PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “PULMONARY FIBROSIS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 55 records · Page 3Linked to original sources
PubMed1993

Elevated levels of lung surfactant protein A in sera from patients with idiopathic pulmonary fibrosis and pulmonary alveolar proteinosis.

An enzyme-linked immunosorbent assay using monoclonal antibodies to human lung surfactant protein A (SP-A) was applied to sera from patients with lung diseases. We examined whether SP-A appears in the sera of patients with diseases that are known to cause alterations in surfactant composition in bronchoalveolar lavage fluids, and we characterized the SP-A that was found. The level of SP-A in sera from 57 healthy volunteers was 45 +/- 3 ng/ml (mean +/- SEM). The levels in patients with idiopathic pulmonary fibrosis (IPF) (205 +/- 23 ng/ml, n = 32) and pulmonary alveolar proteinosis (PAP) (285 +/- 23 ng/ml, n = 6) were significantly higher than those in healthy control subjects (p < 0.01), whereas those of sarcoidosis (n = 16), pneumonia (n = 14), and tuberculosis (n = 14) were 52 +/- 27 ng/ml, 65 +/- 11 ng/ml, and 49 +/- 23 ng/ml, respectively. Electrophoresis and immunoblotting analysis demonstrated that the fraction isolated from serum of a patient with PAP or IPF by anti-SP-A immunoaffinity column chromatography consisted chiefly of human IgG and IgM, and that it also contained SP-A. Furthermore, IgG was found in preparation of purified human SP-A. SP-A was demonstrated to bind to nonimmune IgG coated onto microtiter wells. Gel filtration analysis revealed that serum SP-A was eluted at fractions of larger molecular size than was the purified SP-A. These findings suggest that SP-A appears in the bloodstream as a complex with immunoglobulin in IPF and in PAP.

Aged↗
PubMed1997

Idiopathic pulmonary fibrosis vs. pulmonary involvement of collagen vascular disease: HRCT findings.

The purpose of this study is to assess the differences of high-resolution CT (HRCT) findings in patients with idiopathic pulmonary fibrosis (IPF) and pulmonary involvement of collagen vascular disease (CVD). We analyzed the HRCT findings of 33 patients with IPF and 23 patients with CVD in terms of predominant pattern, site of involvement, mediastinal lymph node enlargement, pleural change, and pulmonary volume loss. The predominant HRCT pattern was honeycombing for IPF (58%), and ground-glass opacity for CVD (57%). Predominantly subpleural involvement was seen in 90% of IPF and 83% of CVD patients. Mediastinal lymph node enlargement was seen in 61% of the patients with IPF and 13% with CVD (p = 0.0004). Pleural thickening was seen in 97% of the patients with IPF and 35% with CVD and the severity of pleural thickening is statistically significant (p = 0.00001). Pleural effusion was seen in 6% of the patients with IPF and 26% with CVD (p = 0.0351). The hilar height ratio was more than 1.5 in 52% of the patients with IPF and 30% with CVD (p = 0.2620). Although HRCT findings of IPF and pulmonary involvement of CVD are similar and overlap considerably, but patients with IPF showed a tendency to more progressed fibrosis than patients with CVD.

Adult↗
PubMed2003

Pulmonary alveolar proteinosis mimicking idiopathic pulmonary fibrosis.

Pulmonary alveolar proteinosis (PAP) is a rare disorder of unknown etiology. While most biopsies from patients with PAP disclose a prominent positive periodic acid-Schiff, diastase-resistant intra-alveolar exudate, and minimal interstitial thickening, interstitial fibrosis and honeycombing can be the predominant histologic findings. We recently identified two patients with PAP who initially were thought to have idiopathic pulmonary fibrosis, but whose biopsies ultimately disclosed PAP with considerable interstitial fibrosis. Because the clinical and radiographic presentation of PAP can overlap with other interstitial lung disorders, it should be considered in the differential diagnosis of unexplained idiopathic interstitial pneumonias in young patients.

Adult↗
PubMed1994

Elevated IL-8 and MCP-1 in the bronchoalveolar lavage fluid of patients with idiopathic pulmonary fibrosis and pulmonary sarcoidosis.

The potential for interleukin-8 (IL-8) and monocyte chemotactic protein-1 (MCP-1) to induce neutrophil and mononuclear phagocyte accumulation in the lungs of patients with pulmonary sarcoidosis and idiopathic pulmonary fibrosis (IPF) was investigated. Bronchoalveolar lavage (BAL) fluids from 12 patients with IPF and 15 with sarcoidosis were concentrated by reversed-phase chromatography, and their IL-8 and MCP-1 concentrations assessed by enzyme-linked immunosorbent assay (ELISA), chemotaxis, and enzyme-releasing assays with monocytes and neutrophils. ELISA revealed significantly elevated concentrations of MCP-1 (20.1 ng/mg albumin) in the BAL fluids of patients with pulmonary sarcoidosis and those with IPF (41.8 ng/mg) in comparison to 11 normal individuals (4.24 ng/mg) and 15 patients with chronic bronchitis (CB) (5.16 ng/mg). Similarly, the chemotactic activity for monocytes (MCP-1 equivalent) was strongly increased in patients with sarcoidosis (86.03 ng/mg) as well as in those with IPF (54.47 ng/mg). The chemoattractant activity of normal individuals and CB patients was 7- or 3-fold lower, respectively. Patients with IPF and sarcoidosis also had elevated IL-8 levels (15.5 and 26.0 ng/mg, respectively; normals: 2.14 ng/mg; and CB patients: 4.23 ng/mg) and greater neutrophil chemotaxis (60.25 and 49.68 ng/mg, respectively; normals: 0.35 ng/mg; and CB patients: 11.06 ng/mg). These data suggest that increased levels of both MCP-1 and IL-8 may be characteristic for sarcoidosis or IPF. It appears likely that both of these chemoattractants contribute to the influx of monocytes and neutrophils into the pulmonary alveolus and interstitium in these diseases.

Adult↗
PubMed2000

[Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to pulmonary fibrosis].

Pulmonary hypertension is a life-threatening complication of lung fibrosis. Vasodilator therapy is difficult owing to systemic side effects and pulmonary ventilation-perfusion mismatch. We compared the effects of intravenous prostacyclin and inhaled NO and aerosolized prostacyclin in randomized order and, in addition, tested for effects of oxygen and systemic calcium antagonists (CAAs) in eight patients with lung fibrosis and pulmonary hypertension. Aerosolized prostaglandin (PG)I2 caused preferential pulmonary vasodilatation with a decrease in mean pulmonary arterial pressure from 44.1 +/- 4.2 to 31.6 +/- 3.1 mmHg, and pulmonary vascular resistance (RL) from 810 +/- 226 to 386 +/- 69 dyn.s.cm-5 (p < 0.005, respectively). Systemic arterial pressure, arterial oxygen saturation, and pulmonary right-to-left-shunt flow, measured by multiple inert gas analysis, were not significantly changed. Inhaled NO similarly resulted in selective pulmonary vasodilatation, with RL decreasing from 726 +/- 217 to 458 +/- 81 dyn.s.cm-5. In contrast, both intravenous PGI2 and CAAs were not pulmonary selective, resulting in a significant drop in arterial pressure. In addition PGI2 infusion caused a marked increase in shunt flow. Long-term therapy with aerosolized iloprost (long-acting PGI2 analog) resulted in unequivocal clinical improvement from a state of immobilization and severe resting dyspnea in a patient with decompensated right heart failure. We concluded that, in pulmonary hypertension secondary to lung fibrosis, aerosolization of PGI2 or iloprost causes marked pulmonary vasodilatation with maintenance of gas exchange and systemic arterial pressure. Long-term therapy with inhaled iloprost may be life saving in decompensated right heart failure from pulmonary hypertension secondary to lung fibrosis.

Administration, Inhalation↗
PubMed1999

Enhanced insulin-like growth factor binding protein-related protein 2 (Connective tissue growth factor) expression in patients with idiopathic pulmonary fibrosis and pulmonary sarcoidosis.

Connective tissue growth factor is a recently described chemoattractant and fibroblast mitogen which, because of sequence homology and weak binding to insulin-like growth factor (IGF)-1, has been proposed as the eighth member of the IGF binding protein (IGFBP) superfamily, named IGFBP-related protein 2 (IGFBP-rP2). Previous studies have implicated IGFBP-rP2 in a number of heterogeneous fibrotic pathologies, including renal fibrosis, dermal scleroderma, and bleomycin-induced pulmonary fibrosis in mice. Because profibrogenic cytokines may be produced by inflammatory cells, we developed a multiplex competitive reverse transcription/polymerase chain reaction to quantify IGFBP-rP2 transcripts in bronchoalveolar lavage cells from healthy subjects and patients with idiopathic pulmonary fibrosis (IPF) and pulmonary sarcoidosis. IGFBP-rP2 messenger RNA expression was enhanced > 10-fold (P < 0.003) in patients with IPF; > 40-fold (P < 0.006) in stage I/II sarcoidosis patients, and > 90-fold (P < 0.005) in stage III/IV sarcoidosis patients by comparison with healthy nonsmoking control subjects. We suggest these increases are predominantly associated with lymphocyte- and neutrophil-driven IGFBP-rP2 production. These findings, together with previous reports implicating other IGFBPs in the pathogenesis of pulmonary fibrosis, suggest that the complex network of IGFBPs within the human lung is an important determinant of the outcome of the fibroproliferative response to injury.

Adult↗
PubMed2006

Caveolin-1: a critical regulator of lung fibrosis in idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a progressive chronic disorder characterized by activation of fibroblasts and overproduction of extracellular matrix (ECM). Caveolin-1 (cav-1), a principal component of caveolae, has been implicated in the regulation of numerous signaling pathways and biological processes. We observed marked reduction of cav-1 expression in lung tissues and in primary pulmonary fibroblasts from IPF patients compared with controls. We also demonstrated that cav-1 markedly ameliorated bleomycin (BLM)-induced pulmonary fibrosis, as indicated by histological analysis, hydroxyproline content, and immunoblot analysis. Additionally, transforming growth factor beta1 (TGF-beta1), the well-known profibrotic cytokine, decreased cav-1 expression in human pulmonary fibroblasts. cav-1 was able to suppress TGF-beta1-induced ECM production in cultured fibroblasts through the regulation of the c-Jun N-terminal kinase (JNK) pathway. Interestingly, highly activated JNK was detected in IPF- and BLM-instilled lung tissue samples, which was dramatically suppressed by ad-cav-1 infection. Moreover, JNK1-null fibroblasts showed reduced smad signaling cascades, mimicking the effects of cav-1. This study indicates a pivotal role for cav-1 in ECM regulation and suggests a novel therapeutic target for patients with pulmonary fibrosis.

Actins↗
PubMedDate not supplied

[Anatomo-clinical confrontation: idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis and pulmonary fibrosis associated with systemic diseases are the most frequent diffuse interstitial pulmonary diseases. They slowly but irrevocably progress towards terminal respiratory failure. They can also be complicated by severe acute respiratory failure and admission in the intensive care unit can be discussed. Despite invasive mechanical ventilation, anti-infectious and immunosuppressive treatments, the disease carries a high mortality rate. We report and discuss the case of a patient with idiopathic, pulmonary fibrosis (UIP) who underwent rapid clinical deterioration.

Humans↗
PubMed1998

The role of eosinophils in pulmonary fibrosis (Review).

Pulmonary fibrosis is commonly characterized by inflammation of the alveolar wall, leading to derangement of normal alveolar architecture, and interstitial as well as intra-alveolar fibrosis. The process involves cellular interactions via a complex cytokine network and heightened collagen gene expression with abnormal deposition in the lung. Recent studies have identified a myriad of cytokines with potential roles in pulmonary fibrosis. Based on in vivo antibody neutralization studies, important roles for tumor necrosis á (TNFá), macrophage inflammatory protein 1á (MIP-1á) and transforming growth factor (TGF), have been established. The recent demonstration that the eosinophil is a major source for several of these key pro-fibrogenic cytokines during the early stages of fibrosis, strongly suggest a role for the eosinophil in pulmonary fibrosis. In vitro, eosinophils can elaborate factors capable of stimulating fibroblast proliferation, and their presence in lungs undergoing many forms of pulmonary fibrosis has been well documented. Further support for a role for eosinophils in pulmonary fibrosis are suggested by clinical data showing a correlation between lung eosinophil count and a poor prognosis and decreased responsiveness to therapy. This review will focus on the recent findings, which suggest novel potential roles for the eosinophil in the pathogenesis of pulmonary fibrosis.

Animals↗