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PubMed2002

Bleomycin-induced pulmonary fibrosis is attenuated in gamma-glutamyl transpeptidase-deficient mice.

To investigate repair mechanisms in bleomycin-induced pulmonary fibrosis, we used mice deficient in gamma-glutamyl transpeptidase (GGT-/-), a key enzyme in glutathione (GSH) and cysteine metabolism. Seventy-two hours after bleomycin (0.03 U/g), GGT-/- mice displayed a different inflammatory response to wild-type mice as judged by a near absence of neutrophils in lung tissue and bronchoalveolar lavage and a less pronounced rise in matrix metalloproteinase-9. Inflammation in GGT-/- mice consisted mainly of lymphocytes and macrophages. At 1 month, lungs from bleomycin-treated GGT-/- mice exhibited minimal areas of fibrosis compared with wild-type mice(light microscopy fibrosis index: 510 +/- 756 versus 1975 +/- 817, p < 0.01). Lung collagen content revealed a significant increase in bleomycin-treated wild-type (15.1 +/- 3.8 versus 8.5 +/- 0.7 microg hydroxy(OH)-proline/mg dry weight, p < 0.01) but not in GGT-/- (10.4 +/- 1.7 versus 8.8 +/- 0.8). Control lungs from GGT-/- showed a significant reduction of cysteine (0.03 +/- 0.005 versus 0.055 +/- 0.001, p < 0.02) and GSH levels (1.24 +/- 0.055 versus 1.79 +/- 0.065, p < 0.002). These values decreased after 72 hours of bleomycin in both GGT-/- and wild-type but reached their respective control values after 1 month. Supplementation with N-acetyl cysteine partially ameliorated the effects of GGT deficiency. These findings suggest that increased neutrophils and matrix metalloproteinase-9 during the early inflammatory response and adequate thiol reserves are key elements in the fibrotic response after bleomycin-induced pulmonary injury.

Acetylcysteine↗
PubMed2000

Pulmonary nocardiosis during immunosuppressive therapy for idiopathic pulmonary fibrosis.

Nocardiosis is a subacute or chronic suppurative infection caused by Nocardia species. Although it is more common in immunocompromised hosts, idiopathic pulmonary fibrosis (IPF) has not been recognized as a predisposing factor for nocardial infection. We report a case of IPF, in which pulmonary nocardiosis developed during treatment with prednisolone and cyclophosphamide. The risk of pulmonary nocardiosis may be increased in cases of IPF on immunosuppressive therapy. Since IPF often accompanies lung carcinoma, it is important to correctly differentiate nocardiosis from carcinoma.

Aged↗
PubMedDate not supplied

[Pulmonary fibrosis caused by inhalation: silicosis].

Silicosis is an important disease not only for its prevalence and the degree of pulmonary insufficiency it entails but also because it provides a natural model of interstitial fibrotic disease in the lung which is of known origin. This can, in turn, help us understand the pathogenic nature of a great number of pulmonary fibroses whose cause is unknown. The fifty postmortem studies which we describe correspond to miners who had worked in underground mines in the mountainous region near Cartagena (SE Spain) for times ranging from 5 to 36 years. The autopsies showed that they had been exposed to dust containing abundant metallic particles, particularly iron oxide (mixed dust). Although the pathogenic action was related with silica, it was also clearly modified by the composition of the dust associated with it. The basic lesions which are produced in the lung after inhalation of silica (< 5 microns diameter) are coniosis, fibroconiosis and sclerohyalinosis. The sclerohyalino nodules are characterized by abundant collagenization and hyalinization, different types of which can be distinguished according to the disposition of the collagenous fibres. Nodular lesions gradually grow in size even when exposure to dust has ceased. As they grow they get nearer until they join to form conglomerate masses. When the diameter exceeds 3 cm this is called massive fibrosis. Pulmonary tuberculosis was found in 27% of cases. This took the form of lesions, which could be associated to or independent of silicotic lesions. Besides evolutive nodular lesions, a patient suffering from silicosis may show other unspecific lesions which must be correctly evaluated for a more correct clinical-pathological assessment, since, clinically, the respiratory function may be profoundly affected although such silicotic damage may be not very noticeable by radiological examination. Silicosis of the liver and spleen was not infrequent in the autopsies carried out, with basic lesions in all evolutive states being observed, the most evolved in the spleen. This means that silicosis should be considered as a systemic illness.

Animals↗
PubMed1983

Radiation-induced pulmonary fibrosis resolves spontaneously if dense scars are not formed.

The relation of static compliance of excised lungs to collagen accumulation and histologic fibrosis was examined in Syrian hamsters inhaling sufficient 238PuO2 particles to achieve initial lung burdens of 50 or 100 nCi. Control animals were exposed to nonradioactive aerosols. Irradiated lungs from hamsters at both dose levels had compliance reduced to the same extent at point of maximal reduction. However, collagen accumulation was more closely related to 238Pu exposure level than the compliance measurements. Histologic examination revealed both diffuse alveolar thickening and some dense fibrous scars, the former predominating at lower dose levels. Hamsters exposed to 50 nCi 238PuO2 showed normal collagen content and static lung compliance with minimal histologic fibrosis 288 days after exposure. In contrast, hamsters exposed to 100 nCi had significant pulmonary fibrosis at that time and the highest incidence of dense scars at any time period. Such findings are consistent with a stiffening of lung parenchyma. They suggest that the diffuse interstitial fibrosis developed by this injury resolves spontaneously; dense fibrous scars, however, do not.

Animals↗
PubMedDate not supplied

Correlation of ionizing irradiation-induced late pulmonary fibrosis with long-term bone marrow culture fibroblast progenitor cell biology in mice homozygous deletion recombinant negative for endothelial cell adhesion molecules.

Ionizing irradiation damage to the lung is associated with an acute inflammatory reaction, followed by a latent period and then late effects including predominantly pulmonary fibrosis. The cells mediating fibrosis have recently been shown to derive from the bone marrow hematopoietic microenvironment. Initiation of late pulmonary irradiation lung damage has been correlated with up-regulation of VCAM-1 and ICAM-1 in pulmonary endothelial cells, followed by infiltration of macrophages and bone marrow-derived fibroblasts forming the fibrotic lesions of organizing alveolitis/fibrosis. To determine whether the absence of expression of VCAM-1, ICAM-1, or other adhesion molecules known to be relevant to inflammatory cell attachment to lung endothelial cells was associated with a decrease in irradiation-induced lung fibrosis, homozygous deletion recombinant knockout mice lacking each of several adhesion molecules were tested compared to littermates for survival and development of organizing alveolitis following 20 Gy irradiation to both lungs. Bone marrow culture longevity has been shown to be a parameter, which correlates with both hematopoietic stem cell reserve and the integrity of fibroblast progenitors of the supportive hematopoietic microenvironment; radiation lung survival data were correlated to longevity of hematopoiesis in long-term bone marrow cultures established from tibia and femur bone marrow of the same mice. Homozygous deletion recombinant negative mice including VCAM-1-/-, ICAM-1-/-, E-Selectin-/-, or L-Selectin-/- were irradiated to 20 Gy to both lungs and followed for survival and percent organizing alveolitis at time of death compared to each normal littermate. A significant increase in survival (median 190 days) was detected with L-Selectin-/- compared to littermate control mice (median 140 days) or other groups. Long-term bone marrow cultures from L-Selectin-/- mice showed no detectable difference in marrow fibroblasts or hematopoietic cell biology compared to normal littermates; however, E-Selectin-/- mouse long-term bone marrow cultures showed an increase in total cumulative cell production (1.7 x 10(8) cells per flask) compared to bone marrow cultures from normal littermates (1.8 x 10(6) cells per flask) or other groups. As additional controls, transgenic Sod2 mouse long-term bone marrow cultures and those from HPV16, E6 and E7 cytokeratin 14 transgenic mice were also tested. No detectable difference in hematopoiesis was noted in these cultures compared to littermates. The results suggest a complex pattern of involvement of endothelial specific adhesion molecules and marrow fibroblasts in the cell biologic events associated with late irradiation pulmonary fibrosis.

Animals↗
PubMed2002

Hepoxilin analogs inhibit bleomycin-induced pulmonary fibrosis in the mouse.

Bleomycin has been suggested to incite plasma extravasation and influx of inflammatory cells leading to pulmonary fibrosis. We hypothesized that stable analogs of the 12-lipoxygenase product, hepoxilin, may attenuate these effects. We initially investigated the effects of the four hepoxilin analogs (PBT-1 to -4) coadministered intradermally with bleomycin and found that PBT-1 and -2 significantly opposed the vascular permeability effects of bleomycin in rat skin. We subsequently tested the hepoxilin analogs for their actions in opposing the intratracheal bleomycin-evoked acute inflammatory phase of lung fibrosis in the mouse, characterized by a marked accumulation of macrophages and an increase in the rate of collagen synthesis and deposition. We found that the bleomycin-evoked effects on macrophage influx were inhibited by all the hepoxilin analogs (PBT-1, -3, and -4 > PBT-2) administered i.p. for 8 days. Increased total lung collagen was completely abrogated by PBT-1 and -2, whereas PBT-3 and -4 had little effect. A dose-response study with PBT-1 indicated that the effective dose for inhibition of bleomycin-induced inflammatory and histological changes was below 10 microg/day. These studies demonstrate an in vivo action of stable analogs of hepoxilin and support an effect on inflammation and vascular permeability from these novel compounds, especially for PBT-1.

8,11,14-Eicosatrienoic Acid↗
PubMed1996

[Diagnostic value of exercise diffusion capacity test on diffuse pulmonary fibrosis].

Diffusion capacity for carbon monoxide of the lung (DLCO) before-and-after exercise test and 5 routine pulmonary function tests were conducted in 16 patients with diffuse pulmonary fibrosis (DPF), 19 patients with interstitial pneumonia (IP), 17 patients with COPD, and 22 normal subjects. The data showed: in normal subjects the DLCO after exercise increased significantly compared with before (P < 0.001). But in DPF group the DLCO before exercise was below the normal range, and it went down further after exercise, the decreasing rate being 17.95% (P < 0.001). The DLCO in IP and COPD groups did not significantly change before and after the exercise. Two cases of interstitial pneumonia, who had a reduced DLCO after exercise, were followed up for 0.5-1.0 year, and both patients developed into DPF by that time. The results suggest that the DLCO measure before and after exercise test may have important value for DPF diagnosis, and a reduced DLCO after exercise test in IP patients may warn the development from IP into DPF.

Adult↗
PubMed2005

[Lung resections for lung cancer with idiopathic pulmonary fibrosis].

PURPOSE: The aim of this study was to investigate the postoperative complications after lung resections for lung cancer with idiopathic pulmonary fibrosis (IPF). MATERIAL AND METHODS: There were 23 patients who underwent lung resections for lung cancer with IPF. There were 8 major complications. Acute exacerbation of IPF occurred in 4 cases, pulmonary edema in 1 case, bronchofistula in 1 case, bacterial pneumonia in 1 case, prolonged hypoxia in 1 case. Three cases died due to acute exacerbation of IPF (2 cases) and bronchofistula (1 case). RESULTS: There were 4 complications among 7 patients who underwent wedge resections and 4 complications among 16 patients who underwent lobectomy. All the 4 complicated cases who underwent wedge resections had low preoperative percent forced vital capacity (%VC) for 79+/-6%. For the patients who had lobectomy, the preoperative %VC and predicted postoperative %VC was significantly different between the 2 groups of complicated patients and uncomplicated ones (p < 0.05). For the prevention of acute exacerbation of IPF, we used clarithromycin in 11 cases, steroid in 2 cases, ulinastatin in 2 cases. However, the acute exacerbation was occurred in 4 cases. CONCLUSIONS: For the patients of lung cancer with IPF who had low preoperative %VC, even wedge resections should be carefully indicated.

Aged↗
PubMed1990

[Increased oxidant production by peripheral blood granulocytes in patients with idiopathic pulmonary fibrosis].

Oxidant production by peripheral granulocytes stimulated with phorbol myristate acetate (PMA) was investigated in 11 patients with idiopathic pulmonary fibrosis (IPF), 6 with interstitial pneumonia associated with collagen vascular disease (IP-CVD), 8 with sarcoidosis and 12 healthy subjects. Oxidant production was examined by flow cytometry using dichlorofluorescein diacetate. The reactivity of granulocytes to PMA was assessed according to the ratio between mean fluorescent intensity of granulocytes stimulated maximally with PMA and that without PMA, (stimulation index: S.I.). The concentration of PMA that induced half maximal fluorescent intensity of granulocytes (PC 1/2 max) was used as the index of sensitivity. The S.I. was 7.2 +/- 0.45 in IPF, 6.3 +/- 0.6 in IP-CVD, 6.0 +/- 0.71 in sarcoidosis, and 5.8 +/- 0.2 in healthy subjects. However differences between groups were not significant. PC 1/2 max was 7.3 +/- 2.1 ng/ml in IPF, 9.1 +/- 3.0 ng/ml in IP-CVD, 12 +/- 6.9 ng/ml in sarcoidosis and 16.1 +/- 5.8 ng/ml in healthy subjects. There was significant difference between IPF and healthy subjects (p less than 0.05) indicating that peripheral granulocytes in patients with IPF are more highly sensitive to PMA than healthy subjects.

Adult↗
PubMed2005

Quality of life during one-year observation of patients with idiopathic pulmonary fibrosis awaiting lung transplantation.

The aim of the study was to evaluate the quality of life and its changes in patients with idiopathic pulmonary fibrosis (IPF), waiting for lung transplantation. Sixteen IPF patients, who qualified for lung transplantation, and 14 chronic obstructive pulmonary disease (COPD) patients on long term oxygen therapy, taken as a reference group, were assessed. The quality of life was estimated twice, using an SF-36-questionnaire, at the time of qualification for lung transplantation and 12 months later. The IPF and reference patients had similarly profound disturbances in lung function and arterial blood gas content. The IPF patients had a statistically greater score in their physical functioning (PF; 45 vs. 18), the role of limitations due to physical problems (RP; 43 vs. 11), social functioning (SF; 58 vs. 30), and the role of emotional limitations in everyday life (RE; 65 vs. 12) than the reference ones. After a year's observation, there were no differences in SF-36 questionnaire results, except for the role of limitations due to physical problems (RP). A correlation was noted between spirometry and blood gas results and SF-36 questionnaire results in IPF patients qualified for lung transplantation. There was a positive correlation between the partial pressure of oxygen (PaO(2)), on one side, and mental and general health, on the other. There also were positive correlations between FEV1 and SF and FEV1 and the level of bodily pain (BP). We conclude that the SF-36 questionnaire is a sensitive tool to assess the quality of life in IPF patients qualified for lung transplantation. The information gained can help assess the severity of the disease, clinical symptoms, and functional impairment in these patients.

Activities of Daily Living↗
PubMed2006

Racial and ethnic disparities in idiopathic pulmonary fibrosis: A UNOS/OPTN database analysis.

We previously reported poorer survival among non-Hispanic blacks and Hispanics with idiopathic pulmonary fibrosis (IPF) compared to non-Hispanic whites at our center. In the current study, we hypothesized that these disparities would exist in a nationwide cohort of wait-listed patients with IPF. We performed a retrospective cohort study of 2635 patients with IPF listed for lung transplantation between 1995 and 2003 at 94 transplant centers in the United States. The age-adjusted mortality rate was higher among non-Hispanic blacks [hazard ratio (HR) = 1.24, 95% confidence interval (CI) 1.06-1.45, p = 0.009] and Hispanics (HR = 1.29, 95% CI 1.06-1.56, p = 0.01) compared to non-Hispanic whites. These findings persisted after adjustment for transplantation, medical comorbidities and socioeconomic status. Worse lung function at the time of listing appeared to explain some of these differences (HR for non-Hispanic blacks after adjustment for forced vital capacity percent predicted = 1.16, 95% CI 0.98-1.36, p = 0.09; HR for Hispanics = 1.21, 95% CI 0.99-1.48, p = 0.056). In summary, black and Hispanic patients with IPF have worse survival than whites after listing for lung transplant.

Ethnicity↗
PubMed1995

Clinical characteristics of synchronous multiple lung cancer associated with idiopathic pulmonary fibrosis. A review of Japanese cases.

To define the clinical characteristics of multiple lung cancer (LC) associated with idiopathic pulmonary fibrosis (IPF), we reviewed 154 LC patients associated with IPF: 23 patients with synchronous multiple LC (IPF-multiple LC group) and 131 with single LC (IPF-single LC group), and these were compared with 4,931 patients with LC from 1975 to 1977 in Japan (whole LC group). In the IPF-single and IPF-multiple LC groups, most tumors were observed in male patients (91% and 96%), smokers (94% and 100%), and in peripheral regions of the lung (91% and 98%). The incidence of occurrence in the lower lobes, where a fibrotic shadow was prominent, was significantly higher in the IPF-LC groups (58% and 67%) than for the whole LC group (37%). The distribution of histologic types in the IPF-single LC group was similar to that of the whole LC group. However, the incidence of small cell carcinoma was significantly higher in the IPF-multiple LC group (33%) than for the IPF-single LC (14%) and whole LC (12%) groups. These results indicate that the features characteristic to synchronous multiple LC in patients with IPF are as follows: (1) male patients; (2) smokers; (3) small cell carcinoma histologic type; (4) lower lobes; and (5) peripheral type, all of which show a high rate of occurrence.

Aged↗
PubMed1998

Morphometric analysis of insulin-like growth factor-I localization in lung tissues of patients with idiopathic pulmonary fibrosis.

Insulin-like growth factor-I (IGF-I) has been implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF) through its ability to stimulate fibroblast proliferation and collagen synthesis. However, although alveolar macrophages (AM) have been shown to express this growth factor, it is likely to also have other cellular sources. We sought to determine the distribution of cells expressing IGF-I in lung tissues obtained from 10 patients with IPF and 10 control subjects. We evaluated the levels of IGF-I and of a macrophage/monocyte-specific marker, CD68, by immunocytochemistry and quantified by morphometry. In control subjects, IGF-I was localized principally to AM. In contrast, in IPF patients IGF-I was localized to AM, interstitial macrophages, alveolar epithelial cells, and ciliated columnar epithelial cells. The normalized volume density (Vv) of IGF-I-positive (IGF-I+) interstitial macrophages (Vv of IGF-I+ interstitial macrophages/Vv of lung x 100) was increased in patients with IPF as compared with control subjects, and the ratio of Vv of IGF-I+ to CD68(+) interstitial macrophages correlated with: (1) the degree of clinical impairment in patients with IPF as measured by their clinical-radiologic-physiologic (CRP) score; and (2) the degree of collagen deposition in the interstitium. These findings support a role for interstitial macrophages as a source of IGF-I in IPF.

Adult↗
PubMed1993

Effect of dehydroproline as a collagen synthetic inhibitor in paraquat-induced pulmonary fibrosis in beagle dogs.

To investigate the effect of 3,4-DL-dehydroproline (DHP) on paraquat (PQ)-induced pulmonary fibrosis in beagle dogs, 25 beagles without distinction of sex were allocated at random into 2 groups; 17 dogs were PQ-dosed and 8 were saline-dosed. The 17 dogs were given 1 mg PQ/kg/d sc for 7-11 d; 14 of the 17 had decreased body weight on day 11 and were selected, 2 of which were determined at random and necropsied. The remaining 12 dogs were randomly divided into 2 groups of 6 dogs each (DHP-treated or saline-treated). They received either 25 mg DHP/kg/d sc or saline for 14 d and were sacrificed for measurement of lung lipid peroxide and lung hydroxyproline (Hyp) concentrations on day 25 or 180 after the PQ administrations started. Two of the 17 dogs (11.8%) given 1 mg PQ/kg/d sc died on days 9 and 11 of the administration. One dog (5.9%) had no signs of PQ toxicity including no decrease in body weight. The remaining 14 dogs (82.4%) had decreased body weight until day 11 of the PQ administrations. No significant differences in erythrocyte superoxide dismutase, blood catalase and serum-lipid peroxide occurred from the PQ dosing; however, lung lipid peroxide increased 4-fold. On day 180 the lung lipid peroxide of the PQ + saline treated group was still 4-times elevated, while the PQ + DHP treated group had about 1/2 that value. Lung Hyp concentrations in the PQ dosed dogs were significantly increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗