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PubMedDate not supplied

Longitudinal analysis of serum KL-6 levels in patients with systemic sclerosis: association with the activity of pulmonary fibrosis.

OBJECTIVE: To determine whether changes in serum KL-6 levels reflect the activity of pulmonary fibrosis (PF) in patients with systemic sclerosis (SSc). METHODS: KL-6 levels were determined by ELISA in 39 SSc patients. In a retrospective longitudinal study, 250 serum samples were analyzed during a follow-up period of 0.3-6.1 years. RESULTS: KL-6 levels at the first visit were higher in patients with SSc, especially with PF, compared with healthy controls. In the longitudinal study, KL-6 levels in 4 patients with anti-topo I Abs increased rapidly, parallel to the progression of PF. Four patients with inactive PF exhibited elevated, but stable levels of KL-6 during the follow-up. The 31 patients with almost normal KL-6 levels during the follow-up exhibited no deterioration or new onset of PF. CONCLUSION: Rapidly increased serum KL-6 levels during disease course were associated with new onset or deterioration of PF.

Adult↗
PubMed2003

Pulmonary embolism in idiopathic pulmonary fibrosis transplant recipients.

The objectives of the study were the assessment of the incidence of pulmonary embolism (PE) in lung transplant recipients. We performed a retrospective review of the medical records in a tertiary center lung transplant program. A total of 72 lung transplants were performed. There were seven symptomatic PE events diagnosed among six patients (group 1). All PE events were in the subgroup of patients with idiopathic pulmonary fibrosis (IPF) [6 of 23 patients (27%) vs 0 among all other patients (0%); p < 0.001]. All patients were out of the hospital, not receiving oxygen therapy, and were ambulatory at the time of the event. The median time to occurrence of the PE was 175 days posttransplant (range, 26 to 541 days). All patients who developed PEs were men. The group of IPF patients with no PEs was evenly split between genders (group 2; p < 0.009). PE patients required a longer posttransplant hospitalization (mean [+/- SD], 18.5 +/- 3.9 vs 13.5 +/- 4 days, respectively; p < 0.018). Aside from this, there was no apparent difference in patient functional status between the two groups. PEs appear to be relatively common in IPF lung transplant recipients. This should be considered in the differential diagnosis of any such patient who presents with dyspnea or hypoxia posttransplant. Patients do not appear to have been predisposed to their embolic events through lack of activity or prolonged hospital stays.

Female↗
PubMed1991

[Lung injury in idiopathic pulmonary fibrosis and measurement of immunoreactive neutrophil elastase and alpha 1-protease inhibitor in blood].

Idiopathic pulmonary fibrosis (IPF) is thought to develop through slowly progressing lung injury, in which fibrosis occurs as a result of abnormal repair processes. Lung injury in emphysema, in which the normal extracellular matrix is destroyed, is considered to occur mainly because of protease-antiprotease imbalance. In order to examine whether the pathogenesis of IPF involves the proteolytic mechanism of enzymes as in emphysema, concentrations of plasma neutrophil elastase and serum alpha 1-protease inhibitor were measured in patients with IPF, and compared with the levels in patients with emphysema and in normal individuals. In some patients with IPF, the blood concentration of neutrophil elastase was much higher than normal and the degree of imbalance between neutrophil elastase and alpha 1-protease inhibitor was significantly great than in patients with emphysema. In these patients, many years had passed since the onset of the disease, the number of leukocytes and neutrophils and the concentration of LDH in peripheral blood were significantly higher than normal, and the concentration of CEA-II was slightly increased. These data suggest that chronic, massive lung injury had occurred. The blood concentration of neutrophil elastase and alpha 1-protease inhibitor ratio may be useful in assessing the degree of lung injury.

Adult↗
PubMed2003

Laminin-5 gamma2 chain in cryptogenic organizing pneumonia and idiopathic pulmonary fibrosis.

Insufficient reepithelialization of injured alveolar walls may be important in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Laminin-5 is expressed in epithelial cells of healing wounds, promoting cell attachment and migration. In this study we have studied the extent of reepithelialization of newly formed intraluminal connective tissue, the immunohistochemical expression and ultrastructural localization of the laminin-5 gamma2 chain protein, and the synthesis of the laminin-5 gamma2 chain mRNA in regenerating epithelial cells in cryptogenic organizing pneumonia (COP) and IPF. The results show that the mean extent of reepithelialization of intraluminal connective tissue lesions was 76% (SD, +/- 27%) in COP, and 54% (SD, +/- 23%) in IPF (p < 0.025). The laminin-5 gamma2 chain was synthesized and widely expressed in regenerating epithelial cells in both diseases. Immunohistochemistry for surfactant-associated protein A suggests a pneumocyte origin for the regenerating epithelial cells in IPF. It is concluded that both in COP and IPF, regenerating epithelial cells are capable of synthesizing the laminin-5 gamma2 chain needed for adhesive connections to the underlying basement membrane. However, in IPF, the reepithelialization seems to be disturbed or delayed.

Basement Membrane↗
PubMed2003

The shuttle walk exercise test in idiopathic pulmonary fibrosis.

The shuttle walk test (SWT) is a validated, incremental walking test for chronic obstructive pulmonary disease, but not for idiopathic pulmonary fibrosis (IPF). The measurement of maximal oxygen consumption (VO2 max) is considered to be the gold standard measurement of functional capacity. This study examines the relationship between IPF patients' performance on the SWT and VO2 max. Twenty patients were recruited for the study, which consisted of two separate experiments. Firstly, the relationship between SWT performance on a conventional corridor SWT, with that on a programmable treadmill SWT designed to reproduce the corridor SWT was examined (n=10). In the second experiment, the relationship between performance on the treadmill equivalent SWT and VO2 max measurements was studied (n=10). There was a significant correlation between distance walked on the corridor SWT, and that walked on the treadmill equivalent SWT without VO2 max measurements (367 m vs. 410 m) (r=0.91, P=0.0003). There was a significant correlation between distance walked on the treadmill equivalent SWT (277 m), and the directly determined VO2 max (14.87 ml/kg/min) (r=0.74, P=0.01). During both experiments, a significant correlation was also observed between baseline PaO2 and SWT performance, and between DLCO and SWT performance. The shuttle walk test is a simple objective measure of functional capacity in IPF patients, which should facilitate the evaluation of new therapeutic compounds for IPF.

Exercise Test↗
PubMed2001

Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation.

Of patients awaiting lung transplantation, the death rates are highest in those with idiopathic pulmonary fibrosis (IPF), suggesting that many IPF patients are referred late for transplantation. Therefore this study was undertaken to evaluate baseline pulmonary function test (PFT) and high-resolution computed tomography (HRCT) fibrosis scores, and the relationship to survival in IPF patients younger than 65 yr of age. A total of 115 patients with usual interstitial pneumonia (UIP) were studied. At presentation to a tertiary referral center, PFT and HRCT data were collected and analyzed for prognostic significance: the primary outcome measure was patient death. Based on the length of the waiting list for transplantation, prediction of 2-yr survival was examined. DL(CO) percent predicted and HRCT-fibrosis score were found to be independent predictors of survival and in combination gave the best prognostic prediction. The optimal points on the receiver operating characteristic (ROC) curves for discriminating between survivors and nonsurvivors corresponded to 39% DL(CO) percent predicted, and to a HRCT-fibrosis score of 2.25. The combination of these parameters yielded an optimal point with a specificity and a sensitivity of 84% and 82%, respectively. A model based on a combination of DL(CO) percent predicted and HRCT-fibrosis score may optimize the timing of referral for transplantation.

Adolescent↗
PubMed1989

[Fibrosing interstitial pneumonia and idiopathic pulmonary fibrosis].

The most reasonable classification for diffuse interstitial pneumonia/fibrosis is described according to the clinical course, histopathological findings and responses to steroids. Idiopathic pulmonary fibrosis (IPF) represents the most prevalent, most chronic form of the disease with the worst prognosis. The histopathological findings of IPF are usual interstitial pneumonia (UIP) which microscopically is recognized as patchy lesions. In UIP diffuse alveolar inflammation does not change to diffuse alveolar fibrosis, rather the disease progresses via an increase in the number of localized fibrosis lesions. Steroids are ineffective in the treatment of IPF. Lung transplantation is the only effective treatment. The question of when such transplantation should be undertaken constitutes the subject of further study.

Fibrosis↗
PubMed1992

Expression of monocyte chemoattractant protein 1 mRNA in human idiopathic pulmonary fibrosis.

Macrophages are thought to play an important role in the pathologic changes associated with idiopathic pulmonary fibrosis (IPF). The mechanisms for increased monocyte/macrophage recruitment in IPF are unknown. Monocyte chemoattractant protein 1 (MCP-1) is the predominant monocyte chemoattractant secreted by a variety of different cell types in culture. We examined the expression of MCP-1 mRNA and its protein product in vivo in IPF and non-IPF lung specimens by in situ hybridization and immunocytochemistry. The cell types expressing MCP-1 in vivo were identified by immunostaining with specific antibodies. We demonstrated the expression of MCP-1 mRNA in pulmonary epithelial cells, in monocytes/macrophages, and in vascular endothelial and smooth muscle cells. Lung epithelial cells in patients with IPF strongly expressed MCP-1 mRNA and its protein product. In contrast, epithelial cells in non-IPF specimens did not express MCP-1 mRNA. Macrophages and vascular endothelial and smooth muscle cells were shown to express MCP-1 in both IPF and non-IPF lung specimens. These findings provide a basis for the understanding of the in vivo physiologic processes that mediate monocyte/macrophage recruitment and infiltration in the lung interstitium and the pathologic state contributing to an increased alveolar monocyte/macrophage population and inflammation in IPF.

Arteries↗
PubMed2006

Extravascular sources of lung angiotensin peptide synthesis in idiopathic pulmonary fibrosis.

Previous work from this laboratory demonstrated de novo synthesis of angiotensin (ANG) peptides by apoptotic pulmonary alveolar epithelial cells (AEC) and by lung myofibroblasts in vitro and in bleomycin-treated rats. To determine whether these same cell types also synthesize ANG peptides de novo within the fibrotic human lung in situ, we subjected paraffin sections of normal and fibrotic (idiopathic pulmonary fibrosis, IPF) human lung to immunohistochemistry (IHC) and in situ hybridization to detect ANG peptides and angiotensinogen (AGT) mRNA. These were analyzed both alone and in combination with cell-specific markers of AEC [monoclonal antibody (MAb) MNF-116] and myofibroblasts [alpha-smooth muscle actin (alpha-SMA) MAb] and an in situ DNA end labeling (ISEL) method to detect apoptosis. In normal human lung, IHC detected AGT protein in smooth muscle underlying normal bronchi and vessels, but not elsewhere. Real-time RT-PCR and Western blotting revealed that AGT mRNA and protein were 21-fold and 3.6-fold more abundant, respectively, in IPF lung biopsies relative to biopsies of normal human lung (both P < 0.05). In IPF lung, both AGT protein and mRNA were detected in AEC that double-labeled with MAb MNF-116 and with ISEL, suggesting AGT expression by apoptotic epithelia in situ. AGT protein and mRNA also colocalized to myofibroblast foci detected by alpha-SMA MAb, but AGT mRNA was not detected in smooth muscle. These data are consistent with earlier data from isolated human lung cells in vitro and bleomycin-induced rat lung fibrosis models, and they suggest that apoptotic AEC and myofibroblasts constitute key sources of locally derived ANG peptides in the IPF lung.

Angiotensinogen↗
PubMed1988

Idiopathic pulmonary fibrosis. Abnormalities in bronchoalveolar lavage fluid phospholipids.

Bronchoalveolar lavage has been used to sample cells and proteins in the distal lung. One of the major secretory products of the alveolar type II epithelial cells, pulmonary surfactant, can be recovered by lavage. Abnormalities in alveolar type II cells are found in biopsies of patients with idiopathic pulmonary fibrosis (IPF), and abnormalities of pulmonary surfactant phospholipids have been reported after diffuse lung injury in animals and in humans. Therefore, we questioned if abnormalities in lavage phospholipids might also occur in IPF, a chronic inflammatory disease of the alveolar epithelium and interstitium, and, if present, would these abnormalities reflect histopathologic changes or predict responsiveness to therapy. Fifteen untreated patients with IPF, diagnosed by open lung biopsy, were studied and were found to have less than half the amount of bronchoalveolar lavage phospholipid as that recovered from healthy volunteers (p less than 0.05). In addition, patients with IPF had a lower proportion of phosphatidylglycerol and a higher proportion of phosphatidylinositol in the recovered phospholipids than did healthy volunteers (p less than 0.05). The severity of these alterations in phospholipid composition correlated with more advanced fibrotic histopathologic changes. Patients with less depression of total phospholipids in lavage improved with corticosteroid therapy, whereas the patients with more severely decreased total phospholipid recovered in lavage did not.

Adult↗
PubMed2006

Dietary fat and meat intake and idiopathic pulmonary fibrosis: a case-control study in Japan.

SETTING: There is sparse epidemiologic information regarding the role of dietary factors in the development of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: To examine the relationship between specific types of fatty acids and selected foods high in fat and IPF in Japan. DESIGN: Included were 104 cases aged > or = 40 years who had been diagnosed in the last 2 years in accordance with the most recent criteria. Controls aged > or = 40 years consisted of 56 hospitalised patients diagnosed as having acute bacterial pneumonia and four out-patients with common cold. RESULTS: Intake of saturated fatty acids, mono-unsaturated fatty acids, n-6 polyunsaturated fatty acids and meat was independently associated with an increased risk of IPF. Specifically, the multivariate OR for comparison of the highest with the lowest quartile of intake of saturated fatty acids was 6.26 (95%CI 1.79-24.96, P for trend = 0.01) and for meat it was 7.19 (95%CI 2.15-27.07, P for trend = 0.02). Intake of cholesterol, n-3 polyunsaturated fatty acids, fish, eggs and dairy products was not related to the risk. CONCLUSION: These findings suggest that consumption of saturated fatty acids and meat may increase the risk of IPF.

Adult↗
PubMed2002

Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland.

BACKGROUND: The prevalence of sporadic and familial idiopathic pulmonary fibrosis (IPF) cases in Finland was evaluated according to the revised recommendations of the American Thoracic Society. METHODS: All Finnish pulmonary clinics (n=29) were included in the primary screening. Hospital data bases were used to identify patients with the diagnosis "alveolitis fibroticans idiopathica" (J84.1 in ICD-10 classification). The total number of patients with IPF was extrapolated based on the evaluation of random samples of case records in different centres. Families with more than one potentially affected member were identified from a questionnaire study and the diagnosis was verified from the medical records. RESULTS: Using this approach, the nationwide prevalence of IPF in Finland was estimated to be 16-18/100 000. In 90% of the patients lung involvement was assessed by high resolution computed tomographic (HRCT) scanning and in 31% a surgical biopsy specimen was available, further confirming the diagnosis. Seventeen multiplex families with 2-5 affected family members were identified, giving a prevalence of 5.9/million for familial IPF in Finland. Both multiplex and sporadic families were clustered in Eastern Finland. This clustering reflects the demographic history of Finland in the 16th century and suggests that multiplex families may share a common ancestor in the last 20-25 generations. CONCLUSION: The familial form explained 3.3-3.7% of all Finnish cases of IPF diagnosed according to the revised international guidelines. Geographical clustering of multiplex families suggests a recent founder effect in patients with familial IPF.

Aged↗
PubMed2005

Case-control study of medical history and idiopathic pulmonary fibrosis in Japan.

OBJECTIVES: A few epidemiological studies have indicated that a patient's past medical history may contribute to the risk of developing idiopathic pulmonary fibrosis (IPF). A relationship between a history of selected disorders and the risk of IPF was assessed in a multicentre hospital-based case-control study in Japan. METHODOLOGY: Included in the study were 104 patients of IPF, aged 40 years or over, who had been diagnosed within the previous 2 years, in accordance with the most recent criteria. Control subjects, aged 40 years or over, consisted of 56 hospitalized patients diagnosed as having acute bacterial pneumonia and four outpatients with the common cold. Adjustment was made for age, gender, region, pack-years of smoking, employment status, occupational exposure and BMI. RESULTS: Medical histories of hypertension, hyperlipidaemia, coronary heart disease, diabetes mellitus, hepatitis C virus infection, tuberculosis, asthma, atopic dermatitis and allergic rhinitis were not statistically significantly associated with the risk of IPF, although cases were more likely to have suffered from allergic rhinitis and less likely to have been asthmatics than control subjects. Having a child with a history of allergic rhinitis, but not of asthma or atopic dermatitis, was significantly related to an increased risk of IPF. CONCLUSIONS: These findings suggest that a genetic predisposition to allergic rhinitis may be associated with an increased risk of IPF.

Aged↗
PubMed2002

Interleukin-1 gene cluster polymorphisms in sarcoidosis and idiopathic pulmonary fibrosis.

Members of the interleukin-1 (IL-1) family are implicated in the pathogenesis of sarcoidosis and idiopathic pulmonary fibrosis (IPF). We have, therefore, performed a case-control study to investigate a plausible association between sarcoidosis and the polymorphisms in the IL-1alpha, IL-1beta, and IL-1 receptor antagonist (IL-1Ra) genes. Further, as a separate question, we explored whether the aforementioned genes of the IL-1 cluster are associated with IPF. Using PCR with sequence-specific primers, IL-1alpha -889, IL-1beta -511, IL-1beta +3953, and IL-1Ra intron 2 VNTR polymorphisms were determined in 348 white subjects of West Slavonic ancestry (95 patients with sarcoidosis, 54 patients with IPF, and 199 healthy control subjects). The IL-1alpha -889 1.1 genotype was significantly overrepresented in patients with sarcoidosis in comparison with control subjects (60.0 versus 44.2%, p = 0.012, p(corr) = 0.047). The distribution of IL-1beta -511, IL-1beta +3953, and IL-1Ra VNTR genotypes and alleles did not significantly differ between the cases and controls. No association between IPF and the investigated polymorphisms was found. Strong linkage disequilibrium between pairs of polymorphic loci was observed. Further population studies are warranted to confirm the observed association between sarcoidosis and the IL-1alpha polymorphism and also to explore mechanisms of IL-1alpha -889 participation in aberrant immune response in sarcoidosis.

Adolescent↗
PubMed2005

Role of the chemokine receptors CXCR3 and CCR4 in human pulmonary fibrosis.

RATIONALE: The chemokine receptors CXCR3 and CCR4 have recently been described as playing a pivotal role in the mouse model of bleomycin-induced fibrosis. OBJECTIVES: To evaluate the role of these receptors in human idiopathic pulmonary fibrosis (IPF). METHODS: We studied 57 patients: 18 with IPF, 17 with non-IPF (nIPF), 12 with sarcoidosis, and 10 healthy control subjects. MEASUREMENTS: We evaluated the expression of CXCR3 and CCR4 in blood and bronchoalveolar lavage (BAL) T lymphocytes by flow cytometry and the chemokine CXCL10, CXCL11 and CCL17 BAL concentration by singular immunoassay. MAIN RESULTS: Patients with IPF had a significantly lower CXCR3 and a higher CCR4 expression on BAL CD4 T cells compared with the other groups. Among patients with IPF, those treated with corticosteroids exhibited higher CXCR3 and lower CCR4 expression compared with untreated patients. CXCR3 expression correlated with BAL lymphocytes and CCR4 with BAL neutrophils and eosinophils. CXCL10 levels correlated with the expression of CXCR3 on BAL CD4 cells. CXCL11 was undetectable in almost all patients, whereas CCL17 was primarily detectable in patients with IPF. The percentage of BAL CCR4CD4 cells negatively correlated with DL(CO). The changes in the total lung capacity, VC, and of the alveolar-arterial PO2 gradient in patients with IPF and those with nIPF 6 to 12 mo after the first evaluation were associated with CD4CXCR3 percentage on BAL cells. CONCLUSIONS: We found an imbalance in CXCR3/CCR4 expression on BAL CD4 lymphocytes and reduced CXCL10 BAL levels in patients with IPF, suggesting a pivotal role of these molecules in IPF.

Adult↗
PubMed1987

Detection of antibodies in serum of patients with idiopathic pulmonary fibrosis against isolated rat alveolar type II cells.

The serum of 2 patients suffering from idiopathic pulmonary fibrosis (IPF) repeatedly demonstrated a positive antibody reaction against rat alveolar type II cells isolated by the use of a combination of elastase and trypsin for disaggregation and a Percoll density gradient for purification. Neither of the other 8 IPF patients examined nor any of the controls showed a positive reaction. Because the 2 patients with serum antibodies against alveolar type II cells did not show any antinuclear antibodies, it is concluded that the antibodies against the surface of rat alveolar type II cells found in the serum of IPF patients may be autoantibodies involved in the pathogenesis of IPF.

Animals↗