Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It has been well known that the number of mast cells increases during the development of fibrosis in various tissues including the lung. However, the role of mast cells in fibrosis still remains obscure. In the present paper, we evidenced that pulmonary fibrosis could be induced in genetically mast cell-deficient WBB6F1-W/Wv mice as well as WBB6F1-(+/+) mice having mast cells normally by the treatment with bleomycin (BLM, 5 mg/kg, i.v., 10 days), and there was not much difference in the histological changes of lungs between the two strains. An increase in the hydroxyproline content of the lung of WBB6F1-W/Wv mice was rather higher than that of WBB6F1-(+/+) mice. Previously, we reported that tranilast, an antiallergic drug inhibiting chemical mediator release from mast cells, suppressed the development of BLM-induced pulmonary fibrosis in ICR mice, suggesting the possibility that mast cells play certain roles in fibrosis. However, it was evidenced in the present report that tranilast suppressed BLM-induced fibrosis in WBB6F1-W/Wv mice. Tranilast neither suppressed the cytotoxic activity of BLM against KB cells and L-929 cells in vitro, nor inhibited the antitumor activity of BLM against Sarcoma-180 transplanted subcutaneously into ICR mice. Tranilast may act through suppressing BLM-induced activation of lymphoid cells including macrophage and neutrophil. These results indicate an inconsequential role of mast cells in the development of fibrosis. Increases in the number of mast cells and in histamine content of the lung, which were widely reported in the lungs of BLM-treated mice, may be the result of fibrosis.
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PMID:Bleomycin-induced pulmonary fibrosis in genetically mast cell-deficient WBB6F1-W/Wv mice and mechanism of the suppressive effect of tranilast, an antiallergic drug inhibiting mediator release from mast cells, on fibrosis. 171 9

We report the pulmonary pathologic features in 87 open lung biopsies from 67 patients with Wegener's granulomatosis (WG) who were treated at a single institution from 1968 to 1990. At the time of open lung biopsy, 48 patients (72%) had classical WG with renal involvement; 19 (28%) had limited WG without renal involvement. The pathologic features were divided into major and minor manifestations. In the 82 specimens demonstrating no infectious organism, the three major pathologic manifestations of classical WG observed were also useful diagnostic criteria and included: (a) parenchymal necrosis, (b) vasculitis, and (c) granulomatous inflammation accompanied by an inflammatory infiltrate composed of a mixture of neutrophils, lymphocytes, plasma cells, histiocytes, and eosinophils. Parenchymal necrosis was found in 84% of biopsy specimens either as neutrophilic microabscesses (65% of specimens) or as large (67%) or small (69%) areas of geographic necrosis. Areas of geographic necrosis were usually surrounded by palisading histiocytes and giant cells. Additional granulomatous lesions consisted of microabscesses surrounded by giant cells (69%), poorly formed granulomas (59%), and scattered giant cells (79%). Sarcoid-like granulomas were uncommon (4%), and in only one specimen (1%) appeared within an inflammatory lesion of WG. Vascular changes were identified in 94% of biopsy specimens. Vascular inflammation was classified as chronic (37% arterial, 64% venous), acute (37% arterial, 29% venous), non-necrotizing granulomatous (22% arterial, 9% venous), and necrotizing granulomatous (22% arterial, 10% venous). Fibrinoid necrosis was relatively uncommon (11% arterial, 6% venous). Cicatricial changes were found in arteries in 41% of biopsy specimens and in veins in 16%. Capillaritis was present in 31% of specimens. Minor pathologic lesions were commonly observed in biopsy specimens associated with classical WG lesions, but they were usually inconspicuous and not useful diagnostic criteria. These included interstitial fibrosis (26%), alveolar hemorrhage (49%), tissue eosinophils (100%), organizing intraluminal fibrosis (70%), endogenous lipoid pneumonia (59%), lymphoid aggregates (37%), and a variety of bronchial/bronchiolar lesions including acute and chronic bronchiolitis (51% and 64%), follicular bronchiolitis (28%), and bronchiolitis obliterans (31%). These minor lesions were often found at the periphery of typical nodules of WG. However, in 15 specimens (18%) a minor pathologic feature represented the dominant or major finding: pulmonary fibrosis (six specimens, 7%), diffuse pulmonary hemorrhage (six specimens, 7%), lipoid pneumonia (one specimen, 1%), acute bronchopneumonia (one specimen, 1%), and chronic bronchiolitis, bronchiolitic obliterans with organizing pneumonia (BOOP), and bronchocentric granulomatosis (one specimen, 1%).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Surgical pathology of the lung in Wegener's granulomatosis. Review of 87 open lung biopsies from 67 patients. 200 12

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

Intratracheal injection of a small dose of bleomycin in rats induced early alveolar epithelial cell injury and a pneumonitis which subsequently evolved to pulmonary fibrosis. Hydropic degeneration of type I pneumocytes was apparent at 3 days after treatment. Marked interstitial and intra-alveolar pneumonitis developed at 7 days after treatment and was accompanied by hypertrophy and hyperplasia of type II pneumocytes. The inflammatory cell population consisted predominantly of cells with the morphology of large lymphocytes together with a number of eosinophils. Examination by immunoperoxidase and histochemical staining of frozen sections revealed that the lymphoid cells stained positively with the monoclonal antibodies W3/13 and W3/25 but not with other markers. Thus these cells appeared to be helper T lymphocytes. The later development of interstitial fibrosis was accompanied by alveolar microcollapse which contributed to the thickening of alveolar septa observed by light microscopy. The possible role of immunologic and other mechanisms in the pathogenesis of bleomycin-induced pulmonary fibrosis is discussed.
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PMID:Pulmonary responses to bleomycin-induced injury: an immunomorphologic and electron microscopic study. 241 89

A male infant in whom multiple recurrent multiorgan infections developed during the first six months of life was found to have combined immunodeficiency. Progressive pulmonary disease developed at age two years; cytomegalovirus (CMV) was isolated from the respiratory tract and urine. Three separate intramuscular grafts of cultured thymus fragments did not produce change in the course of the illness. Soon after age three years, IgG lambda appeared in the serum as an M-component. The patient died at age three and one-half years, with respiratory insufficiency due to pulmonary fibrosis. At autopsy, a malignant plasma cell infiltrate was limited to the retroperitoneum. The infiltrate replaced lymph node structures and surrounded nerve fascicles, which appeared necrotic, and contained CMV inclusions in ganglion cell nuclei. The plasma cells showed strong monoclonal staining for IgG lambda. Also noted was positive staining for J-chain, which has been reported previously in malignant plasma cells producing IgG. CMV could be responsible for abnormal B-cell proliferation in patients with defective immunoregulation who receive immunotherapy, as in lymphoid abnormalities associated with Epstein-Barr virus.
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PMID:Abnormal B-cell proliferation associated with combined immunodeficiency, cytomegalovirus, and cultured thymus grafts. 608 43

The purpose of this study was to analyze the cellular components of bronchoalveolar lavage fluid throughout the development of bleomycin-induced pulmonary fibrosis in the rat. Animals were killed and lavaged at various times after the administration of a single intratracheal injection of bleomycin. The results demonstrate that a significant influx of inflammatory cells appear in the lavage fluid as early as Day 1 after bleomycin treatment. Polymorphonuclear leukocytes are the first cells to appear and significant concentrations persist for as long as 1 month after bleomycin treatment. There is a very transient yet significant influx of eosinophils on Day 7 after bleomycin treatment. Lymphocytes are present from 3 to 14 days after bleomycin treatment; greater than 97% are T-cells and less than 3% are B-cells. There is a 1:1 ratio of W3/25+ cells (helper cell activity) to OX8+ cells (suppressor cell activity) comprising the lymphocyte population. The blood and lymphoid tissue of these animals contain a normal 2:1 ratio of these subsets. The data demonstrate that specific T-cell populations are present in the air spaces of the lung in response to bleomycin-induced pulmonary fibrosis in this model.
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PMID:Differential cellular analysis of bronchoalveolar lavage fluid obtained at various stages during the development of bleomycin-induced pulmonary fibrosis in the rat. 618 23

Upon analysis of 550 cases of different chronic diffuse pulmonary diseases included in a group of interstitial diseases of the lungs (IDL) the authors came to the conclusion that IDL incorporate such variants as alveolitis, pulmonary vasculitis and pulmonary hemorrhages; granulomatosis covers exogenic allergic alveolitis, alveolitis in chronic active hepatitis; vasculitis group includes such rare diseases as necrotizing sarcoid granulomatosis vasculitis and lymphoid granulomatosis; fibrosing alveolitis--secondary alveolitis in sclerodermia systematica, rheumatoid arthritis, Sjogren's disease, chronic active hepatitis. Knowledge of IDL etiology (environmental, occupational, induced by radionuclides, drugs, viruses, fungi) with focus on drug affection of the lungs is thought of value. Biopsy and bronchial lavage findings are compared clinically and morphologically. Mechanisms of pulmonary fibrosis and approaches to inhibition of pulmonary fibrosis progression are outlined.
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PMID:[Interstitial lung diseases--the clinical aspects of the problem]. 763 86

We treated two types of experimental pulmonary fibrosis elicited in mice by the intratracheal instillation of bleomycin or silica with monoclonal antibodies (mAbs) specific for the leukocytic integrins CD-11a or CD-11b. This treatment completely prevented collagen deposition, as measured by lung hydroxyproline content on Day 15 after instillation. Furthermore, anti CD-11a mAb was also effective when given on Days 20 and 25 and the lung hydroxyproline content determined on Day 30 after instillation, i.e., in the treatment of an established pulmonary fibrosis. Histologic studies indicated that anti CD-11 mAbs attenuated the fibrosing alveolitis induced by silica or bleomycin and in addition markedly decreased the lymphoid infiltration and platelet microthrombi associated with both types of alveolitis. In contrast, these mAbs had little or no effect on the cellularity of the bronchoalveolar lavage, mainly composed of macrophages. In normal mice, anti CD-11 mAbs also decreased the number of interstitial lymphocytes and the lung collagen content. These observations may lead new to therapies for pulmonary fibrosis.
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PMID:Effective treatment of the pulmonary fibrosis elicited in mice by bleomycin or silica with anti-CD-11 antibodies. 767 65

Levels of the N-terminal propeptide of type III collagen (PIIINP) in bronchoalveolar lavage fluid (BALF) are thought to reflect type III collagen production by the lungs, and increased levels have been reported in patients with pulmonary fibrosis. We wanted to know more about the relative proportions of these peptides in normal BALF, whether they altered in pulmonary fibrosis, and whether lymphoid tissue is capable of clearing PIIINPs. In this study, we used a radioimmunoassay which detects the different forms of PIIINP-related antigens with equal specificity, to measure PIIINPs in serum and BALF of patients with cryptogenic fibrosing alveolitis (CFA). To investigate why PIIINP profiles in BALF differed from serum, the absolute concentration and relative proportion of PIIINPs in lymph afferent and efferent to the popliteal lymph node of a sheep were also determined. PIIINP concentrations were significantly greater in serum and BALF of patients with CFA, compared with controls. Gel chromatography indicated that serum antigen distribution, both of patients and controls, contained approximately 20% Col 1-3; the remainder being Col 1. In contrast, BALF contained Col 1-3 and Col 1, together with an antigen of high molecular weight (> 150 kD). The relative proportion of each antigen varied quite widely, but there were no apparent differences between patients and controls. The concentration of PIIINPs in afferent lymph was 295 ng.ml-1 and in efferent lymph was 104 ng.ml-1. Gel chromatography demonstrated that a significant amount of Col 1-3, together with a high molecular weight peptide, had been cleared during passage through the node.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Heterogeneity of type III procollagen N-terminal peptides in BAL fluid from normal and fibrotic lungs. 811 36

A 61-year-old man was admitted to our hospital on October 8, 1991 because of abnormal shadows on chest X-ray at annual checkup at his company. Chest X-ray and CT on admission showed diffuse reticular shadows in bilateral lower lung fields and a nodular opacity approximately 10 mm in diameter in the right lower lung. Since transbronchial lung biopsy was not diagnostic, an open lung biopsy was performed on October 28, 1991. The lung specimens showed diffuse pulmonary fibrosis compatible with usual interstitial pneumonia and an intrapulmonary lymph node containing silicotic nodules. Only 29 cases (including the present case) of intrapulmonary lymph nodes have been reported. Although the causes of intrapulmonary lymph nodes are not clear, smoking is considered to play an important role in the development of pulmonary lymphoid tissue. In our case, the intrapulmonary lymph node contained silicotic nodules. Only several case have been reported to have silicotic nodules in the lymph nodes. As suggested by Kradin, they may be induced by relatively low levels of exposure to dust. Our case also had pulmonary fibrosis (IIP), and is the first reported case of intrapulmonary lymph node associated with IIP. Although it is difficult to determine these two diseases occurred coincidently or not, it is possible that a low level of dust exposure may have contributed to both silicotic nodules in the lymph node and IIP.
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PMID:[A case of intrapulmonary lymph node with silicotic nodules in a patient with idiopathic interstitial pneumonia]. 846 13


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