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PubMed1976

Cyclophosphamide therapy and interstitial pulmonary fibrosis.

Intersitial pneumonia and pulmonary fibrosis developed in a 72-year-old man during therapy with cyclophosphamide, vincristine, and prednisone. After extensive investigations, including an open lung biopsy, cyclophosphamide appeared to be the cause of the pulmonary disease. Complete disappearance of tachypnea and the pulmonary infiltrates occurred after the discontinuation of cyclophosphamide and the institution of prednisone therapy. We concluded that the diffuse pulmonary disease in this patient was a result of cyclophosphamide therapy. The clinical and pathologic findings in this case and a review of the literature of cyclophosphamide pulmonary toxicity are reported.

Adult↗

PubMed1992

Instillation of chemotactic factor to silica-injected lungs lowers interstitial particle content and reduces pulmonary fibrosis.

Silica-induced pulmonary fibrosis usually follows exposure to increased levels of this particulate and its retention in interstitial macrophages of the lung. It is suggested that accelerated clearance of particles from the pulmonary interstitium may ameliorate subsequent fibrosis. To test this hypothesis, one group of mice received 2-mg intratracheal (IT) silica; some particles were phagocytized and cleared during the subsequent inflammatory response, other particles were translocated across the epithelium to reach interstitial macrophages by 2 weeks. These mice later showed increased fibroblast growth, a doubling of lung collagen levels and large silicotic nodules by 16 weeks when much of the silica was still present in the lung. A second group of mice received IT silica, then 2 and 3 weeks later received IT injections of N-formyl-L-methionyl-leucyl-phenylalanine (FMLP), a leukocyte chemoattractant. Subsequently, a significant increase in inflammatory cells was seen and silica was observed mostly in phagocytes within the alveolar spaces. Few interstitial particles were found at 4 weeks, and extensive fibrosis did not develop by 16 weeks; only a few small nodules were seen and little silica was present in the lung. The results indicate that clearance of interstitial particles by a controlled inflammatory response is possible, and that removal of silica from the interstitium decreases the fibrotic response.

Animals↗

PubMed2006

Pulmonary vascular lesions in end-stage idiopathic pulmonary fibrosis: Histopathologic study on lung explant specimens and correlations with pulmonary hemodynamics.

In patients presenting with idiopathic pulmonary fibrosis (IPF), modifications of pulmonary vessels are well defined in fibrotic areas but have not been accurately assessed in the intervening patches of preserved lung. Moreover, the relation between pulmonary vessel lesions and pulmonary hemodynamics is not well known. We therefore designed a retrospective study on lung explant specimens from 26 patients with a firm diagnosis of IPF who had undergone lung transplantation. Our aim was to (1) describe the vascular lesions, especially in preserved lung areas, and (2) correlate them with pulmonary hemodynamics. In dense fibrotic zones, thickening of the arterial and venous wall with severe luminal narrowing was present in each patient. In architecturally preserved lung zones, occlusion of venules and small pulmonary veins was observed in 65% of the patients, although there were only mild changes of muscular pulmonary arteries. We found a significant positive correlation between the macroscopic extent of lung fibrosis and mean pulmonary artery pressure, but we failed to find a relation between mean pulmonary artery pressure and venous/venular lesions in nonfibrotic areas. Our study points out that in many patients with IPF, nonfibrotic lung areas demonstrate an occlusive venopathy, the signification of which remains undetermined.

Adult↗

PubMed2002

Mechanisms of colchicine effect in the treatment of asbestosis and idiopathic pulmonary fibrosis.

The objective of this study was to evaluate the mechanisms of colchicine action in pulmonary fibrosis. The study included 10 patients with pulmonary fibrosis (idiopathic pulmonary fibrosis 5, asbestosis 4, and scleroderma 1) who had been admitted to Bellevue Hospital Center, a tertiary care public hospital in New York City. We administered colchicine 0.6 mg orally for 12 weeks to patients with pulmonary fibrosis. Symptoms, high resolution CT scans, pulmonary function tests, and bronchoalveolar lavage parameters were compared prior to and after treatment. Results showed declines in dyspnea index, selective improvement in several CT scans, but no statistically significant change in BAL cells, cytokines, fibronectin, or hydroxyproline. However, there was a decline in hydroxyproline in the BAL fluid in 8/10 patients. We concluded that colchicine has a mild antifibrotic effect which may be in inhibiting collagen formation since there was no effect on the inflammation that accompanies fibrosis.

Aged↗

PubMed1998

A case of syphilitic interstitial pulmonary fibrosis.

Diagnosis of late pulmonary syphilis was made in a 72-year old woman on the basis of her medical history of untreated genital syphilis, serological and radiological findings, and the response to therapy. The diagnosis was confirmed two years later by autopsy findings. There was a good correlation between chest x-ray radiography, computed tomography scans, and autopsy findings. In the diagnosis of sarcoidosis and other diseases with similar radiological interstitial lung pattern, syphilitic interstitial pulmonary fibrosis should be carefully excluded.

Aged↗

PubMed1990

Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment.

Patients with idiopathic pulmonary fibrosis (IPF) inevitably experience declines in functional status that are most frequently due to progressive pulmonary fibrosis. However, the cause of the clinical deterioration is often uncertain, and disease progression is difficult to distinguish from disease-associated complications or adverse effects of therapy. In studies of the clinical course of IPF, mortality is most frequently due to respiratory failure (38.7%); other causes of death include heart failure (14.4%), bronchogenic carcinoma (10.4%), ischemic heart disease (9.5%), infection (6.5%), and pulmonary embolism (3.4%). Other, usually nonfatal, disease-associated complications include pneumothorax, corticosteroid-induced metabolic side effects and myopathy, and therapy-related immunosuppression. In evaluating clinical deterioration in patients with IPF, disease-associated complications and adverse effects of therapy should be distinguished from progressive pulmonary fibrosis. The cause of clinical deterioration will alter the therapeutic intervention required and will influence patient prognosis and duration of survival. This article examines the causes of clinical deterioration in patients with IPF and the diagnostic procedures for assessing disease-associated complications and staging IPF progression.

Adrenal Cortex Hormones↗

PubMed2003

High-resolution CT of asbestosis and idiopathic pulmonary fibrosis.

OBJECTIVE: We studied high-resolution CT of asbestosis and idiopathic pulmonary fibrosis to determine whether differences-other than the frequency of associated pleural changes-could be discerned between the two diseases. MATERIALS AND METHODS: High-resolution CT scans of 80 patients with asbestosis and 80 patients with idiopathic pulmonary fibrosis were retrospectively reviewed. Two chest radiologists assessed the type and distribution of parenchymal and pleural abnormalities on high-resolution CT. RESULTS: Subpleural dotlike or branching opacities (65/80), subpleural curvilinear lines (55/80), mosaic perfusion (39/80), and parenchymal bands (38/80) were more common in patients with asbestosis (p < 0.0001). Visible intralobular bronchioles (62/80), bronchiolectasis within fibrotic consolidations (47/80), and honeycombing (61/80) were more common in patients with idiopathic pulmonary fibrosis (p < 0.0001). The frequencies of interlobular septal thickening, ground-glass opacities, fibrotic consolidation, and emphysema were similar in both groups. Parenchymal bands and fibrotic consolidation were more commonly seen (p < 0. 05) in patients with asbestosis associated with pleural disease (n = 66) than in patients with asbestosis without pleural disease (n = 14). Also, statistically significant differences were noted between high-resolution CT findings of patients with asbestosis without pleural disease and those of patients with idiopathic pulmonary fibrosis, except for parenchymal bands. CONCLUSION: Specific combinations of high-resolution CT findings strongly suggest either asbestosis or idiopathic pulmonary fibrosis. We found that CT findings that might have represented bronchiolar obstruction in the subpleural region were more prominent in patients with asbestosis than in those with idiopathic pulmonary fibrosis, whereas bronchiolar dilatation was more prominent in patients with idiopathic pulmonary fibrosis than in those with asbestosis.

Asbestosis↗

PubMed1988

Different patterns of gas exchange response to exercise in asbestosis and idiopathic pulmonary fibrosis.

To analyse the pattern of pulmonary gas exchange during maximal exercise (Emax) in asbestosis, we compared nine subjects with this disease (1 female/8 male), aged 54 +/- 11 yrs (mean +/- SD), to nine patients (1 female/8 male) with idiopathic pulmonary fibrosis (IPF) of a similar age, height, weight and smoking history, both at rest and during Emax. No differences were observed in dynamic and static lung volumes between the groups. However, patients with IPF had a lower DLCOsb and KCO (p less than 0.005 and 0.05, respectively). At rest, both groups showed mild arterial hypoxaemia (76 +/- 11, asbestosis, vs 77 +/- 11 mmHg, IPF), widened AaPO2 (32 +/- 14 vs 31 +/- 13 mmHg) and slight increases in VD/VT (47 +/- 12 vs 46 +/- 11%), respectively. During Emax, PaO2 fell to 51 +/- 7 mmHg in patients with IPF whereas those with asbestosis had PaO2 of 73 +/- 21 mmHg (p less than 0.05). Conversely, those with asbestosis were able to reduce VD/VT (from 47 +/- 12 to 39 +/- 10%, p = 0.01) as opposed to those with IPF (from 46 +/- 11 to 47 +/- 13%). Furthermore, DLCOsb and AaPO2 during Emax were highly correlated only in IPF (r: -0.84, p less than 0.01). Despite the finding that both diseases represent a diffuse pulmonary fibrosis with a similar degree of resting ventilatory impairment, the pattern of gas exchange during exercise is different in each. These differences may be related to the underlying morphology of each process, which probably includes more airway disease and less pulmonary vascular involvement and/or a different degree of interstitial fibrotic change in asbestosis.

Asbestosis↗

PubMed2007

Safety and tolerability of bosentan in idiopathic pulmonary fibrosis: an open label study.

Idiopathic pulmonary fibrosis (IPF) is a fatal disease for which no effective treatment exists. In the present study, 12 IPF patients underwent analysis of gas exchange properties using the multiple inert gas elimination technique on day 1 before and after the administration of 125 mg bosentan, a dual endothelin antagonist. Following this, patients received chronic administration for 12 weeks (62.5 mg b.i.d. in week 1, 125 mg b.i.d. thereafter). The primary objective was to determine the effect of bosentan on gas exchange (day 1) and on oxygen saturation and minute ventilation (week 2). With one exception, where redistribution of total pulmonary blood flow from normal ventilation/perfusion (V'/Q') areas (93% before, 72% after bosentan) to low V'/Q' areas (0% before, 22.2% after) was encountered, no patient showed any change in gas exchange (mean+/-SD shunt flow (% of cardiac output) 8.5+/-3.4% before, 6.1+/-2.3% after bosentan; day 1) or oxygen saturation and minute ventilation (week 2). Similarly, none of the secondary parameters was significantly changed either at week 2 or at the end of the study period (week 12). Five patients developed respiratory infections and two died because of pneumonia; this was judged as being unrelated to bosentan intake. In conclusion, bosentan administration does not seem to induce clinically relevant gas exchange abnormalities in idiopathic pulmonary fibrosis patients.

Administration, Oral↗

PubMed2005

Mechanisms and mediators of pulmonary fibrosis.

The mechanisms of pulmonary fibrosis are complex, and several hypotheses have been put forward to explain how fibrosis develops. It was long thought that inflammation was a key event in the process; however, this has recently been challenged. The inflammation hypothesis has led to the description of numerous inflammatory and profibrotic mediators in the pathogenesis of fibrosis. Inhibition of inflammation can attenuate fibrosis in animal models but is less successful in humans. More recently, an important role for epithelial injury, circulating mesenchymal precursor cells, epithelial-to-mesenchymal transition, and vascular remodeling have been described in various models of fibrosis. Many of the classic inflammatory and profibrotic mediators are important in these processes, as well. The development of effective therapies will require the understanding of the complex interplay of the various mediators and the mechanisms of remodeling.

Animals↗

PubMed1995

Significance of early intra-alveolar fibrotic lesions and integrin expression in lung biopsy specimens from patients with idiopathic pulmonary fibrosis.

To study the pulmonary structural remodeling in idiopathic pulmonary fibrosis (IPF), ultrastructural, immunohistochemical, and light microscopic morphometric observations were made on 11 pulmonary biopsy specimens from patients with IPF. The morphometric study was done using sequentially cut tissue sections stained for keratin-alcian blue periodic acid-Schiff (PAS), fibronectin, and type IV collagen-alcian blue PAS. Most of the early fibrotic lesions, which were alcian blue- and fibronectin-positive, were intra-alveolar in location. Intra-alveolar fibrosis is considered to be essential for the fusion of alveolar walls in IPF. A strong reaction for integrin alpha 5 beta 1 and vinculin was found in epithelial cells and mesenchymal cells in areas of intra-alveolar fibrosis. These findings show that these cells are active in adhesion to fibronectin in areas of early intra-alveolar fibrosis. Some of the epithelial cells, including cytoplasmic hyaline-laden cells, showed evidence of inadequate adhesion to the extracellular matrix, and this may constitute one of the mechanisms of progression of fibrosis in IPF.

Adult↗

PubMed1989

Cyclophosphamide treatment of interstitial pulmonary fibrosis in polymyositis/dermatomyositis.

Interstitial pulmonary fibrosis is a serious complication of inflammatory muscle disease associated with significant morbidity and mortality. We describe 3 consecutive cases complicating dermatomyositis (1 case, anti-Jo-1 antibody negative) and polymyositis (2 cases, anti-Jo-1 antibody positive) in which cyclophosphamide was added to prednisone therapy with favorable outcomes.

Adult↗

PubMed2004

Diagnostic clues in the idiopathic pulmonary fibrosis of children.

Idiopathic pulmonary fibrosis (IPF) in children is a heterogeneous group of progressive disorders characterized by variable extents of inflammation and interstitial deposition of collagen fibers with numerous putative triggers. Children affected with this condition show breathlessness, non-productive cough and bibasilar/inspiratory dry rales. Diagnosis can be obtained by the exclusion of all known causes of fibrosing alveolitis such as neoplasms, toxic treatments, collagen vascular diseases, occupational exposure or granulomatous diseases.

Child↗

PubMed2004

Pharmacological strategies in the idiopatic pulmonary fibrosis of children.

Idiopathic pulmonary fibrosis is characterized by radiographically evident interstitial infiltrates and progressive dyspnea with rapid decline of overall lung functions. The clinical course of this disease is highly variable; however, its prognosis remains poor without adequate and prompt therapeutical support. Due to the fact that barely a quarter of patients responds to steroids alone, cytostatics are often prescribed simultaneously with low dose corticosteroids in the pediatric age too. Cytokine or anti-cytokine therapy and the role of new antifibrotic drugs are still at the experimental stage of definition and will be discussed in the following review.

Adrenal Cortex Hormones↗

PubMed2000

Circulating cytokeratin 8:anti-cytokeratin 8 antibody immune complexes in sera of patients with pulmonary fibrosis.

BACKGROUND: It has been suggested that the humoral immune system plays a role in the pathogenesis of idiopathic pulmonary fibrosis (IPF) and pulmonary fibrosis associated with collagen vascular disorders (PF-CVD). Although circulating immune complexes in patients' sera have been suggested, none of the antigens have been characterized. OBJECTIVES: The purpose of this study is to characterize the antigen of the immune complexes in patients' sera of pulmonary fibrosis. METHODS: As we previously established that one of the antibodies against A549 cells (lung alveolar type II cells) was anti-cytokeratin 8 (CK8), we confirmed the existence of anti-CK8 antibody in patients' sera by Western immunoblot. In addition, we tried to demonstrate circulating CK8:anti-CK8 immune complexes in patients' sera by Western immunoblot. Furthermore, we established an enzyme-linked immunosorbent assay to quantitate CK8:anti-CK8 immune complexes. RESULTS: In patients with pulmonary fibrosis, anti-CK8 antibodies were clearly demonstrated in sera by Western immunoblot. In addition, circulating CK8:anti-CK8 immune complexes were also clearly demonstrated by Western immunoblot. It was possible to establish ELISA to quantitate CK8:anti-CK8 immune complexes. If the cutoff value, which was determined based on the highest value of normal volunteers, was introduced, high CK8:anti-CK8 antibody complexes were demonstrated in 9 of 31 patients (29.0%) with IPF and PF-CVD. CONCLUSIONS: This is the first study to clarify the antigen of the circulating immune complex in sera of patients with IPF. These results suggest that circulating CK8:anti-CK8 immune complexes may have played a role in the process of lung injury in pulmonary fibrosis.

Aged↗

PubMed2006

Idiopathic pulmonary fibrosis: an update.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung condition of uncertain aetiology that should be considered in the differential diagnosis of patients who experience breathlessness, cough and reduced exercise tolerance. IPF is characterized histologically by the presence of usual interstitial pneumonia, and often has typical radiological appearances. Long-term successful management options are limited and frequently unsuccessful; as the disease progresses, palliation of symptoms becomes the mainstay of treatment. In a minority of patients, lung transplantation provides the only hope of long-term survival. The median survival of patients with IPF is approximately 3 years, which in turn emphasizes the need for further investigation into its pathogenesis and potential disease-modifying pharmacological therapies.

Acute Disease↗

PubMed2003

Diabetes mellitus may increase risk for idiopathic pulmonary fibrosis.

STUDY OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease of unknown etiology. The incidence of IPF increases with age. Aging contributes to lifestyle-related diseases, including diabetes mellitus (DM); therefore, it is possible that lifestyle-related diseases may affect either the initiation or progression of IPF. DESIGN: Case-control study. SETTING: Nippon Medical School and Omiya City Clinic. PATIENTS AND PARTICIPANTS: Sixty-five consecutive patients with IPF who were admitted to Nippon Medical School Hospital from 1995 to 2000, and 184 control subjects selected from 15,798 subjects who were admitted to Omiya City Clinic for routine medical examination between September 1999 and August 2000. MEASUREMENTS: Age, sex, smoking history, and results of physical examinations, blood examinations, and lung function testing were extracted from medical records and were compared with the diagnostic criteria for lifestyle-related diseases. RESULTS: The adjusted odds ratios for cigarette smoking were 5.40 (95% confidence interval [CI], 2.30 to 12.66) and 4.06 (95% CI, 1.80 to 9.15) for diabetes. There were no differences in clinical characteristics of patients with IPF that could be related to the presence of DM. CONCLUSION: DM may be a risk factor for IPF.

Aged↗

PubMed1986

Bronchoalveolar lavage fluid neutrophils increase after corticosteroid therapy in smokers with idiopathic pulmonary fibrosis.

Patients with idiopathic pulmonary fibrosis (IPF) are often cigarette smokers and are often being treated with corticosteroids at the time of bronchoalveolar lavage. We addressed the question of whether or not the bronchoalveolar lavage fluid (BALF) neutrophil content of patients with IPF undergoes changes in smokers different from those in nonsmokers after institution of corticosteroids. Eighteen patients were studied (10 smokers and 8 nonsmokers). Fourteen patients (6 smokers and 8 nonsmokers) were treated orally with prednisone. The histologic assessment of alveolar inflammation and inflammatory small airways disease was no different in smokers than in nonsmokers. None of the smokers treated with prednisone had pathologic evidence of emphysema in addition to IPF. Five of 6 smokers showed an increase in BALF neutrophils after 3 months of prednisone (p less than 0.05), whereas the nonsmokers' BALF neutrophils decreased or remained unchanged. This increase in BALF neutrophils in smokers was not associated with concomitant or subsequent clinical deterioration but, in fact, with clinical improvement after 3 months of therapy. These data indicate that the combination of cigarette smoking and corticosteroid therapy influences the BALF neutrophil content in patients with IPF and suggest that interval changes in BALF neutrophil content may not reflect the status of the inflammatory process or structural derangements in the lungs of some patients with IPF.

Aged↗