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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary fibrosis is an irreversible accumulation of connective tissue in the interstitium of the lung. The pathogenesis of pulmonary fibrosis is not well understood. Research on animal models and studies of human lung disease suggest the initiating events may be a combination of pulmonary injury and the recruitment of inflammatory cells, mainly macrophages. A number of well characterized cytokines, including TGF-beta, have been either found in the injured lung or produced by inflammatory cells removed from the lung. In an animal model of pulmonary fibrosis, TGF-beta production is increased prior to collagen synthesis and is mainly produced by alveolar macrophages. In advanced idiopathic pulmonary fibrosis, a human fibrotic lung disease, extensive TGF-beta deposition can be detected by immunohistochemical staining, primarily in epithelial cells in areas of lung regeneration and remodelling. This suggests that the pathogenesis of the progressive fibrosis characteristic of this lung disease may be an aberrant repair process.
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PMID:The role of TGF-beta in pulmonary fibrosis. 171 97

Lung transplantation currently involves the allografting of one or both lungs from a cadaver or brain-dead donor to selected patients with progressive end-stage pulmonary disease for whom there are no other viable treatment options. Expanding experience since 1986 in Canada, the United States, and Europe has demonstrated that both SLT and DLT can provide adequate pulmonary function and palliation for extended periods in some patients with otherwise fatal lung disease. A more rapid expansion of this technology has been constrained by the scarcity of suitable donors and the current limits of organ preservation time. Lung transplantation has evolved as a clinical procedure achieving a favorable risk-benefit ratio and acceptable 1- and 2-year survival rates. Lung transplantation is applied as a therapeutic option for patients with end-stage pulmonary disease. However, the transplant community has not yet reached consensus regarding patient selection criteria or absolute contraindications to LT. Specific selection criteria for the optimal treatment of all LT candidates do not exist as yet and are currently evolving. The majority of candidates have had pulmonary fibrosis, bronchiectasis, emphysema, pulmonary hypertension, or CF. Additional clinical information and experience will be useful in refining evaluation of risk-benefit ratios of SLT and DLT, which may vary for specific subsets of patients.
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PMID:Single and double lung transplantation. 182 79

From 1975 until 1987, 122 patients without (Group I, mean age 34 yrs) and 20 with pre-existing lung disease (Group II, mean age 66 yrs) were treated for their first spontaneous pneumothorax. Mean follow-up was 5.2 yrs (max. 12.8 yrs). There were 32 chest X-ray verified recurrences, 72% occurring during the first 2 yrs. One, 5 and 10 yr cumulative recurrence freedoms +/- standard error in groups I and II combined were 85 +/- 3%, 75 +/- 4% and 69 +/- 5%, respectively. Using Cox regression analysis in groups I and II combined, pulmonary fibrosis, age greater than or equal to 60 yrs, and height/weight ratio were independent predictors of recurrence. Combinations of these risk factors identified four risk strata with 10 yr recurrence freedoms ranging from 83-0%. Independent predictors in group I alone were pulmonary fibrosis, age greater than or equal to 60 yrs, height/weight ratio, and nonsmoking, resulting in four risk strata with 10 yr recurrence freedoms ranging from 98-31%. Recurrences after first-time treated spontaneous pneumothorax showed a distinct time-related pattern and should accordingly be analysed as a time-related event. In both the total patient-population and in the group without pre-existing lung disease, independent predictors of recurrence were identified, which allowed the patients to be substratified into groups with widely different recurrence rates.
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PMID:Independent risk factors for cumulative recurrence rate after first spontaneous pneumothorax. 186 47

Five cases of juvenile progressive systemic sclerosis (SSc) are reported (4 girls and 1 boy). The age of onset of the disease ranged from 4 to 13 years. The clinical features included Raynaud's phenomenon present in 4 of 5 cases; hyperpigmentation, skin tightening and contractures of the large joints were noted in all 5 cases. One patient initially diagnosed as having eosinophilic fasciitis developed SSc 3 months later. Another patient was diagnosed initially as having juvenile rheumatoid arthritis. There was one case of pulmonary fibrosis and another of mild restrictive lung disease. Two cases of esophageal and intestinal hypomotility were reported. Scleroderma nephropathy was absent in all 5 cases.
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PMID:Juvenile progressive systemic sclerosis: report of five cases. 189 68

At the stage of established pulmonary fibrosis there is no effective treatment. Hence the necessity of an early diagnosis at the "cellular" stage of diffuse interstitial fibrosing pneumopathy, which offers the best chances of improvement under corticosteroid therapy. Current biopathological researches on the dynamic mechanisms of fibrosis (fibrogenesis, fibrolysis) should open therapeutic lines involving cytokines and/or their receptors, including at the stage of gene transcription.
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PMID:[Treatment of diffuse interstitial pulmonary fibrosis]. 189 8

I briefly review comments I made concerning recent advances in techniques of cell and molecular biology, including molecular cloning and characterization of genes, gene therapy, and newer applications of these techniques to the study of lung disease during the 1990 annual meeting of the Swiss Society of Pneumonology. Recent preliminary findings from my own laboratory utilizing the example of immunohistochemical and in situ hybridization techniques to study connective tissue and cytokine growth factor expression and remodeling in human pulmonary fibrosis are also discussed.
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PMID:Applications of cell and molecular biology to pneumonology. 200 9

The modern era of lung transplantation followed the introduction of cyclosporin and the development of en bloc heart-lung transplantation in 1980. This was followed in 1983 by the first long-term successful unilateral lung transplant by the Toronto group. The same group carried out the first successful double lung transplantation in 1986. Heart-lung transplantation remains the most common operation for patients with end-stage lung disease but is being gradually replaced by single lung transplantation for end-stage pulmonary hypertension, pulmonary fibrosis and emphysema. The major indication for double lung transplantation is cystic fibrosis or emphysema in subjects less than 45 years of age. The probability of survival after lung transplantation is approximately 65% after one year and 60 and 50% after two and three years respectively. This compares favorably to the early results obtained with heart transplantation. These survival figures represent a marked improvement in the prognosis of patients with end-stage lung disease who are candidates for lung transplantation since such subjects should have an expected survival of less than two years without this intervention. The major problems encountered with lung transplantation are technical difficulties during the operation, serious infections and acute episodes of rejection, both of which are most common in the first six months following the operation, and bronchiolitis obliterans, which is a late complication most probably related to chronic rejection. Organ availability remains a major limiting factor to the availability of lung transplantation and, as a consequence, numerous patients die while awaiting a donor organ.
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PMID:[Lung transplantation: the current state of knowledge]. 204 38

The clinician confronted to the diagnosis of infiltrative lung disease has to consider a wide spectrum of aetiologies. Among them, diffuse fibrosing interstitial lung disease is characterized by the deposition of collagen within alveolar structures. Either acute alveolar injury or chronic inflammatory alveolitis can induce pulmonary fibrosis. Basic research investigates the cells, the cytokines, and the extracellular matrix involved in the perpetuation of the fibropoliferative process.
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PMID:[Diffuse infiltrative lung diseases and fibrosis]. 206 14

In order to shed light on the histological changes occurring in the lungs of patients with rheumatoid arthritis (RA), we scrutinized an open lung biopsy file of 199 patients and selected the patients with RA. The histopathological patterns observed were: pulmonary rheumatoid nodules (4 cases, including one with rheumatoid pneumoconiosis); usual interstitial pneumonia (UIP) (2 cases); desquamative interstitial pneumonia (2 cases); bronchiolitis obliterans with patchy organizing pneumonia (2 cases); follicular bronchiolitis (1 case); organizing pneumonia always associated with bronchiolitis (3 cases); granulomatous reaction (3 cases); obliterating vasculitis (3 cases); granulomatous vasculitis (1 case); lymphoid hyperplasia (2 cases); and localized pulmonary fibrosis (1 case). The clinical data and laboratory findings for the histopathological groups overlapped and did not properly predict the anatomical picture. Both patients with UIP died of lung disease. Otherwise the prognosis in the series was good.
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PMID:Open lung biopsy of patients with rheumatoid arthritis. 208 43

Pulmonary function was studied in 48 patients 4-13 yrs after treatment for Hodgkin's disease with mantle-field irradiation followed by standard mechlorethamine, Oncovin, procarbazine and prednisone (MOPP) chemotherapy. The patients were found to have a restrictive lung disease suggestive of pulmonary fibrosis. Low age at therapy (less than or equal to 30 yrs, median 24 yrs) was associated with a significantly more pronounced restrictive lung function impairment than older age (greater than 30 yrs, median 40 yrs) suggesting a higher susceptibility to the pulmonary side-effects of therapy. In addition younger smokers had a significantly greater reduction in diffusion capacity and forced expiratory volume in one second than older smokers, suggesting a higher susceptibility to the additional adverse effect of smoking. With longer follow-up nonsmokers had an increase in static lung volumes. It is suggested that this may be the result of more frequent pulmonary infections in such patients as compared with the general population. However, the duration of follow-up was not associated with changes in other indices of lung function.
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PMID:Influence of age and duration of follow-up on lung function after combined chemotherapy for Hodgkin's disease. 209 Apr 76


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