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)

Methotrexate is frequently used in the treatment of severe psoriasis, and its hepatotoxicity has long been recognized by dermatologists. Pulmonary complications resulting from use of the drug are uncommon but should be considered in any patient on methotrexate who develops pulmonary symptoms in the absence of infection. We describe two patients, one who developed an acute pneumonitis and one with progressive pulmonary fibrosis following long-term, low-dose methotrexate for psoriasis. Early recognition of these complications by lung function testing and withdrawal of the drug, when necessary, may arrest or reverse methotrexate-induced lung disease.
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PMID:Pulmonary complications following methotrexate therapy. 381 74

Various anomalies of pulmonary surfactant have been described in relation to acute respiratory distress syndromes, hypersensitivity lung disease and pulmonary sarcoidosis. Phosphatidylcholine (PC) is the essential phospholipid component of pulmonary surfactant. Cytidine diphosphocholine (CDP-choline) is an essential intermediary in the biosynthesis of PC. The authors studied two groups of patients: one group consisted of diffuse interstitial pulmonary fibrosis and the other consisted of pulmonary sarcoidosis with parenchymal involvement. They observed quantitative and qualitative abnormalities of the phospholipid fractions of surfactant and more particularly of PC. The finding of a marked decrease in this phospholipid, especially in the cases of pulmonary fibrosis, justified the study of the therapeutic effects of CDP-choline. After one month of treatment with this substance, at a dose of 1 g I.M. per day, the PC fraction had returned to normal and, at the same time, there was an improvement in the PaO2 at rest and after exercise. Long term administration of CDP-choline appears to be valuable in the maintenance of the phospholipid equilibrium of pulmonary surfactant and in the improvement of the quality of alveolar gas exchange.
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PMID:[Respiratory function and alveolar biological changes under the effect of CDP-choline in pulmonary interstitial pathology: pulmonary fibrosis and sarcoidosis]. 383 93

Analysis of bronchoalveolar lavage fluid (BAL) is an effective method of detecting an inflammatory response in the lungs of animals in toxicological studies. Alterations in BAL that are the most sensitive indications of an inflammatory response are an increased content of serum proteins and an influx of neutrophils (PMNs). Elevation of the cytoplasmic enzyme lactate dehydrogenase (LDH) is a useful indicator of cytotoxicity. The pulmonary inflammatory response to particles (either mineral dusts or soot) in the lung includes greatly increased activities of such lysosomal enzymes as beta-glucuronidase and beta-N-acetylglucosaminidase in BAL. Examination of alterations in BAL in rats and mice during chronic exposure to high levels of diluted diesel exhaust revealed that steadily increasing levels of LDH, beta-glucuronidase, and hydroxyproline in BAL correlated better with the development of pulmonary fibrosis than did measures of an inflammatory response (protein, PMNs). Analysis of BAL has proven useful, both for detection of lung injury in toxicological screening tests and for determination of the mechanisms of developing chronic lung disease. Future work shows promise of developing assays for BAL analysis to identify the specific site or type of pulmonary injury present.
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PMID:New approaches for the evaluation of pulmonary toxicity: bronchoalveolar lavage fluid analysis. 389 79

About 80 drugs are known to cause lung disease. Cytostatic agents are the most common cause of this type of iatrogenic disease. In addition to the "intercalating" type of cytostatic, other groups, especially the alkylating (busulfan) and the antimetabolites groups (methotrexate), have also been incriminated. Cytostatic drug-induced lung disease is a difficult diagnosis based on the results of clinical, radiological, respiratory function, histopathological and biological investigations. The results of bronchoalveolar lavage are vital, especially in lung disease due to a drug hypersensitivity reaction; the diagnosis of drug-induced pulmonary fibrosis due to a toxic mechanism is much more difficult and risky. Early diagnosis of these drug-induced lung diseases is obviously important.
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PMID:[Pulmonary changes caused by cytostatic drugs]. 391 27

To assess the role of changes in lung collagen in pulmonary fibrosis, the content of this protein was measured in biopsy and autopsy lung from patients with cryptogenic fibrosing alveolitis (CFA), a fibrotic lung disorder of unknown cause. The collagen concentration was measured in lung samples from 21 patients with CFA (14 autopsy and seven open-lung biopsy) and 17 normal subjects; total lung collagen was determined in the right lung of 10 patients who died from CFA and the results were compared with those from 10 normal lungs. There was a wide variation in the collagen concentrations but the mean value (+/- SEM) for patients with CFA (217 +/- 13 mg/g dry weight) was significantly higher (P less than 0.02) than that of the controls (155 +/- 15 mg/g dry weight). The mean collagen concentration of the autopsy samples (243 +/- 20 mg/g dry weight) was significantly higher (P less than 0.05) than that of the biopsy samples (165 +/- 24 mg/g dry weight). The mean total collagen was markedly raised (P less than 0.001) in right lungs of patients with CFA (32.5 +/- 4.3 g) compared with normal lungs (14.0 +/- 1.1 g). When corrected for the predicted lung volume this difference in total lung collagen remained statistically significant (P less than 0.01, mean for patients 4.7 +/- 0.7 mg/ml, controls 2.3 +/- 0.2 mg/ml). These results demonstrate an increased deposition of lung collagen in this form of pulmonary fibrosis. They also suggest that there is a greater collagen concentration in lungs of patients with later disease, indicating a progressive deposition of collagen during the course of the disease.
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PMID:Biochemical evidence for an increased and progressive deposition of collagen in lungs of patients with pulmonary fibrosis. 394 76

Alveolar inflammation is thought to underlie the development of pulmonary fibrosis in several forms of diffuse lung disease including the connective tissue diseases. The relationship between inflammation and the clinical manifestations of systemic sclerosis (scleroderma), such as skin and lung involvement, is less clear. We therefore evaluated 14 never-smoking patients with systemic sclerosis with pulmonary involvement by bronchoalveolar lavage (BAL) and compared the results with those found in eight nonsmoking patients with idiopathic pulmonary fibrosis (IPF) and eight normal subjects. The patients with scleroderma also underwent gallium citrate Ga 67 scanning. We found that patients with scleroderma and pulmonary involvement have alveolitis that appears to wane with time. In addition, patients with systemic sclerosis have a cellular profile in lavage fluid that appears to differ from that of patients with IPF. Finally, we found a significant correlation between BAL cellular recovery and the single-breath carbon monoxide diffusing capacity in patients with systemic sclerosis but not in patients with IPF. We conclude that inflammation may play an important role in the pathogenesis of the pulmonary disease of scleroderma and that different mechanisms may lead to fibrosis in IPF and scleroderma.
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PMID:Role of inflammation in the lung disease of systemic sclerosis: comparison with idiopathic pulmonary fibrosis. 395 Apr 67

A case of a rare entity, pulmonary tumorlets that metastasized to a peribronchial lymph node, is reported. The patient, a 38-year-old man, underwent a right pneumonectomy for end-stage lung disease caused by bronchiectasis, chronic bronchitis, and pulmonary fibrosis. No tumors were detected on radiologic or on gross examination of the lung. Microscopically, multiple tumorlets were identified in the fibrotic pulmonary parenchyma. Five peribronchial lymph nodes were found and appeared grossly normal. A microscopic focus of metastatic tumor, histologically identical to the pulmonary tumorlets, was discovered in one of these nodes. This metastasis was identified only because a diligent search for peribronchial lymph nodes was undertaken, and because sections of each node were obtained. Pathologists usually do not extensively examine peribronchial lymph nodes in cases of chronic inflammatory disease of the lung, even when small tumorlets are discovered as incidental findings, because it is presumed that the tumorlets have not metastasized. As shown by our case, this presumption is not always correct. Pulmonary tumorlets may metastasize to peribronchial lymph nodes more frequently than has been previously recognized.
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PMID:Carcinoid tumorlets of the lung with metastasis to a peribronchial lymph node. Report of a case and review of the literature. 398 42

To evaluate the P wave changes in the electrocardiogram (ECG) in chronic lung diseases, we examined ECGs from twenty patients with interstitial pulmonary fibrosis (the FLD group) and twenty patients with chronic obstructive lung disease (fifteen with pulmonary emphysema and five with bronchial asthma, the COLD group). In the COLD group, the amplitude of P waves in leads II and III increased and the axis of the P wave shifted rightward. In the FLD group, however, these changes were not observed. Furthermore, the axis of the P wave exceeded +70 degrees in all the patients in the COLD group, and was less than +70 degrees for all the patients in the FLD group. The increased P wave terminal force in lead V1 was found in nine patients (45%) of the FLD group and in ten patients (50%) in the COLD group. We concluded that the rightward shift of the axis of the P wave was a characteristic of obstructive lung disease and that it was mainly caused by the overinflation of the lung. This P wave change was not observed in interstitial pulmonary fibrosis.
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PMID:P-wave changes in obstructive and restrictive lung diseases. 403 26

The radiological appearances of diffuse pulmonary shadowing are not specific and frequently histological examination is necessary to provide a diagnosis or assess the activity of the disease. From July 1979 to May 1983 open lung biopsy was performed in 101 patients through a limited submammary incision. Twenty seven patients had undergone prior invasive investigations and 27 were taking corticosteroids at the time of biopsy. In 92 patients the histological appearances after open lung biopsy were sufficiently specific to permit diagnosis. Respiratory tract infection occurred in six patients and in eight there was some superficial infection of the wound. These complications were more frequent in patients taking corticosteroids but the difference was not statistically significant. The chest drain was removed usually on the first postoperative day. In three critically ill patients an air leak occurred after removal of the drain, requiring formal surgical re-exploration and drainage in two cases and a brief period of intercostal drainage in the third. Four patients in the series died. All had severe pre-existing lung disease; in three open lung biopsy was performed in the late stages of severe, rapidly progressive lung disease eluding diagnosis; the fourth patient, who had massive pulmonary fibrosis from asbestos lung disease, developed a respiratory tract infection and died from progressive respiratory failure. Open lung biopsy can be performed with minimal morbidity and a high diagnostic yield. The approach used in this series provides a safe and reliable operation with good cosmetic results.
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PMID:Open lung biopsy in patients with diffuse pulmonary shadowing. 409 75

In 1964, 219,085 persons were examined during a tuberculosis survey in Vancouver, B.C. One hundred and fifteen new cases of tuberculosis and 929 cases of significant non-tuberculous lung disease were found. In a four-month follow-up of the non-tuberculous cases it was found that of the 742 patients who had named a physician when examined 26.6% had not made contact with him. Of those who did contact the physician, the follow-up was considered "poor" in only 30 patients (2.1%). Seventeen patients had died in the four-month interval and 81 who could not be located after the initial survey were considered "lost". Rates were determined for 37 diagnoses per 1000 patients screened. The most common diagnosis was localized pulmonary fibrosis (1.69/1000). Carcinoma was found in 0.30/1000 and solitary lung density in 0.17/1000 population screened.
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PMID:Non-tuberculous chest disease found in a mass x-ray survey in Vancouver, B.C. 591 Nov 54


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