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

The universal features of the histopathology of fibrotic lung disease are derangement of parenchymal collagen and infiltration of the parenchyma with chronic inflammatory cells. To determine if this cellular reaction might be associated with autoimmunity to a consitituent of the alveolar interstitium, peripheral blood lymphocytes were exposed to human type I collagen in vitro and evaluated for the production of migration inhibition factor and cytotoxicity. Data from 18 patients with idiopathic pulmonary fibrosis, 8 patients with pulmonary fibrosis other than idiopathic pulmonary fibrosis, 12 patients with nonfibrotic lung disease, and 9 normals demonstrated that circulating lymphocytes from more than 94% of patients with fibrotic lung disease take part in processes where the recognition of collagen results in migration inhibition factor production and lysis of collagen-coated sheep red blood cells. These collagen-induced cell-mediated phenomena are obviated with human T-lymphocyte antiserum. Collagen-induced migration inhibition factor production and cytotoxicity were found in less than 20% of patients with nonfibrotic disease and were not found in normals. Qualitatively, there was no organ (lung, skin) or species (human, rabbit) collagen specificity in these assays, but human lung alpha 2 chains were recognized more often than alpha 1(I) chains. Circulating lymphocytes from patients with fibrotic disease are present in a normal T to B ratio. These lymphocytes did not incorporate [3H]thymidine when exposed to collagen but did when exposed to T-cell mitogens. These in vitro observations suggest that circulating T-lymphocytes and lung collagen may be intimately associated in the pathogenesis of human fibrotic lung disease.
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PMID:Pathogenic mechanisms in pulmonary fibrosis: collagen-induced migration inhibition factor production and cytotoxicity mediated by lymphocytes. 6 60

Pulmonary fibrosis was induced in eight baboons with bleomycin; five untreated animals were controls. After 45-65 U/kg of bleomycin, lung volumes and diffusing capacity were reduced, and static lung pressure-volume curves were shifted to the right. Right middle lobes were resected at this time in five bleomycin-treated and two control animals. Compared to controls, right middle lobes from bleomycintreated animals had increased weight and contained increased amounts of total protein, collagen, elastin, and DNA; synthesis of collagen and noncollagen protein were also elevated. Occasional alveolar septae were edematous and infiltrated by mononuclear inflammatory cells; a slight increase in collagen was demonstrable histologically. Four of six treated animals died with extensive diffuse interstitial fibrosis after 95 U/kg of bleomycin. Biochemical analyses revealed significantly elevated lobar contents of dry weight, protein, elastin, and collagen. Two animals survived 95 U/kg of bleomycin and were terminated 6 mo after treatment. In these animals, physiologic studies were indicative of restrictive lung disease, but lung histology was nearly normal. Lung weight, total protein, and DNA had returned to control values, but collagen and elastin were increased in amount and concentration. Bleomycin induces an intense inflammatory response in the lung. During this inflammation, connective tissue proliferation occurs in concert with proliferation of other tissue components. Cessation of bleomycin treatment is followed by resolution of inflammation manifested by decreases in tissue mass, cellular content, and nonconnective tissue protein. Collagen and elastin deposited during inflammation are less successfully removed during resolution, leading to a stage characterized by increased concentrations of these proteins. A similar sequence of tissue alterations may occur in idiopathic diffuse interstitial fibrosis of man in response to various lung injuries.
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PMID:Bleomycin-induced diffuse interstitial pulmonary fibrosis in baboons. 7 49

Two cases of fatal pneumopathy during cytostatic therapy for acute lymphatic leukemia of childhood, are reported with pathoanatomical lung findings and general clinical features. Histology revealed massed atypical epithelial proliferation in the bronchiolar terminal pathways (tumourlets) with multinucleated polymorphic giant cells beside pulmonary fibrosis. As causative factors for pulmonary chages hypersensitivity reactions, direct toxicity, or pharmacologic effects are discussed. Formal pathogenesis is explained by an impairment of endothelial cells in alveolar capillaries followed by permeability disorders and interstitial edema with disturbed perfusion. Disseminated intravasal microthrombi are frequent. Restitution to integrity appears possible only under favorable conditions. If the exsudative turns into the proliferative phase, intraalveolar and interstitial pulmonary fibrosis may develop with atypical epithelial proliferations. The prognosis of cytostatics-induced pneumopathies depends essentially on the time when it is diagnosied.
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PMID:Fatal pneumopathy after cytostatic treatment for leukemia in children. 28 47

Fibrosing alveolitis, or interstitial pulmonary fibrosis, is a common manifestation of neurofibromatosis, and was observed in 7 of 70 patients with the disease. Though neurofibromatosis is congenital, fibrosing alveolitis does not appear until adulthood, and occurs in 20% of patients with the disease who are over 30 years old. Characteristic radiographic findings include linear, interstitial density, and large upper lobe bullae; this combination limits the differential diagnosis. Pathological examination demonstrates alveolar wall thickening progressing to fibrosis and lung destruction. Pulmonary function tests can show obstructive or restrictive lung disease.
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PMID:Fibrosing alveolitis in patients with neurofibromatosis. 40 16

In world industry and agriculture as a whole, the number of people with asthma and complex pneumopathies related to chemical and organic pollution seems important. Indeed, subjects with an atopic inclination are often the first to be jeoparized. However, it must be stressed that occurrence of asthma in relation with work should always lead to investigate an anomaly in professional hygiene. For other workers this latter eventuality constitutes in the long run a threat of precipitin pneumopathy, chronic bronchitis, pulmonary fibrosis, or even cancer (in the case of nickel). Selection upon hiring is an unsatisfactory measure. The improvement of the atmospheric conditions at work should always be sought for. In some professional asthma cases, we were able to confirm that medication provides efficient protection. This solution, however, seems only slightly satisfactory since the subject is still left in contact with substances which have harmful effects other than asthma. It is therefore important that doctors track down and explore the cases of professional asthma, declaring their existence to social security and work inspection organizations, in order to establish an epidemiological knowledge, regularly updated, which would provide an indispensable basis for any prevention through improvement of working conditions.
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PMID:[Asthma and professional life (author's transl)]. 49 90

A case of pulmonary vasculitis complicating ulcerative colitis is presented, and the literature is reviewed. Unexplained pulmonary problems complicating severe ulcerative colitis should prompt a review of rare etiologies to include pulmonary vasculitis, apical pulmonary fibrosis, and salicylazosulfapyridine-induced lung disease. Lung biopsy will establish a diagnosis of pulmonary vasculitis.
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PMID:Pulmonary vasculitis complicating childhood ulcerative colitis. 58 12

Although the carcinogenic properties of asbestos are presently attracting a good deal of attention, asbestosis is still the earliest lung disease resulting from exposure to it and its incidence in an exposed population is the most useful indicator of the degree of dust control exercised over a period of time. The diagnosis of asbestosis depends upon: (1) An adequate occupational exposure history. (2) Physical signs of pulmonary fibrosis. (3) Progressive radiological changes. (4) Confirmatory measurements of altered lung function. Asbestosis is a clinical entity and is readily diagnosed when all the above-mentioned criteria are met. Problems in diagnosis are encountered when one or more of the diagnostic criteria listed above cannot be substantiated. At the present time every effort is made to diagnose the disease in its early stages in the hope that removal from further exposure will prevent the direct and indirect complications.
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PMID:Asbestosis -- a diagnostic enigma: a personal view. 59 91

Electron microscopic studies of lung were made and compared in 17 patients with lung disease (10 with idiopathic pulmonary fibrosis, 3 with collagen--vascular diseases, 3 with sarcoidosis, and 1 with chronic eosinophilic pneumonia) and in 5 control patients. In control patients, the alveolar epithelial cells were normal, and no hemidesmosomes were present between the plasma membranes and the basal laminae. In comparison, cuboidal alveolar epithelial cells were present in 15 of the patients with fibrotic lung disease; in 9 of these the alveolar epithelial cells were multilayered. In 7 of the latter 9 patients (5 with idiopathic pulmonary fibrosis and 2 with collagen-vascular diseases), the basal laminae of the alveolar epithelial cells were attached to the plasma membranes by hemidesmosomes and to the underlying interstitial connective tissue by "anchoring fibrils." These fibrils measured from 4000 to 6000 A in length and from 200 to 600 A in width. One or both ends of the anchoring fibrils inserted into thebasal lamina, often forming arcs through which collagen fibrils and connective tissue microfibrils penetrated. Anchoring fibrils showed a complex pattern of transverse banding, which differed from that of collagen and appeared to be symmetric about the center of the fibril. These anchoring fibrils, which resemble those in normal skin and other tissues, were not found in lungs of control patients. In addition, there was a significant correlation between the severity of the pulmonary fibrosis and the presence of anchoring fibrils. These observations suggest that in severe fibrotic lung disease, anchoring fibrils reinforce the attachment of the basal lamina of multilayered alveolar epithelial cells to interstitial connective tissue.
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PMID:Anchoring fibrils. A new connective tissue structure in fibrotic lung disease. 67 68

A 12-year-old boy was hospitalized for resection of a bronchiectatic lesion. Investigation of an elevated cencentration of serum IgE led to a diagnosis of allergic bronchopulmonary aspergillosis. ABPA has rarely been described in the pediatric age group. This hypersensitivity lung disease is characterized by intermittent wheezing, fever, recurrent pulmonary infiltrates, eosinophilia, hyperimmunoglobulinemia E, and Type I (allergic) skin reactivity to aspergillus extract. Hyphae of aspergillus may also be found in expectorated brown mucus plugs. Type III (Arthus) skin test response and presence of precipitating antibody to this fungus may be demonstrated. Central bronchiectasis or pulmonary fibrosis may result from uncontrolled progression of this disease.
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PMID:Hyperimmunoglobulinemia E in a child with allergic bronchopulmonary aspergillosis and bronchiectasis. 93 1

Primitive interstitial pneumopathies are only a temporary classification. They seem to be due to an evolution for the worse of a "diffuse alveolar damage" of unknown origin. Evolution towards pulmonary fibrosis and respiratory insufficiency is their common risk. Etiopathogenic hypotheses would favour the immunological etiology. A diagnosis of primitive interstitial pneumopathy is reached by elmination when no known cause can be found. Liebow's classification remains valuable for pathology but is debatable where anatomo-clinical correlations, etiological hypotheses and the eventual effect of treatment are concerned. Today these primitive forms still represent 40% of the interstitial lung diseases, the causes of which are progressively discovered.
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PMID:[Primary interstitial pneumopathies]. 100 68


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