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PubMed1997

Exposure and mineralogical correlates of pulmonary fibrosis in chrysotile asbestos workers.

OBJECTIVES: The relation between lifetime cumulative exposure to asbestos, pathological grade of pulmonary fibrosis, and lung burden of asbestos at death, was explored in a necropsy population of former workers in a chrysotile asbestos textile plant in South Carolina. METHODS: Estimates of cumulative, mean, and peak exposures to asbestos were available for 54 workers. Necropsy records and lung tissue samples were obtained from hospital files. Matched control cases were selected from consecutive necropsies performed at the same hospitals. The extent and severity of pulmonary fibrosis was graded on tissue sections. Mineral fibres in lung tissue were characterised by transmission electron microscopy combined with x ray spectroscopy. RESULTS: A significant positive correlation (r = 0.67, P < 0.0001) was found between lifetime cumulative exposure to asbestos and total lung burden of asbestos fibres. This relation was also found for the individual types of asbestos associated with the exposure: chrysotile and tremolite. Pulmonary fibrosis was correlated with both cumulative exposure to asbestos (r = 0.60, P < 0.01) and the concentration of asbestos fibres in the lung (r = 0.62, P < 0.0001). The concentration of tremolite fibres in the lung provided a better estimate of lung fibrosis than did the concentration of chrysotile. Asbestosis was usually present in asbestos textile workers with more than 20 fibre-years cumulative exposure. The lengths and aspect ratios of chrysotile asbestos, but not amphibole asbestos, were greater in the lungs of asbestos fibre workers than in the control population. Textile workers with lung cancer had significantly greater cumulative exposures and fibrosis scores than workers without lung cancer. CONCLUSIONS: Both cumulative exposure to asbestos and lung fibre burden are strongly correlated with severity of asbestosis. The data also support the hypothesis that the high prevalence of asbestosis and lung cancer in this population resulted from exposure to long fibres of chrysotile asbestos in the workplace.

Asbestos, Serpentine↗
PubMed2004

[Regulative effect of STAT1 on inflammation of lung tissue in bleomycin-induced rat interstitial pulmonary fibrosis].

OBJECTIVE: To investigate the role of signal transducer and activator of transcription 1 (STAT1) in alveolar macrophage (AM) in bleomycin-induced rat pulmonary fibrosis. METHODS: Fifty adult female Wistar rats were randomly divided into two groups. The rats of BLM group were intratracheally instilled with bleomycin (BLM), and those of the control group with normal saline(NS). The kinetic change of STAT1 activation and the intercellular adhesion molecule-1 (ICAM-1) expression in AM were examined. RESULTS: STAT1 was slightly activated in AM of NS group. After bleomycin treatment, the STAT1 activation of AM significantly increased on day 1, reached the peak value on day 7, and then gradually decreased, yet it remained significantly above the value of the NS group on day 28 (P < 0.05). There were a few positive staining cells for ICAM-1 expression in AM of NS group. After intratracheal instillation of bleomycin, the number of positive staining cells for ICAM-1 expression significantly increased on day 1, reached the peak value on day 7, gradually decreased from then on, but still it was higher than that of the NS group on day 28 (P < 0.05). There was a significant correlation between STAT1 activation and ICAM-1 expression in AM (r = 0.913, P < 0.01); moreover, ICAM-1 expression in AM was significantly correlated with the degree of inflammation in lung tissue (r = 0.947, P < 0.01). CONCLUSION: STAT1 was found abnormally activated in AM in bleomycin-induced rat interstitial pulmonary fibrosis (IPF). The abnormal STAT1 activation may play a role in the pathogenesis of acute alveolitis and pulmonary fibrosis.

Animals↗
PubMed1995

[Progressive pulmonary fibrosis in a case of Sjögren's syndrome with asbestos exposure].

A 62-year-old man had an occupational history of exposure to asbestos for 27 years (1947-1974). Dry mouth, dry eyes and swollen gums were noted in 1974. Sjögren's syndrome was diagnosed in 1975. The chest radiograph in 1984 showed bilateral pleural thickening and small reticular opacities in the left lower lung field. In 1990, he complained of dyspnea and cough, and diffuse interstitial pneumonia was diagnosed. While being treated for diffuse interstitial pneumonia, pulmonary fibrosis progressed rapidly, and advanced pulmonary fibrosis was obvious in 1992. Laboratory data showed hyper gamma-globulinemia (36.8%) and a high level of IgG (3772 mg/dl) in 1976. These values decreased to within the normal ranges during the subsequent clinical course. The results of lymphocyte subset counts in 1988 were normal. With the progression of diffuse interstitial pneumonia in 1990, the lymphocyte subset counts showed a low percentage of CD4 (19.2%), a low CD4/8 ratio (0.46), and a low percentage of CD20 (26.8%). In 1992, as pulmonary fibrosis progressed despite treatment, the disorder of lymphocyte subsets became worse. The CD4 percentage was very low (5.0%), as was the CD4/8 ratio (0.08), and the percentage of CD20 (1.4%); the CD8 percentage was high (64.7%), as was the percentage of Leu 7 (49.0%). These immunologic and pulmonary changes could be not explained by Sjögren's syndrome. Determining what factor induced these changes is difficult, but asbestos exposure is a likely cause.

Asbestos↗
PubMed2006

Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) and sarcoidosis are common diagnoses in patients attending chest clinics, but little is known about the epidemiology of these diseases. We used data from a general practice database to provide information on the current incidence of IPF and sarcoidosis in the UK. METHODS: Data were extracted for all patients with a diagnosis of IPF or sarcoidosis between 1991 and 2003. The whole population of the database was used to calculate disease incidence stratified by age, sex, region, and time period. Poisson regression was used to compare the incidence between populations and Cox regression was used to compare survival between populations. RESULTS: 920 cases of IPF (mean age 71 years, 62% male) and 1019 cases of sarcoidosis (mean age 47 years, 47% male) were identified. The overall incidence rate per 100 000 person-years was 4.6 for IPF and 5.0 for sarcoidosis. The incidence of IPF increased progressively between 1991 and 2003 (p<0.00001), and was highest in Northern England and Scotland (p<0.0001). The survival of patients with IPF was stable over time. In contrast, the incidence of sarcoidosis was highest in London, West Midlands and Northern Ireland and remained stable over time. CONCLUSIONS: The incidence of IPF has more than doubled between 1990 and 2003; this is not due to the ageing of the UK population or an increased ascertainment of milder cases. The incidence of sarcoidosis has not changed during this time period. Our findings suggest that more than 4000 new cases of IPF and 3000 new cases of sarcoidosis are currently diagnosed each year in the UK.

Adult↗
PubMed1999

ICAM-1 mediates lung leukocyte recruitment but not pulmonary fibrosis in a murine model of bleomycin-induced lung injury.

Bleomycin-induced lung injury has been extensively used as a model of interstitial pneumonia and pulmonary fibrosis. Intercellular adhesion molecule (ICAM)-1 is a ligand for lymphocyte function-associated antigen (LFA)-1alpha and has been shown to be required for leukocyte migration into inflamed areas. The purpose of this report was to investigate the role of the ICAM-1/LFA-1alpha pathway in a murine model of bleomycin-induced lung injury. Animals received 75 mg x kg(-1) bleomycin (BLM) i.v. followed by treatment with phosphate-buffered saline (BLM group), anti-ICAM-1 and LFA-1alpha monoclonal antibodies (mAb) (BLM+mAb group). Inflammatory cell counts of bronchoalveolar lavage (BAL) fluid, hydroxyproline content and histological findings were compared between these groups. In the BLM group, significant increases in total cell count, macrophage count and neutrophil count of BAL fluid were observed on days 7 and 14. In the BLM+mAb group, bleomycin-induced accumulation of neutrophils was significantly reduced on days 7 and 14 (p<0.01). However, the administration of mAb to ICAM-1 and LFA-1alpha did not decrease the lung hydroxyproline content or the histopathological fibrosis grading score, indicating that the antagonism of ICAM-I and LFA-1alpha did not attenuate bleomycin-induced pulmonary fibrosis. This study suggests that the intercellular adhesion molecule-1/lymphocyte function-associated antigen-1alpha pathway mediates the accumulation of inflammatory cells in the injured lung caused by bleomycin; however, other mechanisms are important for the subsequent development of pulmonary fibrosis.

Animals↗
PubMed2001

Idiopathic interstitial pneumonias: a re-appraisal of idiopathic pulmonary fibrosis.

Over the last 30 years the clinical and histopathological definitions of the diffuse lung diseases have evolved considerably. Initially pathological entities were defined in parallel with clinico-radiological diagnoses, but these have more recently become consolidated into a more meaningful combined classification. These refinements have impacted on the diffuse lung diseases in particular, and have defined individual diseases more precisely than in previous classifications in which a number of distinct entities had been grouped together and mistaken for idiopathic pulmonary fibrosis, resulting in much confusion. The American Thoracic and European Respiratory Societies' committees, charged with the task of defining the idiopathic interstitial pneumonias, have recently published a statement on idiopathic pulmonary fibrosis, and a statement on the other idiopathic interstitial pneumonias should follow this year. Of these diseases, idiopathic pulmonary fibrosis is the most lethal, and this review deals with the impact that the changes in the nomenclature will have on our understanding of this and the other diseases with which idiopathic pulmonary fibrosis was previously confused and explores the implications of our new understanding on clinical practice. It also attempts to highlight areas of previous dogma in the literature that now need to be re-considered in the context of these more recent statements.

Anti-Inflammatory Agents↗
PubMed1976

[A contribution to the symptomatology of diffuse interstitial pulmonary fibrosis (DIPF) (author's transl)].

Diffuse interstitial pulmonary fibrosis was detected in 64 patients (33 males and 31 females). The diagnosis was obtained in each case from the anamnesis, the clinical findings, the chest X-ray, disturbances of the respiratory function, and from the progress of the disease. The analysis of the material produced the following parameters ranging from indicative to typical: Dry cough was reported by 70% of the patients as being trouble-some. Dyspnoea on exertion and at rest existed in 95%, and gradually increased during the progress of the disease in 76,5% of these cases. On auscultation ear-near crepitant rale was heard in 78% above the affected pulmonary parts. In 62 patients (96,5%) the radiogram showed disseminated streaky-reticular as well as finely to coarsely mottled infiltrations. In more than half of these cases (53%) an increase in severity was observed during the follow-up. A predominantly restrictive disturbance of ventilation existed in 85,5%. Exclusive restriction was found in 65,5%. Effort hypoxemia was detected in 92% of our cases which was especially impressive when the arterial oxygen pressure at rest was within the normal range. The electrocardiographic findings seemed to be less unequivocal. All in all it can be stated that in the synopsis of its manifestation DIPF can be positively identified by clinical means. This fact as well as therapeutical problems are discussed taking available publications into consideration, and respective conclusions are drawn for the clinician.

Aged↗
PubMed2003

The role of microvascular injury in the evolution of idiopathic pulmonary fibrosis.

Interstitial lung disease compatible with idiopathic pulmonary fibrosis (IPF) developed in 19 previously healthy patients. Although interstitial and/or honeycomb parenchymal fibrosis was present in all, there were patchy areas of paucicellular septal capillary injury along with corroborative direct immunofluorescent evidence of a humorally mediated microvascular injury syndrome. Significantly elevated factor VIII levels were seen in 17 of 18 patients tested. Antiphospholipids were present in all 18 patients tested, comprising antibodies of phosphatidylethanolamine, beta-2 glycoprotein, phosphatidylcholine, and/or phosphatidylserine. Anti-Ro and/or anti-ribonucleoprotein (RNP) antibodies were seen in 4 patients. Serologic evidence of infection with cytomegalovirus (CMV) was found in 9 patients and parvovirus B19 (B19) in 9 patients; 1 patient was not tested. Molecular studies revealed B19 DNA in 6 of 6 B19-seropositive patients. In situ hybridization studies revealed CMV RNA in pulmonary cells in patients with serologic evidence of active CMV infection despite the absence of cytopathic changes typical of CMV infection. Antiphospholipid antibodies, antiendothelial cell antibodies, and/or endotheliotropic viral infections related to B19 and CMV may be of pathogenetic importance to the evolution of IPF. This report underscores the potential importance of microvascular injury in the evolution of IPF.

Adult↗
PubMed1994

Treatment by human recombinant soluble TNF receptor of pulmonary fibrosis induced by bleomycin or silica in mice.

Intratracheal instillation of bleomycin (0.08 U) or silica (2 mg) to mice leads, after 15 days, to a patchy pulmonary fibrosis associated with a significant increase of the lung hydroxyproline. Since tumour necrosis factor (TNF) seems to be an important mediator in pulmonary fibrosis, we wondered whether this fibrosis might be prevented by a new TNF-alpha antagonist. Infusion of a 55 kD human recombinant soluble TNF receptor rsTNFR-beta, at a rate of 20 micrograms.day-1, prevented the bleomycin/silica induced increase of lung hydroxyproline content, as measured 15 days after instillation. Infusion of rsTNFR-beta was also effective in the treatment of an established fibrosis, i.e. administered 25 or more days after instillation of bleomycin or silica, since it reduced lung collagen content. Recombinant soluble TNFR-beta had no significant influence on the number of cells, mostly macrophages, recovered by bronchoalveolar lavage. The examination of histological sections indicated that the rsTNFR-beta reduced the proportion of areas of damaged lung and, in silicosis, the formation of nodules with a rich collagen content. This study suggests that rsTNFR-beta might be useful in the therapy of pulmonary fibrosis.

Animals↗
PubMedDate not supplied

[Content of trace elements and steroid hormones in blood and tissues of internal organs in experimental pulmonary fibrosis induced by intratracheal administration of bleomycetin].

Pulmonary fibrosis was produced in rats by intratracheal administration of bleomycetin. The development of the fibrous process in the lungs was attended by marked reduction of the zinc content in the tissues of the viscera, plasma, and formed elements of the blood, as well as of corticosterone and testosterone in blood plasma. Respiratory insufficiency is a probable cause of the developing zinc-deficiency which leads to disturbed synthesis of steroid hormones. Injection of exogenous testosterone normalizes partly the level of steroid hormones in the blood, improves the general condition of the animals, and facilitates an increase of body weight.

Animals↗
PubMed2000

Idiopathic pulmonary fibrosis, clinical features in Thai patients.

Idiopathic pulmonary fibrosis (IPF) or cryptogenic fibrosing alveolitis (CFA) is the most common type of chronic diffuse parenchymal lung disease. We identified twenty patients who were diagnosed with this disease in Phramongkutklao Hospital. Clinical features of these patients were reported. All presented with dyspnea and the severity of dyspnea was grade 3-4 in 14 patients (70%). Every patient had bilateral basilar crackles on auscultation. The median survival time of newly diagnosed IPF in this study was only 16 months. Comparison of Thai patients with those of other reports from Western countries showed differences in severity of dyspnea and the median survival time.

Adult↗
PubMed2002

Idiopathic pulmonary fibrosis: new insights in its pathogenesis.

Idiopathic pulmonary fibrosis (IPF) is a unique type of chronic fibrosing lung disease of unknown etiology. The sequence of the pathogenic mechanisms is unknown, but the disease is characterized by epithelial injury and activation, the formation of distinctive subepithelial fibroblast/myofibroblast foci, and excessive extracellular matrix accumulation. These pathological processes usually lead to progressive and irreversible changes in the lung architecture resulting in progressive respiratory insufficiency and an almost universally terminal outcome in a relatively short period of time. While research has largely focused on inflammatory mechanisms for initiating the fibrotic response, recent evidence strongly suggests that disruption of the alveolar epithelium is an underlying pathogenic event. Although treatment to date has proved largely ineffective, this new approach has opened up several promising therapeutic avenues.

Collagenases↗
PubMed2006

Th1/Th2 cytokine gene polymorphisms in patients with idiopathic pulmonary fibrosis.

We investigated 30 patients with idiopathic pulmonary fibrosis (IPF) and 103 healthy volunteers for the cytokines polymorphisms of the IL-1alpha, IL-1beta, IL-1R, IL-1RA, IL-2, IL-4, IL-6, IL-10, IL-12, tumor necrosis factor-alpha, interferon-gamma, transforming growth factor-beta, IL-1beta, IL-2, IL-4, and IL-4RA genes. The strongest correlation of a genotype with the disease was found for gene polymorphisms at the promotor region of IL-4, where the CT genotypes at the positions (-590) and (-33) were more frequent in the IPF group (P < 0.0001, P(corr) < 0.0022; vs P < 0.0001, P(corr) < 0.0022). Our results support the idea of the pathogenic role of cytokine gene polymorphisms in the etiology and pathogenesis of IPF, with emphasize on the IL-4 promotor gene polymorphisms.

Adult↗
PubMed1994

Idiopathic pulmonary fibrosis. Epidemiologic approaches to occupational exposure.

Idiopathic pulmonary fibrosis (IPF) risk-related factors were epidemiologically investigated on the basis of 1,311 Japanese IPF autopsy cases selected from the annual complications of autopsy data records in Japan during a 12-yr period. Age and sex distribution of the subjects revealed a high peak in their seventh decade with males predominating. The IPF rate was more than two times higher (p < 0.01) among subjects engaged in occupations that exposed them to dust or organic solvents compared with those in other jobs. To ascertain job characteristics, an autopsy-case control study was conducted using other annual volumes of the autopsy data records and a similar tendency was observed. Then, a live-case control study was undertaken of 86 subjects with IPF. A significantly higher odds ratio was noted among metal production workers and miners compared with healthy and hospital control subjects (1.37 and 1.34, respectively, p < 0.01), and also a significantly lower odds ratio among subjects who frequently eat fish. Taken together with results of recent in vitro studies, the intrapulmonary deposition of hazardous dusts, especially metallic dusts, appears to play at least a partial role in initiating IPF.

Adolescent↗
PubMed1979

Collagenase in the lower respiratory tract of patients with idiopathic pulmonary fibrosis.

To test the hypothesis that idiopathic pulmonary fibrosis (IPF) is mediated through collagenase present in the lower respiratory tract, we used the fiberoptic bronchoscope to obtain fluid from the lower respiratory tract of 24 patients with IPF, 18 controls and nine patients with sarcoidosis. The fluid was analyzed for a variety of enzymes, including collagenase. Fifteen of 21 patients with IPF showed collagenase activity, whereas normal controls and patients with sarcoidosis showed none (P greater than 0.001, for all comparisons). In two patients with IPF who were re-evaluated after eight to 24 months, the collagenase activity was persistent. Fluid from patients with IPF also contained elevated levels of a non-specific neutral protease (P greater than 0.01 compared with controls), but there was no elastase activity in fluid from patients with IPF or from controls. The collagenase found in lavage fluid in IPF cleaved lung collagen into collagenase-specific TCA and TCB fragments. We conclude that in IPF the collagen of the lung is subjected to sustained lysis, followed by disordered resynthesis, and that the presence of active collagenase in the lower respiratory tract is a specific feature of the alveolitis associated with this disease.

Adult↗
PubMed1995

Increased procoagulant and antifibrinolytic activities in the lungs with idiopathic pulmonary fibrosis.

To elucidate the pathophysiology of idiopathic pulmonary fibrosis (IPF), we examined procoagulant (tissue factor:TF), fibrinolytic (tissue type plasminogen activator:t-PA and urokinase type plasminogen activator:u-PA) and antifibrinolytic (plasminogen activator inhibitor-1:PAI-1 and PAI-2) activities in bronchoalveolar lavage (BAL) supernatant fluids and BAL cell lysates obtained from IPF patients. The results indicated that TF levels in BAL supernatant fluids from IPF patients were higher than those of normal subjects, especially in patients with progressive disease, suggesting that TF levels in the lung correlate with disease activity. PAI-1 levels in BAL supernatant fluids were significantly higher in IPF patients than in normal subjects (1.7 +/- 4.1 vs 0 ng/mg protein). PAI-2 levels in BAL cell lysates were also significantly higher in IPF patients than those in normal subjects (14.4 +/- 12.2 vs 3.0 +/- 3.0 ng/mg protein). However, u-PA levels in both BAL supernatant fluids and BAL cell lysates did not differ between the two groups. These observations suggest that u-PA inhibition exceeded u-PA activity in alveolar lining fluid resulting in an antifibrinolytic condition. Immunohistochemical analysis showed that TF was intensely stained in cuboidal epithelial cells and PAIs were positively stained in alveolar macrophages (AMs) and cuboidal epithelial cells, suggesting that cuboidal epithelial cells as well as AMs contribute to the increased procoagulant and antifibrinolytic activities in the lungs of IPF patients.

Aged↗
PubMed2002

Hyperplastic epithelial foci in honeycomb lesions in idiopathic pulmonary fibrosis.

Seventy-two cases of idiopathic pulmonary fibrosis (IPF) were examined from 2856 consecutive autopsy cases at the Japanese Red Cross Medical Center in Tokyo from 1973-1996. Primary lung cancer had arisen in 31 of 72 cases of IPF (43%), significantly higher than the incidence in cases without IPF (8.1%) and in the cases with non-IPF chronic lung diseases (11.9%). Hyperplastic epithelial foci in the honeycomb lesions of IPF cases were significantly more prominent in the lower than in the upper lobe, in cases with or without lung cancer, and they were more prominent in the lower lobe of IPF with than in those without cancer. The length of hyperplastic epithelial foci in the lower lobe of IPF cases was longer than that in interstitial pneumonia-associated with collagen vascular diseases. There was a higher PCNA labeling index of hyperplastic epithelial foci in IPF cases than in cases of interstitial pneumonia-associated with collagen vascular diseases. The PCNA labeling index was almost the same between smokers and nonsmokers with IPF. Overexpression of p53 was observed in hyperplastic epithelial foci in honeycomb lesion of IPF. DNA ploidy analysis of hyperplastic epithelial foci in the paraffin sections of 12 IPF cases revealed aneuploidy patterns in eight cases. These results strongly suggest that accelerated cell proliferation occurs in the honeycomb lesion of IPF, and that regenerative epithelia becomes susceptible to carcinogenic agents in addition to the smoking effect.

Adult↗