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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We sought to explore immunological factors in patients who died with rapidly fatal fibrosing lung diseases (Hamman-Rich syndrome). A retrospective review of cases of interstitial lung disease showed 12 recent deaths from Hamman-Rich syndrome. The mean age was 62, men outnumbering women 3 : 1. Five patients had proved collagen vascular disease (rheumatoid arthritis three, lupus two). Four others had a history of allergic disorders, syphilis, chronic eosinophilia, or hypersensitivity reactions. One patient showed disappearance of immunofluorescence as fibrosis advanced, which has not previously been reported. The study suggests a possible aetiological link between disorders of immunity and Hamman-Rich syndrome. The evidence also supports the notion that Hamman-Rich syndrome is an accelerated variant of the more indolent interstitial pneumonias.
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PMID:Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis. 16 92

Ultrastructural study of pulmonary biopsy specimens from patients with fibrotic lung disease disclosed the presence of nuclear inclusions in 1% or less of cuboidal alveolar epithelial cells in 9 of 19 patients, including 6 of 12 patients with idiopathic pulmonary fibrosis, 2 of 3 patients with collagen-vascular disease, and 1 of 3 patients with sarcoidosis. Nuclear inclusions were not observed by ultrastructural study in 5 control patients. The inclusions consisted of masses or aggregates of tubules which probably were derived from the inner nuclear membranes. These tubules were smooth-walled, showed branchings and bifurcations, were composed of single trilaminar membranes, usually had a clear content, and ranged from 500 to 1000 A in diameter. They resembled nuclear tubules which occur in other cell types under conditions of rapid growth or specific hormonal stimulation. Statistically significant differences between the groups of patients with and without nuclear inclusions in cuboidal alveolar epithelial cells were not found with respect to smoking history, degree of fibrosis in the lung biopsy specimen, or the degree of pulmonary physiologic impairment. However, the average age of the patients having nuclear inclusions was significantly greater than that of patients not having nuclear inclusions. In addition, the frequency of indentations in the nuclei of cuboidal alveolar epithelial cells was greater in patients with nuclear inclusions than in patients without nuclear inclusions. Highly significant correlations were observed between the presence of nuclear inclusions and the presence of a) anchoring fibrils and hemidesmosomes along the basal surfaces of alveolar epithelial cells and b) multilayering of the alveolar epithelium.
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PMID:Nuclear inclusions in alveolar epithelium of patients with fibrotic lung disorders. 42 30

Cellular and immunoglobulin components of bronchoalveolar fluid recovered by bronchoscopic lavage were evaluated in 32 control patients, 10 normal volunteers, and 60 patients with the following interstitial lung diseases: idiopathic pulmonary fibrosis, pulmonary fibrosis associated with collagen-vascular disease, eosinophilic granuloma, sarcoidosis, and hypersensitivity pneumonitis. The percentage of lymphocytes distinguished two general disease categories: those with increased lymphocytes (sarcoidosis and hypersensitivity pneumonitis); and those with normal lymphocytes (idiopathic pulmonary fibrosis, pulmonary fibrosis associated with collagen-vascular disease, and eosinophilic granuloma). Patients in all five disease categories had elevated IgG levels and percentages of neutrophils compared with control patients, with the highest proportion of neutrophils found in idiopathic pulmonary fibrosis. Immunoglobulin levels also helped distinguish among patient groups, in that patients with hypersensitivity pneumonitis had lavage IgG/albumin ratios greater than 1, whereas patients with sarcoidosis had ratios less than 1; and with infrequent exceptions, the finding of IgM in lavage fluid was limited to patients with hypersensitivity pneumonitis.
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PMID:Bronchoalveolar lavage in interstitial lung disease. 69 24

Bronchopulmonary lavage (BAL) has provided a fresh dimension for the investigation of pulmonary and multisystem disorders. BAL fluid may be analysed for cells and chemical mediators in the diagnosis and also serially for the management of several granulomatous disorders including sarcoidosis, extrinsic allergic alveolitis, chronic beryllium disease, talc granulomatosis, tuberculosis, Langerhans' histiocytosis-x and Crohn's disease. It may also provide information in fibrosing alveolitis, collagen vascular disease, occupational and drug-induced lung disease, acquired immune deficiency syndrome, bronchial asthma, neoplasia, transplantation, pulmonary alveolar proteinosis and eosinophilic lung disease. This survey analyses the value of BAL and how it has provided a new window for the chest physician.
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PMID:Bronchopulmonary lavage (BAL). A window of the lungs. 134 40

Since July 1988, a total of 92 transbronchial biopsies (TBB) have been performed in 18 patients (aged 3-16 years). Twelve patients (67%) were heart-lung transplant (HLT) recipients undergoing surveillance for pulmonary graft rejection and infection. The remainder included immunocompromised patients at risk of opportunistic infections (n = 4), patients with fibrosing alveolitis (n = 1) and a collagen vascular disorder with suspected lung involvement (n = 1). TBB was performed through either a fiberoptic (n = 50) or a rigid (n = 41) bronchoscope, all under general anesthesia. On one occasion a cardiac bioptome was used through an endotracheal stent. The sensitivity of TBB for diagnosing acute and chronic rejection in HLT patients was 88% and 60%, respectively (specificity, 91% and 100%). Definitive diagnoses were made in 4 (67%) of the non-HLT group. Bronchoalveolar lavage (BAL) was performed during each procedure for microbiological and cytological examination. Thirty-four pathogenic organisms including Pseudomonas aeruginosa (16/34), Staphylococcus aureus (8/34), and Candida albicans (5/34) were isolated from BAL culture. Complications included pneumothorax (8%), transient pyrexia (7%), and dyspnea (2%).
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PMID:Technique and use of transbronchial biopsy in children and adolescents. 161 50

Fractional analysis of bronchoalveolar lavage (FABAL) fluid was performed in 6 control patients and 41 patients with various interstitial lung disease. The cell differential counts in the first 30 ml fraction of BAL (FBAL-I), which is considered to be the bronchial lavage, differed from those of the 50 ml second and third fraction (FBAL-III). Hypersensitivity pneumonitis, pulmonary tuberculosis, and sarcoidosis showed a high recovery of lymphocytes (52%); however, the former two disorders were occasionally, associated with neutrophil airway inflammation, whereas sarcoidosis was not. The percentage recovery of neutrophils in total FBAL was considerably high in patients with diffuse panbronchiolitis, and relatively high in those with collagen vascular disease, idiopathic pulmonary fibrosis, pneumoconiosis, and control smokers. However, these neutrophils were largely recovered from FBAL-I, suggesting the presence of airway inflammation. Thus, it is valuable to apply the FBAL method to determine the topographic distribution of inflammatory cells in the lungs. It was also found that the lymphocyte morphology in the lavage fluid was of value in establishing the diagnosis of hypersensitivity pneumonitis, and it is critical whether or not mast cells and basophils are present in BALF since they indicate the pathologic state of allergy or fibrosis. Although present in various fibrotic lung diseases in a limit number, langerhans cells are a diagnostic marker for histiocytosis X.
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PMID:[Airway and alveolar inflammation assessments with bronchoalveolar lavage in various interstitial lung disorders]. 163 46

To investigate the physiologic impairment and the role played by pulmonary function tests in interstitial lung disease (ILD), we performed spirometry tests and tested the single-breath carbon monoxide diffusing capacity of 27 patients with diffuse interstitial pulmonary fibrosis (DIPF) and 35 patients with collagen vascular disease (CVD). All of the patients showed irregular linear opacities on their chest X-ray films. Corresponding chest roentgenograms for each patient were evaluated according to the International Labour Organization (ILO) classification. A modified Baseline Dyspnea Index (BDI), which incorporate a patient's physical activity into the recording, was used to quantitate dyspnea. Patients with DIPF demonstrated a comparable reduction in both lung volumes and diffusing capacity. In contrast, patients with CVD had a greater reduction in diffusing capacity than in forced vital capacity (FVC) and total lung capacity (TLC). There were no significant correlations between the type of linear opacities and the severity of the pulmonary function abnormalities, or between the opacities and the dyspnea in either disease group. The profusion of pulmonary infiltrates also did not affect the lung function except for diffusing capacity in patients with DIPF. However, there was a significant loss of FVC, TLC, forced expiratory volume in one second (FEV1) and diffusing capacity with increasing levels of dyspnea. We conclude that pulmonary function parameters may not be equally affected in DIPF and CVD. Approaches using the ILO classification for analysis of chest roentgenograms provide limited information regarding the functional status of patients. Pulmonary functions are significantly related to the extent of a patient's physical activity, if the severity of dyspnea is evaluated carefully using a quantifiable system.
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PMID:Lung volume, diffusing capacity, chest roentgenogram and dyspnea index in interstitial lung disease. 167 99

The interstitial lung diseases are comprised of a group of pulmonary disorders characterized clinically by diffuse infiltrates on the chest radiograph and histologically by distortion of the gas exchanging portion of the lung. The physiologic correlates are restriction of lung volumes and impaired oxygenation. The term "interstitial" when applied to these diseases is actually a misnomer because it implies that the inflammatory process is limited specifically to the area between the alveolar epithelial and capillary endothelial basement membranes. The diseases currently grouped as "interstitial" also frequently involve the alveolar epithelium, alveolar space, pulmonary microvasculature, and less commonly, the respiratory bronchioles, larger airways, and even the pleura. The enormous differential diagnosis of interstitial lung disease can be made manageable by understanding that pneumoconiosis, drug-induced disease, and hypersensitivity pneumonitis account for over 80% of the responsible entities and can usually be identified from the patient's history. The nine remaining diseases/disease categories include: sarcoidosis, idiopathic pulmonary fibrosis, bronchiolitis obliterans-organizing pneumonia, histiocytosis X, chronic eosinophilic pneumonia, collagen vascular disease-associated interstitial lung disease, granulomatous vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome, lymphomatoid granulomatosis), Goodpasture's syndrome, and pulmonary alveolar proteinosis. The diagnosis of a specific interstitial lung disease can be made via various means including the patient's history, specific serologies, bronchoalveolar lavage, transbronchial biopsy, and biopsy of extrathoracic tissues or open lung biopsy. A directed diagnostic approach can be formulated based on an understanding of these techniques and a thorough knowledge of the clinical presentations and specific diagnostic criteria for each of the major diseases. This monograph will serve as a guide for the clinician to use in evaluating and treating patients with interstitial lung disease. We begin by reviewing the clinical presentation, diagnostic criteria, and management of specific interstitial lung diseases excluding pulmonary infection, neoplasm, and sarcoidosis. Pneumoconiosis and drug-induced syndromes are not discussed in detail, but the agents responsible and pertinent exposures are presented in tabular form in the discussion of the general diagnostic approach.
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PMID:Interstitial lung disease. 199 45

Excessive release of reactive oxygen metabolites (ROM) from lung inflammatory cells has been claimed to be of major pathogenetic significance in diffuse fibrosing alveolitis. In the present study, the content of oxidized methionine residues [Met(O)] as a percentage of total methionine (Met) in BAL-derived proteins was used to assess the biologic effect of ROM. In addition, procollagen-III-peptide was measured in BAL fluid as a marker of fibroblast activation. We investigated bronchoalveolar lavage (BAL) samples from seven control patients without evidence of interstitial lung disease and from 42 patients with fibrosing alveolitis caused by idiopathic pulmonary fibrosis (IPF), n = 20, or by collagen vascular disease (CVD), n = 22. Met(O) was elevated in the patients with IPF or CVD compared with that in the control subjects (8.86 +/- 1.26 and 8.13 +/- 1.44% versus 3.36 +/- 0.49%, p less than 0.01 and p less than 0.05, respectively; mean +/- SEM). A positive correlation was found between percentage of neutrophils in BAL and Met(O) in both groups separately and combined (IPF, r = 0.84; p less than 0.001; CVD, r = 0.44; p less than 0.05; IPF and CVD, r = 0.60; p less than 0.001), whereas an inverse relationship existed between Met(O) and the percentage of alveolar macrophages in BAL (IPF, r = -0.59; p less than 0.01; CVD, r = -0.24; NS; IPF and CVD, r = -0.41; p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pathogenetic significance of reactive oxygen species in diffuse fibrosing alveolitis. 206 21

Oxidant production by peripheral granulocytes stimulated with phorbol myristate acetate (PMA) was investigated in 11 patients with idiopathic pulmonary fibrosis (IPF), 6 with interstitial pneumonia associated with collagen vascular disease (IP-CVD), 8 with sarcoidosis and 12 healthy subjects. Oxidant production was examined by flow cytometry using dichlorofluorescein diacetate. The reactivity of granulocytes to PMA was assessed according to the ratio between mean fluorescent intensity of granulocytes stimulated maximally with PMA and that without PMA, (stimulation index: S.I.). The concentration of PMA that induced half maximal fluorescent intensity of granulocytes (PC 1/2 max) was used as the index of sensitivity. The S.I. was 7.2 +/- 0.45 in IPF, 6.3 +/- 0.6 in IP-CVD, 6.0 +/- 0.71 in sarcoidosis, and 5.8 +/- 0.2 in healthy subjects. However differences between groups were not significant. PC 1/2 max was 7.3 +/- 2.1 ng/ml in IPF, 9.1 +/- 3.0 ng/ml in IP-CVD, 12 +/- 6.9 ng/ml in sarcoidosis and 16.1 +/- 5.8 ng/ml in healthy subjects. There was significant difference between IPF and healthy subjects (p less than 0.05) indicating that peripheral granulocytes in patients with IPF are more highly sensitive to PMA than healthy subjects.
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PMID:[Increased oxidant production by peripheral blood granulocytes in patients with idiopathic pulmonary fibrosis]. 217 52


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