic lung disease patients

BACKGROUND: The authors carried out a study in a group of lung disease patients, about the behaviour of the plasmatic levels of nitrites (stable, specific and irreversible end-products of nitric oxide). METHODS: The series consisted of 13 male patients (mean age 65 +/- 7 years) with chronic obstructive pulmonary disease with type 1 respiratory failure; 33 male subjects (mean age 58 +/- 5 years) without internistic disease were considered as controls. For each subject the determination of nitrite plasma levels by the Gutman and Hollywood method based on the Griess colorimetric reaction was performed. RESULTS: The mean value of the plasmatic nitrites was significantly reduced (p < 0.05) as compared to the controls (11 +/- 0.48 mumol/l vs 21 +/- 0.92 mumol/l). CONCLUSIONS: The authors hypothesized that in chronic lung disease patients there would be a condition of initial pulmonary hypertension; in this condition long-term endothelium-dependent nitric oxide production, aimed at the vasodilating effects with secondary excessive exhaled amount of NO, might cause a reduction in nitrite plasma levels. These levels may represent an early marker of pulmonary hypertension and suggest interesting therapeutic treatments through inhalation of exogenous NO.     

类风湿关节炎患者肺泡灌洗液检测结果分析

目的通过对类风湿关节炎（RA）患者进行高分辨CT（HRCT）和肺泡灌洗液检测（BLAF）,探讨诊断类风湿肺间质病变的有效方法。方法选择RA患者31例,进行高分辨CT、肺泡灌洗液检测。结果 31例患者中,HRCT检测发现肺间质病变1例（阳性率为3.2%）,BLAF检测发现肺间质病变4例（阳性率为12.9%）;BLAF的肺间质病变阳性检出率高于HRCT,但差异无统计学意义（χ2=1.957 9,P〉0.05）。结论 BLAF是探询早期发现及明确类风湿性关节炎引发呼吸系统损害的更为有效的检查方法,比HRCT能更好地明确肺间质病变病变的病理类型,有助于治疗方案的制定。     

Computed tomography of diffuse lung disease

The place of computed tomography (CT) scanning in the detection of pulmonary nodules is now well established. Due to the increasing spatial resolution of CT and shorter scan times, patients with diffuse lung disease can now be examined more effectively. Narrow section CT demonstrates the morphological detail of some diffuse lung diseases at an earlier stage than conventional radiography.     

Interstitial Lung Disease and Profound Hypoxaemia in a Severely-malnourished Child with Very Severe Pneumonia and Potential Lymph-node Tuberculosis: An Uncommon but Serious Co-morbidity

A nine-month old boy was initially admitted at the Acute Respiratory Infection Unit of Dhaka Hospital of icddr,b and soon after transferred to the Intensive Care Unit of the same hospital. The boy had problems of very severe pneumonia (confirmed by radiology), severe hypoxaemia, severe malnutrition, and Down''s syndrome. The patient was treated according to the hospital protocol for the management of pneumonia and malnutrition. During the hospital stay, hypoxaemia was persistent with very little improvement of pneumonia; a number of differentials, such as pneumocystis jirovecii pneumonia, lymph-node tuberculosis, were added to the problems. Subsequently, the patient''s hypoxaemia improved with the empirical use of antitubercular drugs. However, the patient again developed persistent hypoxaemia and, after unsuccessful treatment for a hospital-acquired pneumonia, the problems further expanded to include interstitial lung disease (ILD). This was confirmed by high-resolution computed tomography, and the patient was treated with prednisolone for 6 months, along with antitubercular drugs. He fully recovered from ILD, hypoxaemia, and pneumonia both clinically and radiologically. Therefore, severely-malnourished children having wet cough and pneumonia with persistent hypoxaemia should be assessed for the possible existence of interstitial lung disease. This may help provide a prompt and appropriate management to reduce morbidity and deaths in such patients.Key words: Hypoxaemia, Infant, Interstitial lung disease, Lymph-node tuberculosis, Severe malnutrition, Very severe pneumonia     

碎石路征—83例薄层CT表现

目的：通过列举导致碎石路征表现各类疾病,比较其薄层CT的表现与组织病理学表现的关系。方法：回顾性的分析309医院放射科CT扫描数据库有关碎石路征报道的胸部薄层CT83例患者资料,对碎石路征表现的病例进行研究。结果：83例均表现为碎石路征的病例,包括感染35例（细菌感染6例,病毒性感染16例,真菌感染1例,混合感染12例）;急性呼吸窘迫综合征（ARDS）4例;急性肺水肿3例;间质性肺疾病（UIP,NSIP）18例;腺癌3例;淋巴管癌病3例;淋巴瘤肺浸润2例;放射性肺炎5例;结节病1例;肺泡蛋白沉积症4例;肺泡出血4例;类脂性肺炎1例。结论：碎石路征是一种非特异性的薄层CT征象,可见于感染性、肿瘤性以及一些原因不明的疾病。如果缩小鉴别诊断范围,则可提示某种疾病的诊断。     

Case report: a case of wood-smoke-related pulmonary disease

CONTEXT: Biomass serves as a major fuel source for >50% of the world's population. The global burden of disease attributed to indoor air pollution from biomass combustion accounts for approximately 3% of worldwide disability-adjusted life-years lost. This is due to pneumonia in children and chronic obstructive pulmonary disease and lung cancer in women. CASE PRESENTATION: A 53-year-old man from Mexico was referred to the pulmonary clinic for evaluation of chronic productive cough and pulmonary nodules. In his youth, he worked at a charcoal plant in Mexico, where he burned wood and was exposed to massive amounts of smoke. His evaluation revealed thickened bronchovascular bundles with nodules on thoracic computed tomography, dark black plaques in large airways on bronchoscopy, and carbon-laden macrophages and fibrotic scars on lung biopsy. DISCUSSION: The patient was diagnosed with "hut lung," a term that refers to the noninfectious, nonmalignant respiratory manifestations of chronic, high-level exposures to biomass smoke. This is the first reported case of hut lung associated with charcoal production. This case highlights that histopathologic abnormalities of the lung parenchyma may be present in patients with only mild symptoms and that clinical progression is likely a function of both the duration and intensity of exposure. RELEVANCE TO CLINICAL PRACTICE: As residents of lesser developed countries continue to be exposed to high levels of biomass smoke at work or at home and continue to immigrate to developed countries, it is important that health care providers in developed countries be aware of biomass-smoke-related pulmonary disease.     

粮谷尘致肺损害的实验研究

粮谷尘为混合性粉尘，含游离ＳｉＯ２９．７７％，分离培养出普通高温放线菌。染尘动物肺脏早期病变主要为小灶性肺泡炎、灶性间质性肺炎、异物肉芽肿等，大、中支气管壁脱颗粒肥大细胞增多。９０ｄ以后，上述病变明显减少，但逐渐出现少数小的细胞结节，间质轻度纤维组织增生、支气管慢性炎症和小叶中央型肺气肿样病变，观察１ａ（年）未见明显纤维化。     

A retired shipyard worker with rapidly progressive pulmonary interstitial fibrosis

We present a case of progressive interstitial fibrosis in a retired shipyard worker who was exposed to asbestos during the postwar era of the late 1940s and 1950s, when asbestos exposures in the workplace were not regulated. Forty years later, at 63 years of age, the patient presented with restrictive lung disease. The patient was diagnosed with asbestos-related pleural disease and parenchymal asbestosis. He remained stable for the next 7 years, but then he began to manifest rapid clinical progression, which raised the possibility of an unusual variant of asbestosis, a concomitant interstitial process, or an unrelated disease. Lung biopsy was not undertaken because of the patient's low pulmonary reserve and limited treatment options. An empiric trial of oral steroids was initiated, but his pulmonary status continued to deteriorate and he died of pulmonary failure at 72 years of age. Many diseases result in pulmonary interstitial fibrosis. Ideally, open lung biopsy should be performed, but this procedure inevitably causes complications in many patients with end-stage restrictive lung disease. Furthermore, while the presence of asbestos bodies in tissue sections is a sensitive and specific marker of asbestos exposure, neither this finding nor any other charge is a marker indicative of asbestosis or the severity of asbestosis. With the enactment of the Asbestos Standard in the United States, asbestos exposures have been decreasing in this country. However, industries that produce asbestos products and wastes continue to expand in developing countries. Prevention of asbestos-related lung disease should be a global endeavor, and asbestos exposures should be regulated in both developed and developing countries.