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

Six obstetric patients with severe pulmonary insufficiency are presented. The volume respirator and quality intensive care remain the basis of treatment. Experience with extracorporeal oxygenation is discussed. The patients demonstrated the typical progression seen in lung disease. An acute, severe insult is followed by hypoxia and pulmonary insufficiency with ensuing pulmonary fibrosis. Serious infection, coagulopathies, and mechanical problems of ventilation add to the difficulty of treatment and the high mortality rate in what may be referred to as an adult respiratory distress syndrome. It is important for the clinician to take an aggressive approach in the management of severe respiratory insufficiency.
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PMID:Pulmonary insufficiency associated with pregnancy. 111 66

Left heart diseases, in particular mitral stenosis, are often associated with anatomic and functional alterations of the lung. According to the pulmonary structures involved they could be named chronic secondary intersticial and vascular lung diseases. Congenital heart diseases with pre- or post-tricuspid shunts are also often associated with anatomic and functional alterations of the lung. This condition also constitutes a chronic secondary vascular lung disease (atrial septal defect) or a chronic primary vascular lung disease ( ventricular septal defect, patent ductus arteriosus). Primary lung diseases (interstitial pulmonary fibrosis, pulmonary emphysema, recurrent pulmonary embolism) are often associated with right ventricular hypertrophy with or without dilation, a condition commonly named chronic cor pulmonale. On the whole the interrelationships between heart and lung diseases are as follows: a) anatomic and functional alterations of the lung due to left heart diseases are mediated through pulmonary venous hypertension; b) anatomic and functional alterations of the lung due to congenital heart diseases are mediated through the increased pulmonary blood flow with or without transmission of the systemic blood pressure to the pulmonary vasculature, and c) anatomic and functional alterations of the right ventricle due to primary or secondary lung diseases are mediated through arterial pulmonary hypertension. In summary, the interrelationships between heart and lung diseases are mainly mediated through the pulmonary venous or pulmonary arterial hypertension.
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PMID:Cardiac and pulmonary diseases. A pathophysiologic interelationship. 113 Sep 7

Chest radiographs of 39 patients with ankylosing spondylitis were studied. Three showed apical pulmonary fibrosis, two with cavitary lesions. Other known causes of lung disease were excluded. Symptoms and roentgenographic evidence of spondylitis were present for many years prior to the onset of pulmonary symptoms, which variably included shortness of breath, cough, hemoptysis, pleuritic chest pain, fever, and chills. Apical pulmonary lesions of unknown cause were absent in 53 age, sex, and racematched osteoarthritis control patients. The findings suggest that apical pulmonary fibrosis may be an extra-skeletal manifestation of ankylosing spondylitis, the frequency of which approaches that of spondylitic heart disease.
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PMID:Pulmonary manifestations of ankylosing spondylitis. 120 76

Two examples of immunologically mediated diseases of the lung have been discussed. The first, extrinsic allergic alveolitis, is caused by sensitivity to inhalation of an organic dust. It is largely a restrictive type of lung disease and is probably caused by a combination of type 3 and type 4 allergic reactions. The second disease, allergic bronchopulmonary aspergillosis, is caused by a type 1 and type 3 allergic response to Aspergillus antigen in a basically atopic, generally asthmatic individual. It is vital to keep the diagnostic index of suspicion high so as to recognize these diseases early enough to prevent irreparable tissue damage. Extrinsic allergic alveolitis may masquerade as idopathic pulmonary fibrosis or Hamman-Rich syndrome. Allergic aspergillosis may be called Loeffler pneumonia or PIE syndrome (pulmonary infiltrates with eosiniphilia). Failure to diagnose properly may result in extensive pulmonary fibrosis or bronchiectasis and condemn the patient to a lifetime as a pulmonary cripple.
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PMID:The Jewish Hospital of St Louis Therapeutic Grand Rounds No. 14. Hypersensitivity pneumonitis. 125 4

The authors report ten cases of drug induced lung diseases, complicated by respiratory failure of whom five were attributed to cytotoxic drugs and five to non cytotoxic drugs. The drug induced lung disease presented as acute respiratory distress syndrome in two cases, alveolar interstitial lung disease in three cases, purely interstitial in five cases. There was acute respiratory failure (ARF) in eight cases and chronic respiratory failure (CRF) in two cases. Among the five patients admitted for cytotoxic drug induced lung disease and ARF, four recovered and one died of diffuse destructive pulmonary fibrosis. Among the five patients having non cytotoxic drug induced lung disease, three were in ARF and recovered. The other two had CRF and died of diffuse pulmonary fibrosis. The diagnostic of drug induced lung disease was established in each case with the chronology of the clinical events, the exclusion of other possible causes of the lung disease and the evolution after removal of the incriminated drug. Broncho-alveolar lavage (BAL) had a major diagnostic value. It was contraindicated by respiratory failure in five cases. The predominant alveolar cell type was lymphocyte (four cases), eosinophil (three cases) and neutrophil (one case), BAL was realized with a provocation test and demonstrated the pathogenic role of cyclothiazide in one case. No specific information was given by histology. The prognosis did not seem to be linked to the severity of the initial clinical picture, or to the nature of the underlying neoplastic disorder, but to the degree and evolution of the pulmonary fibrosis.
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PMID:[Drug-induced pulmonary diseases: diagnostic, therapeutic and prognostic aspects. Apropos of 10 personal case reports]. 128 22

The influence of dexamethasone on levels of total fibronectin (tFn), cellular fibronectin (cFn), plasma fibronectin (pFn), and albumin in lung secretions was determined in tracheal aspirate samples collected from 45 infants with bronchopulmonary dysplasia during a 6-week course of dexamethasone therapy. Secretory component for IgA (SC) was used as a reference protein. Thirty-seven infants (82%) survived and had their endotracheal tubes successfully removed. Corticosteroid therapy was associated with a significant decrease in the cFn/SC ratio. There was also a significant decrease in albumin/SC and pFn/SC ratios, suggesting decreased capillary permeability with corticosteroid therapy. Four of the remaining infants did not improve while receiving corticosteroids and died of respiratory failure at 3 to 8 weeks of age. In these "no response" infants, tFn/SC, cFn/SC, pFn/SC, and albumin/SC ratios when corticosteroid therapy was initiated were threefold to fourfold greater (p < 0.01) than ratios in survivors. Another group of four infants initially responded to corticosteroids but subsequently died with severe pulmonary cystic degeneration at 4 to 6 months of age; in these infants, tracheal aspirate tFn/SC, cFn/SC, and albumin/SC ratios were significantly lower than in survivors and remained unchanged during corticosteroid therapy. The decrease in the concentrations of plasma fibronectin and albumin in tracheal aspirate samples from the survivors suggests that the rapid clinical improvement seen in infants with bronchopulmonary dysplasia after the initiation of dexamethasone therapy is due in part to improvement in the integrity of the alveolar-capillary barrier. In addition, the decrease in the aspirate levels of cFn suggests the potential for corticosteroids to limit pulmonary fibrosis in the surviving infants. The depressed levels of fibronectin observed in the infants with severe cystic lung disease may represent an impaired healing response to lung injury.
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PMID:Effect of dexamethasone therapy on fibronectin and albumin levels in lung secretions of infants with bronchopulmonary dysplasia. 140 98

Oxygen-mediated lung injury can stimulate a fibroproliferative response resulting in the alteration of the pulmonary extracellular matrix and subsequent scarring of parenchymal tissue. Fibronectin (FN), a component of the extracellular matrix, appears in increased quantities in fibrotic lung disease. Alveolar macrophages (AMs) are a potential source of this molecule. Using quantitative in situ hybridization, we demonstrated that AMs from rabbits acutely exposed to 100% oxygen (hyperoxia) for up to 64 h have 20-fold greater levels of FN mRNA relative to cells from control animals. When animals were allowed to recover in room air for up to 72 h after maximal oxygen exposure, AM FN mRNA abundance approached baseline levels. Furthermore, in oxygen-exposed animals, the fraction of lavaged cells expressing FN mRNA was increased 10-fold relative to controls. Although there was marked cell-to-cell variation, we conclude that the AM is a potential source of FN in the events leading to hyperoxia-induced pulmonary fibrosis.
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PMID:Increased fibronectin mRNA in alveolar macrophages following in vivo hyperoxia. 141 30

Significant progress has been made since the first successful human heart-lung transplantation (HLT) for pulmonary vascular disease performed in 1981. The refinement of surgical techniques, use of cyclosporin as the main immunosuppressant, technique of distant organ procurement to expand the donor organ pool, and improved diagnosis and management of pulmonary infection and rejection have all contributed to this accomplishment. This has inevitably coincided with the extension of this procedure to other groups of patients with end stage heart and lung disease. Initially, HLT was offered to patients with cardiac disease associated with pulmonary hypertension. Because of the success, consideration was given to transplantation for parenchymal pulmonary diseases, initially pulmonary fibrosis and emphysema, and then suppurative lung disease such as in cystic fibrosis (CF). However, the application of HLT to patients with CF lagged behind because of concern related to the risk of sepsis, the systemic nature of the disease, malnourishment, and fear of recurrence of the epithelial CF defect in the transplanted lungs.
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PMID:Lung transplantation for cystic fibrosis. 145 9

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease caused by bronchial colonization with Aspergillus fumigatus that affects approximately 10% of patients with cystic fibrosis (CF). The diagnosis in CF patients is difficult because the cardinal symptoms of ABPA occur frequently in CF, ie, pulmonary infiltrates and wheezing, as well as the frequent colonization with A fumigatus that leads to humoral reactivity. If left untreated, ABPA leads to bronchiectasis and pulmonary fibrosis. The pathogenesis of ABPA seems to be a prolonged asthmatic late-phase reaction orchestrated by CD4+ Th2-like T cells in response to persistent pulmonary A fumigatus allergen exposure. Thus, polyclonal and A fumigatus-specific IgE antibodies (and IgA and IgG) and blood pulmonary eosinophilia are stimulated by Th2-derived cytokines such as IL-4 and IL-5. In addition, IL-4 would also promote pulmonary transendothelial migration of eosinophils, basophils, and lymphocytes via induction of cell adhesion molecules and their ligands. IgE mast cell interactions would also contribute to the bronchial reactivity and inflammation. Recent advances have begun to identify immunodominant A fumigatus allergens. Evaluation of the quantity of IgE antibodies (and IgA and IgG) and T-cell cytokine responses to specific A fumigatus allergens should aid in the diagnosis and immunopathogenesis of ABPA, especially in CF patients.
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PMID:Allergic bronchopulmonary mycosis complicating cystic fibrosis. 147 42

Pulmonary involvement in scleroderma is characterized by interstitial fibrosis and pulmonary hypertension. Although bronchiolitis obliterans organizing pneumonia (BOOP) may be associated with a variety of connective tissue diseases and their treatment, there are only rare reports of bronchiolitis associated with scleroderma. We describe 2 patients with scleroderma and rapidly evolving pulmonary infiltrates, which upon biopsy showed histologic findings of BOOP. Each patient had severe restrictive lung disease and markedly diminished diffusion capacity. Treatment with high dose prednisone showed a good response in one patient. The rapid course of pulmonary findings in these patients differs from the usual course of pulmonary fibrosis in scleroderma. Although BOOP is a rare finding in scleroderma, our findings suggest that rapid pulmonary decompensation or atypical findings for pulmonary fibrosis should prompt consideration for an open lung biopsy. Finding a potentially steroid responsive disorder in an otherwise steroid unresponsive disorder has important clinical implications.
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PMID:Bronchiolitis obliterans organizing pneumonia and scleroderma. 151 74


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