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PubMed1993

Pneumomediastinum in pulmonary fibrosis. Detection by computed tomography.

Pneumomediastinum was found in 5 of 34 patients (14.7 percent) with pulmonary fibrosis on computed tomographic (CT) scan of the chest. Small collection of air was seen in the retrosternal space in two patients, preaortic space in one patient, pretracheal space in two, subcarinal space in three, at the hilum in one, and perivertebral space in three patients. Concomitant pneumothorax was noted in two patients, both on the left side. Patients showed either honeycombing on CT (four patients) or presented with violent cough (four patients). Pneumomediastinum seen in these patients was thought to be the result of rupture of alveoli or honeycomb cysts due to raised intrapulmonary pressure on coughing, and subsequent tracking of air along the vascular sheath and accumulation in the mediastinum.

Adult↗

PubMed2001

Effect of prolonged low-dose methylprednisolone therapy in acute exacerbation of idiopathic pulmonary fibrosis.

We report on a 74-year-old man with an acute exacerbation of idiopathic pulmonary fibrosis (IPF) who was successfully treated with prolonged low-dose methylprednisolone, initiated at a loading dose of 2 mg/kg, followed by 2 mg/kg per day for 14 days. The dose was then tapered. The exacerbation observed on the chest radiograph and high-resolution computed tomography was found to have abated after the treatment. This successful case suggests the feasibility of this methylprednisolone treatment protocol for patients with IPF accompanied by accelerated deterioration. Furthermore, this case suggests possible similarities between acute exacerbation of IPF and late acute respiratory distress syndrome, as the same treatment protocol was previously proved to be beneficial for patients with late acute respiratory distress syndrome.

Acute Disease↗

PubMed1981

The development of bleomycin-induced pulmonary fibrosis in neutrophil-depleted and complement-depleted rats.

The development of bleomycin-induced pulmonary fibrosis was studied in rats depleted of neutrophils for 1 week and in rats depleted of complement for 1 week. Lung injury was assessed by histologic evaluation and by the biochemical measurement of total lung collagen. Qualitatively, histologic evidence of damage induced by bleomycin showed that damage progressed in a similar manner in neutropenic and complement-depleted animals when compared with normal animals. However, analysis of total lung collagen at 1 week after bleomycin administration revealed that collagen deposition was increased in neutropenic animals and decreased in animals depleted of complement when compared with untreated animals that received bleomycin. There were no significant differences in lung collagen levels at 1 month after bleomycin treatment in neutrophil-depleted or complement-depleted animals when compared with animals that received bleomycin alone.

Animals↗

PubMed1993

Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases.

We treated three patients with idiopathic pulmonary fibrosis who had an acute clinical exacerbation. We analyzed their clinical, radiographic, therapeutic, and pathologic findings. Their initial symptoms were influenza-like illness or cough with fever, and all had leukocytosis and elevation of C-reactive protein. Infectious events were ruled out by extensive bacteriologic and serologic examination. The patients' lung injury scores progressed rapidly to severe lung injury compatible with adult respiratory distress syndrome. Findings from bronchoalveolar lavage fluid showed marked neutrophilia and elevation of albumin concentrations. All patients showed various degrees of improvement following corticosteroid therapy. Histologic findings from open lung biopsy specimens showed both usual interstitial pneumonia (UIP) and organizing acute lung injury pattern. Whether these two forms of interstitial pneumonia (UIP and acute lung injury pattern) are variants of one disease or are unrelated and also the effectiveness of corticosteroid therapy on such conditions remain to be determined by further studies.

Acute Disease↗

PubMed2003

Clinicopathological and therapeutic patterns of idiopathic pulmonary fibrosis in Kuwait: a prospective study.

Though common, the exact incidence and clinical pattern of idiopathic pulmonary fibrosis (IPF) in Gulf countries are not known. The results of a four-year prospective study undertaken in two tertiary hospitals in Kuwait are presented. The mean age at diagnosis of the 52 patients studied was 55.40 +/- 11.87 years. Thirty-two (61.5%) patients were male and 22 (42.3%) were smokers. The mean duration of symptoms at diagnosis was 2.1 +/- 0.92 years. Digital clubbing was found in 34 (65%) patients. The mean FVC, TLC and TLCO were 57%, 64.4% and 55% of predicted normal, respectively. The FVC value showed a significant difference between smokers and non-smokers (p < 0.05). HRCT findings were abnormal in all patients. Typical histological and high-resolution computed tomography findings of usual interstitial pneumonia, desquamative interstitial pneumonia and non-specific interstitial pneumonia were observed. This study revealed that IPF is prevalent in Kuwait, with patterns showing some similarities to those established elsewhere. The response to treatment was not encouraging, especially in the usual interstitial pneumonia subtype.

Adult↗

PubMed2003

Acute exacerbation of idiopathic pulmonary fibrosis: report of a series.

This study describes five cases presenting an acute clinical course of pulmonary fibrosis, in the absence of specific precipitating factors. A retrospective chart review of five patients with histologically proved usual interstitial pneumonia was carried out in 2001-2002. Clinical data, bronchoalveolar lavage (BAL) findings, high resolution computed tomography and histological features were reported. On admission all cases presented hypoxemia and dyspnoea, while some showed an increase of carbohydrate antigen 19.9 or laboratory tests typical of infection, although appropriate cultures were all negative. Altogether, four subjects died and only one is on follow-up. A pattern of diffuse ground-glass or alveolar opacification superimposed on reticular and linear findings was evident on lung imaging in all cases. Marked neutrophilia, together with type II reactive cells hyperplasia, was detected on BAL. Histological findings, from open lung biopsy or autopsy, showed all the aspects of usual interstitial pneumonia with superimposed features of acute lung injury, such as diffuse alveolar damage, with or without hyaline membranes, type II reactive cells hyperplasia and numerous fibroblastic foci. This study also underlines the diagnostic value of bronchoalveolar lavage versus open lung biopsy.

Acute Disease↗

PubMed2005

In situ assessment of oxidant and nitrogenic stress in bleomycin pulmonary fibrosis.

Reactive oxygen species (ROS) and nitric oxide (NO) have a role in the development of pulmonary fibrosis after bleomycin administration. The ROS production induces an antioxidant response, involving superoxide dismutases (SODs), catalase, and glutathione peroxidases. We compared in situ oxidative burden and antioxidant enzyme activity in bleomycin-injured rat lungs and normal controls. ROS expression and catalase, glucose-6-phosphate-dehydrogenase (G6PHD), and NOS/NADPH-diaphorase activity were investigated by using histochemical reactions. Nitric oxide synthase (e-NOS and i-NOS) and SOD (MnSOD, Cu/ZnSOD, ECSOD) expression was investigated immunohistochemically. After treatment ROS production was enhanced in both phagocytes and in type II alveolar epithelial cells. Mn, Cu/Zn, and ECSOD were overexpressed in parenchymal cells, whereas interstitium expressed ECSOD. Catalase and G6PHD activity was moderately increased in parenchymal and inflammatory cells. NOS/NADPH-d activity and i-NOS expression increased in alveolar and bronchiolar epithelia and in inflammatory cells. It can be suggested that the concomitant activation of antioxidant enzymes is not adequate to scavenge the oxidant burden induced by bleomycin lung damage. Inflammatory cells and also epithelial cells are responsible of ROS and NO production. This oxidative and nitrosative stress may be a substantial trigger in TGF-beta1 overexpression by activated type II pneumocytes, leading to fibrotic lesions.

Animals↗

PubMed2004

Innate immunity dictates cytokine polarization relevant to the development of pulmonary fibrosis.

New data support the importance of the innate immune response in the resolution or progression of pulmonary fibrosis. The presence of CXC chemokine receptor 3-expressing cells, specifically pulmonary NK cells, is necessary to produce IFN-gamma. This is critical in the polarization of the immune response to injury toward a favorable Th1 response and resolution. In contrast, a Th2 response is associated with progressive fibrosis.

Animals↗

PubMed1994

Monocyte chemoattractant protein-1 in idiopathic pulmonary fibrosis and other interstitial lung diseases.

Macrophages play a crucial role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). To examine the mechanisms for increased monocyte/macrophage recruitment in IPF and nonIPF interstitial lung diseases (nonIPF) the localization of monocyte chemoattractant protein-1 (MCP-1) was investigated in 14 cases of IPF, seven cases of nonIPF, and seven normal control lungs (CTRL) by immunohistochemistry using a specific anti-MCP-1 monoclonal antibody, F9. By double immunohistochemical staining using F9 and one of the cell type specific antibodies significant differences in the staining pattern of MCP-1 were observed between IPF and nonIPF. In IPF MCP-1 was observed in cuboidal and flattened metaplastic epithelial cells, alveolar macrophages, and vascular endothelial cells. In contrast, no epithelial cells were stained for MCP-1 in nonIPF cases, although alveolar macrophages and vascular endothelial cells were labeled. Northern hybridization analysis of selected cases showed marked expression of MCP-1 messenger RNA (mRNA) in IPF and nonIPF compared with CTRL. These findings suggest that the MCP-1 production in IPF and nonIPF plays an important role in the recruitment of monocyte/macrophages. Monocyte chemoattractant protein-1 production by epithelial cells in IPF may be caused by the metaplastic nature of the epithelial cells and may be one of the key factors inducing the irreversible progression of IPF.

Adult↗

PubMed2001

Fibroblasts from idiopathic pulmonary fibrosis and normal lungs differ in growth rate, apoptosis, and tissue inhibitor of metalloproteinases expression.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disorder characterized by fibroblast proliferation and extracellular matrix accumulation. However, studies on fibroblast growth rate and collagen synthesis have given contradictory results. Here we analyzed fibroblast growth rate by a formazan-based chromogenic assay; fibroblast apoptosis by in situ end labeling (ISEL) and propidium iodide staining; percent of alpha-smooth muscle actin (alpha-SMA) positive cells by fluorescence-activated cell sorter; and alpha1-(I) collagen, transforming growth factor (TGF)-beta1, collagenase-1, gelatinases A and B, and tissue inhibitor of metalloproteinase (TIMP)-1, -2, -3, and -4 expression by reverse transcriptase/polymerase chain reaction in fibroblasts derived from IPF and control lungs. Growth rate was significantly lower in IPF fibroblasts compared with controls (13.3 +/- 38.5% versus 294.6 +/- 57%, P < 0.0001 at 13 d). Conversely, a significantly higher percentage of apoptotic cells was observed in IPF-derived fibroblasts (ISEL: 31.9 +/- 7.0% versus 15.5 +/- 7.6% from controls; P < 0.008). alpha-SMA analysis revealed a significantly higher percentage of myofibroblasts in IPF samples (62.8 +/- 25.2% versus 14.8 +/- 11.7% from controls; P < 0.01). IPF fibroblasts were characterized by an increase in pro-alpha1-(I) collagen, TGF-beta1, gelatinase B, and all TIMPs' gene expression, whereas collagenase-1 and gelatinase A expression showed no differences. These results suggest that fibroblasts from IPF exhibit a profibrotic secretory phenotype, with lower growth rate and increased spontaneous apoptosis.

Apoptosis↗

PubMed2026

Idiopathic pulmonary fibrosis risk loci in East Asian populations mirror those of European populations.

RATIONALE: Common and rare variants that are associated with the risk of developing idiopathic pulmonary fibrosis (IPF) have been identified predominantly in European ancestry populations. OBJECTIVES: To better understand the genetic variants that contribute to IPF in individuals with Asian ancestry, we conducted a genome-wide association study of IPF in East Asian populations. METHODS: We included 1026 patients with IPF and compared them to 1723 unaffected controls of Japanese and Korean ancestry. Genome-wide association analysis was conducted in the Japanese and Korean ancestry cohorts separately and combined using meta-analysis. Restricted maximum likelihood was used to estimate the SNP-based heritability and local ancestry of chromosome 11 was inferred for each subject. MEASUREMENTS AND MAIN RESULTS: We identified loci on chromosomes 4 (FAM13A; rs7690839), 5 (TERT; rs7734992), 6 (DSP; rs2076295), and 11 (MUC5B; rs35705950) that were significantly associated with risk of IPF. Importantly, the sentinel variants in each of these loci are the same as, or in strong linkage disequilibrium with, the risk variants that have been observed in studies of European ancestry populations. In aggregate, common variants (not including the MUC5B promoter variant) account for approximately 25% of the risk of developing IPF in these East Asian ancestry cohorts. Moreover, local ancestry analysis indicates that the presence of MUC5B promoter variant in the East Asian population is not a result of admixture with European ancestry populations. CONCLUSIONS: We conclude that the IPF risk loci in East Asian populations are shared with those of European ancestry populations, although their risk allele frequencies and effect sizes differ. These findings indicate shared genetic risk factors of IPF across ancestries.

Aged↗

PubMed1979

[Long-term follow-up of lung function parameters in patients with idiopathic pulmonary fibrosis treated with prednisone and azathioprin or d-penicillamine (author's transl)].

The prognosis of idiopathic pulmonary fibrosis is poor, it amounts to 4-6 years (after beginning of the symptoms). Spontaneous remissions are very rare, the corticosteroid monotherapy shows a certain success in only 11-16-20% of the cases. Therefore, since 1970 we carry out a combined therapy (see above); the results, confirmed by pulmonary function tets (vital capacity, static compliance, pO2 at rest and after exercise) will be presented. In summary: (1) Azathioprin and Prednisone: After a treatment of 2 years: 6 patients improved, 2 deteriorated, 3 died; the corresponding figures after 4 years were 6/1/4, after 6 years 3/-/5, after 8 years 2/-/3; (2) D-Penicillamine and Prednisone: After 2 years 3/2/4, 1 unchanged; after 4 years 1/-/4, 1 unchanged. The results of the therapy are discussed especially regarding prognosis of the disease and used drugs.

Azathioprine↗

PubMedDate not supplied

Chronic idiopathic pulmonary fibrosis in five dogs.

Five dogs presented with chronic and progressive pulmonary illness characterized by progressive dyspnea, exercise intolerance, and significant inspiratory crackles on auscultation. Radiographically, there was a widespread and diffuse interstitial lung pattern with varying degrees of bronchial involvement. Histopathological changes included thickened alveolar septa, interstitial fibrosis, and pneumocyte hyperplasia. Based on the clinical, radiographic, and histopathological changes, a diagnosis of idiopathic pulmonary fibrosis was made. Idiopathic pulmonary fibrosis is a chronic disease characterized by inflammation and fibrosis of the pulmonary interstitium and peripheral airspaces, which has been poorly characterized in the dog.

Animals↗

PubMed1983

Retention of inhaled particles in hamsters with pulmonary fibrosis.

Aerosol retention was studied in hamsters 30, 60, and 90 days after the initiation of interstitial pulmonary fibrosis by a combination of bleomycin (bleo), 0.16 U/100 g body weight given intratracheally, and O2, for 72 h. Groups of bleo-O2-treated and control animals were exposed (awake) for 25 min to a 99mTc-labeled insoluble aerosol (activity median aerodynamic diameter, 0.45 micron; geometric standard deviation, 1.3). Within 5 min after exposure, the hamsters were killed and their lungs were excised and dried at total lung capacity, and sliced into 1-mm sections. Slices were dissected into pieces, and an evenness index (EI) was calculated for each piece (formula: see text). With uniformity of retention, all Els would be 1. The distribution of Els in control animals had a mean of 1.0 and a SD of 0.27; 0% of the Els were less than or equal to 0.20. Total retention diminished and was less uniform in bleo-O2-treated animals. At 30 days, the SD increased to 0.62, and 6% of the Els were less than or equal to 0.20. At 60 and 90 days, nonuniformity decreased but was still greater than that in the control animals (SD60 = 0.42, SD90 = 0.36). When examined histologically, individual pieces with low Els had more disease than those with high Els. Local decreases in compliance caused by fibrosis may have altered regional ventilation and retention. Our data also correlate with the progression of fibrosis from a focal lesion at 30 days to a more diffuse lesion at 90 days.

Aerosols↗

PubMed2006

[Effects of gene therapy with replication-defective adenovirus ericlosing Egr-1 promoter and Smad7 cDNA on irradiation-induced pulmonary fibrosis: experiment with mice].

OBJECTIVE: To investigate the effects of gene therapy with replication-defective adenovirus enclosing Egr-1 promoter and Smad7 cDNA on irradiation-induced pulmonary fibrosis. METHODS: The recombinant replication-defective adenovirus AD. Egr-Smad7 enclosing Egr-1 promoter and Smad7 cDNA was constructed. 288 C57BL mice were randomly divided into 6 groups: AD. Egr-Smad7 group (Group RA, receiving intratracheal instillation of AD. Egr-Smad7 of the dose of 10(9) pfu/0.1 ml), AD. Egr-Smad7 + radiation group (Group RAR, receiving intratracheal instillation of AD. Egr-Smad7 of the dose of 10(9) pfu/0.1 ml and then radiation to the chest 14 h later), replication-defective adenovirus group (Group AV, receiving intratracheal instillation of replication-defective adenovirus of the dose of 10(9) pfu/0.1 ml), replication-defective adenovirus + irradiation group (Group AVR, receiving intratracheal instillation of replication-defective adenovirus of the dose of 10(9) pfu/0.1 ml and then radiation to the chest 14 h later), blank control group (Group C), and pure irradiation group (Group R), each group was re-divided into 6 subgroups of 8 mice to be observed 0, 1, 2, 4, 8, and 12 weeks after the treatment. The mice were killed at different time points and their lungs were taken out. The levels of type I collagen, type III collagen, connective tissue growth factor (CTGF), and transforming growth factor-beta1 (TGF-beta1) were detected by ELISA. The level of hydroxyproline was examined by alkaline hydrolysis method. The lung tissues were stained with HE to undergo pathological examination. RESULTS: The TGF-beta1 levels of the irradiation groups all increased, peaking in the second week (all P < 0.05), all significantly higher than those of Group C. However, the TGF-beta1 levels at different time points of Group RAR were all significantly lower then those of the other irradiation groups. The CTGF levels of different groups at different time points were all significantly higher than those of Group C (P < 0.05 or P < 0.01), and the CTGF levels of Groups RA and AV were decreased to almost normal 12 weeks after the irradiation. The levels of type I collagen and type III collagen of the 1 and 2-week subgroups of Group RAR were significantly lower than those of Group C (all P < 0.01), then gradually increased, and were slightly higher than those Group C 12 weeks later. The levels of type I collagen and type III collagen at different time points of the other groups were all significantly higher than those of Group C (P < 0.05 or P < 0.01). However, The levels of type I collagen at different time points of Group RAR were all lower than those of the other groups except Group C, and the levels of type III collagen in the first to eighth weeks after irradiation of Group RAR were all lower than those of the other groups except Group C. The hydroxyproline level of the 1 and 2 week subgroups of Group RAR were significantly lower than those of Group C (all P < 0.01), and then gradually increased. The hydroxyproline levels of the other irradiation groups all gradually increased significantly, peaking at the 12 th week (all P < 0.01). 1 approximately 2 weeks after irradiation Groups RAR, RA, and AV showed remarkable pulmonary congestion changes, even more remarkable then those in Group R, 8 approximately 12 week later, fibrosis changes were found in Group R and AVR, and 12 weeks later the histological structure of lung of Group AV, RAR, and RA returned almost normal. CONCLUSION: Radioactive rays induce Egr-1 promoter to regulated the expression of exogenous Smad7 gene that blocks the signal transduction of TGF-beta. Thus use of AD. Egr-Smad7 may become a novel strategy of gene therapy in prevention and treatment of pulmonary fibrosis.

Adenoviridae↗

PubMed1994

Bronchoalveolar lavage as a possible cause of acute exacerbation in idiopathic pulmonary fibrosis patients.

In the past 13 years (1980 to 1992), bronchoalveolar lavage (BAL) was performed on 124 idiopathic pulmonary fibrosis (IPF) patients (29 F and 95 M, 60 +/- 1 years, mean +/- S.E.) at Tohoku University Clinic and Hospital. Among them, three patients showed acute exacerbation immediately after BAL and died of progressive respiratory failure after 2.5 weeks, 2 months and 3.5 months, respectively, despite intensive care. They were all older patients (79, 74 and 66 years old) and we failed to find any evidence of viral, bacterial or fungus infections either before or after BAL in blood, sputum or BAL fluid samples. Further, all autopsied lungs showed interstitial pneumonia and fibrosis and no evidence of infectious diseases. In contrast, no progressive deteriorations after BAL were observed in 282 patients (122 F and 160 M, 48 +/- 1 years old) with other pulmonary diseases (sarcoidosis, bronchial asthma, collagen diseases and other interstitial lung diseases), who received BAL during the same period. This suggests that BAL itself sometimes induces a progressive degeneration in IPF patients, especially in older patients.

Aged↗

PubMed2005

Anticoagulant therapy for idiopathic pulmonary fibrosis.

STUDY OBJECTIVE: To evaluate the effect of anticoagulant therapy on the survival of patients with idiopathic pulmonary fibrosis (IPF). DESIGN: Prospective study. SETTING: Five hospitals located in the Miyagi prefecture in Japan, including a university hospital, a Red Cross hospital, two public general hospitals, and a municipal hospital. PATIENTS: Fifty-six patients with IPF (mean age, 69.4 years; range, 47 to 89) admitted to the hospitals from April 2001 to April 2004. INTERVENTIONS: Patients were assigned to receive prednisolone alone or prednisolone plus anticoagulant therapy. The anticoagulants included oral warfarin in an outpatient setting and low-molecular-weight heparin for rehospitalized patients with severely progressive respiratory failure. MEASUREMENTS AND RESULTS: There was no difference in baseline characteristics, including age, gender, clinical condition, pulmonary function, and plasma d-dimer level between the non-anticoagulant group and the anticoagulant group. The overall survival and hospitalization-free periods were assessed. There was a significant difference between survival curves of the non-anticoagulant group and the anticoagulant group, with a 2.9 hazard ratio (p = 0.04, Cox regression model). There was no significant difference in the probability of a hospitalization-free period between groups. The major cause of clinical deterioration was acute exacerbation during follow-up in the present study. Therefore, the mortality and plasma d-dimer levels in patients with an acute exacerbation were also assessed. The mortality associated with acute exacerbations of IPF in the anticoagulant group was significantly reduced compared to that in the non-anticoagulant group (18% vs 71%, respectively; p = 0.008, Fisher Exact Test). Furthermore, the plasma d-dimer levels in patients who died were significantly higher than those in survivors during acute exacerbation of IPF (3.3 +/- 2.3 microg/mL vs 0.9 +/- 0.7 microg/mL, p < 0.0001). Histologic analysis performed in three patients who died due to an exacerbation of IPF in the non-anticoagulant group demonstrated the features of usual interstitial pneumonia and acute lung injury. CONCLUSIONS: Our data suggested that plasma d-dimer levels are associated with mortality in patients with an acute exacerbation of IPF, and that anticoagulant therapy has a beneficial effect on survival in patients with IPF.

Aged↗

PubMed1998

Idiopathic pulmonary fibrosis associated with rectal carcinoma--a paraneoplastic syndrome or coincidence?

The association between lung cancer and idiopathic pulmonary fibrosis (IPF) is well documented, complicating > or = 10% of cases involving IPF. This association is considered to be a result of neoplastic degeneration, rather than a paraneoplastic phenomenon. However, rectal carcinoma has only rarely been linked to paraneoplastic manifestations. We describe a young patient with IPF that preceded the appearance of rectal carcinoma by 6 mo. The possible association between the two disorders is discussed.

Carcinoma↗