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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifteen children with scleroderma have been presented. All had characteristic cutaneous abnormalities at onset and during the course of disease. All were girls. All had visceral involvement, primarily of the gastrointestinal tract, heart, and lungs. The presence of visceral disease might have been missed without specific and extensive diagnostic procedures, including gastrointestinal barium studies, esophageal motility, pulmonary function and carbon monoxide diffusing capacity, and plethysmography. Raynaud's phenomenon was frequent and accompanied by evidence of occlusive vascular disease. Contractures around joints were readily evident and arthralgias were common, but evidence of objective arthritis was absent. Sixty percent of the patients in this series had ANA. Overlap syndromes with myositis and SLE were present in 7 patients. Three of 15 children died 6-10 years after onset of disease.
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PMID:Scleroderma in children. 26 12

Pulmonary function was studied in 22 patients with systemic lupus erythematosus without pulmonary clinical symptoms. The most striking features were: a) a restrictive functional pattern with hyperinflation, characterized by a decreased vital capacity and increased residual volume; b) alteration of the elastic properties of the lung, with increased pulmonary elastance; c) impairment of the alveolar-capillary gas transfer capacity, with very significant changes of the CO diffusion and arterio-alveolar gradients for O2 and CO2. No marked differences were found in functional disturbance among patients in the active or inactive phase of the disease.
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PMID:Pulmonary function in systemic lupus erythematosus patients without respiratory symptoms. 49 93

Lung involvement was assessed in 30 consecutive patients with systemic lupus erythematosus (SLE), not selected by respiratory symptoms. Pulmonary function tests revealed a higher rate of abnormality than either clinical history or radiography. The single breath carbon monoxide diffusing capacity was below 80 per cent of the predicted value in 24 patients (80 per cent), and a reduced total lung capacity was present in 13 (43 per cent). There was a weak correlation between the severity of the functional defect and disease activity, assessed antinuclear factor and DNA binding. No correlation was found with serum complement of Clq precipitation. Since pulmonary fibrosis in SLE is uncommon it cannot account for the high frequency of abnormal findings, and the pathogenesis of the functional changes is probably multifactorial. In seven of the patients with the smallest lung volumes, measurements of static pressure volume curves and of maximum respiratory pressures indicated extrapulmonary volume restriction. In five of these patients, diaphragm function was specifically assessed and found to be grossly abnormal in four. The inability of the diaphragm to generate normal pressure may be due to either severe weakness or immobility following extensive pleural adhesions. The well recognized syndrome of "shrinking lungs" and high "sluggish" diaphragms with clear lung fields on radiography is probably due to dysfunction of the diaphragm rather than to primary intrapulmonary pathology.
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PMID:Diaphragm function and lung involvement in systemic lupus erythematosus. 60 14

To determine the prevalence of pulmonary dysfunction in lupus erythematosus, 24 patients with systemic lupus erythematosus (SLE) and 5 patients with discoid lupus erythematosus (DLE) were studied. Diffusing capacity for carbon monoxide was abnormal in 17 (71 percent) SLE patients. A restrictive ventilatory defect was present in 6 (25 percent) and arterial hypoxemia in 4 of 23 (17 percent). The mean ratio of forced expiratory volume in one second to forced vital capacity (FVC) was 83 percent. To test for the presence of small airways disease, maximum expiratory flow rate at 50 percent of FVC was measured on air and on an 80 percent helium-20 percent oxygen mixture. Ten patients (5 smokers and 5 nonsmokers) with SLE were nonresponders to helium suggesting small airways disease. Pulmonary dysfunction was present in 90 percent (9/10) of SLE patients with a previous history of pleuritis and/or pneumonitis, and in 71 percent (10/14) without respiratory symptoms or history of lung disease and with a normal chest radiograph. Pulmonary function tests were normal in DLE patients except for an abnormal response to helium and/or mild arterial hypoxemia in two patients, all of whom were smokers. These data indicate that there is a high prevalence of pulmonary function abnormalities in SLE including patients without clinically evident pleuropulmonary disease.
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PMID:Systemic and discoid lupus erythematosus: analysis of pulmonary function. 68 97

Several series have suggested that pulmonary function abnormalities are common in systemic lupus erythematosus. However, only isolated studies have attempted to relate these abnormalities to immunological aspects of the diseases. In the present study respiratory symptoms, pulmonary function tests, and immunological data were reviewed in 22 patients with systemic lupus erythematosus. Seventeen subjects had either clinical evidence or abnormalities of lung function suggestive of pulmonary involvement. A restrictive ventilatory defect or reduction in pulmonary diffusing capacity for carbon monoxide was demonstrated in 14 of the patients only 4 of whom were dyspnoeic. There was no correlation between pulmonary involvement, co-existent renal lupus, and immunological abnormality.
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PMID:Pulmonary involvement in systemic lupus erythematosus. 74 99

1.1. Diffusing capacity of the lungs was measured by using a Mark 4 Resparameter. The single-breath diffusing capacity (D) for carbon monoxide was found to be dependent on the lung volume (VA) during the breath-hold. The same applied to D/VA and to the time constant (tau) for carbon monoxide uptake. This confirms the theoretical considerations and the results of other investigators. This effect should be taken into account especially when making serial determinations of D on the same subject. 1.2. The repeatability of the method was found to be reasonably good; the coefficient of variation was about 4% in normal subjects, as well as in a series of patients with rheumatoid arthritis. 1.3. The effect of the correction for haemoglobin concentration was calculated and tabulated for a set of possible values of DM/Vc and haemoglobin. This correction did not reduce the scatter of D values in normal subjects, but it was adopted for clinical use for theoretical reasons. Caution in its use and interpretation of the correction is emphasized. 2.1. Healthy males and females, 20-69 years of age, were examined to establish reference values for the D measurement. Multiple linear regression equations were calculated stepwise, and equations based on age and height were then used in the clinical prediction equations. Similarly, prediction equations were calculated for clinical spirometry. 2.2. The prediction equations were compared with some published reference values. The equations provide a basis for the evaluation of the effect of various factors, such as age and smoking, in different populations. 3.1. Respiratory function, clinical symptoms and chest x-ray findings were examined in patients with connective tissue diseases. Three different investigations were thus formed: a) consecutive patients (free of lung disease other than that possibly due to a connective tissue disease) with definite rheumatoid arthritis (RA, 21 cases), systemic lupus erythematosus (SLE, 18 cases) or scleroderma (SCL, 6 cases) were subjected to detailed tests. b) 129 patients from the Rheumatism Foundation Hospital, Heinola, Finland, were subjected to measurement of diffusing capacity and vital capacity in addition to chest radiography and routine clinical assessment c) the histological findings on 12 patients subjected to a needle biopsy of the lung in order to exclude other conditions were compared with the results of diffusing capacity and x-ray examinations. 3.2. Restrictive impairment of the respiratory function was the general finding in all of these groups. The reduction in diffusing capacity was out of proportion to the reduction in lung volume, however, in most cases with abnormally low values. Low D values were encountered in about half of the RA and SLE and in all of the SCL patients in group a, and in 13% of the "average" RA patients studied in Heinola. The typical histological finding in patients with reduced D was a thickening of the alveolar wall...
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PMID:Single-breath pulmonary diffusing capacity. Reference values and application in connective tissue diseases and in various lung diseases. 107 26

Lung function was prospectively studied in a group of non-selected systemic lupus erythematosus (SLE) female patients who consecutively came to our Lung Function Laboratory. There was no previous history of smoking in any patient nor there was clinical and/or radiographic evidence of lung affectation prior to the diagnosis of SLE. Some respiratory lung function abnormalities were observed in 17 patients (65%). The most frequent functional anomaly was the alteration in carbon monoxide transfer (DLCO), which was present in 10 cases (38%). On the other hand, a restrictive ventilatory failure was observed in 8 patients (31%) and a obstructive ventilatory failure in 6 other (23%). Out of these, 3 patients (12% of the total number of cases) presented bronchial hyperreactivity, which could also be confirmed in a patient presenting a mixed ventilatory failure. Isolated air entrapment could be observed in 2 patients, which could indicate disfunction of small airways in these cases. There was no correlation between functional respiratory failure and clinical activity of SLE, duration of the disease, other organ involvement, treatment and/or serum antibodies. Given that a great number of these functional lung anomalies (42%) exist in the absence of any clinical and radiological anomalies and that an important subgroup of patients are responsive to bronchodilators, we consider that the systemic evaluation of lung function must be obliged in the clinical treatment of SLE patients.
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PMID:[An elevated prevalence of subclinical pulmonary involvement in systemic lupus erythematosus]. 180 Oct 67

The clinical course of chronic diffuse interstitial lung disease (ILD) was studied in 14 patients with SLE. The mean duration of follow-up was 7.3 years. All patients had dyspnea on exertion, pleuritic chest pain, chronic cough, and basilar rales. Chest roentgenogram showed diffuse or basilar infiltrates, pleural disease, and elevation of both diaphragms. Systemic corticosteroids were given early in the course of the illness for lung involvement and multisystem disease. Diffusing capacity for carbon monoxide (DLCO) and inspiratory vital capacity (IVC) improved or remained unchanged in the majority of patients. Respiratory complaints improved in all patients; however, two patients died of pulmonary fibrosis and another died of bacterial pneumonia. Alveolar septal deposits of immunoglobulins and complement were found. This study showed that while variability existed among individual subjects, the clinical progression of ILD was slow and tended to improve or stabilize with time.
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PMID:A long-term study of interstitial lung disease in systemic lupus erythematosus. 221 53

A number of lines of evidence suggest that the lupus-like symptoms associated with procainamide therapy may be caused by products of metabolic N-oxidation. In the present study, the perfusion of the isolated rat liver with a hemoglobin-free solution containing procainamide (100 microM) resulted in the rapid appearance of the N-oxidation metabolite procainamide hydroxylamine in the perfusate. Addition of procainamide hydroxylamine in vitro to whole rat blood (1-40 microM) resulted in a concentration-dependent loss of proliferative response among mononuclear cells isolated from the treated blood and cultured with mitogens (phytohemagglutinin, PHA-P: concanavalin A, Con A; and pokeweed mitogen, PWM), as well as a loss of viability. Similar effects on lymphocyte mitogen responsiveness were observed when procainamide hydroxylamine (1-40 microM) was added to rat whole splenic cell populations. Carbon monoxide or ascorbic acid pretreatment inhibited the toxicity of procainamide hydroxylamine to lymphocytes in whole blood, but only carbon monoxide pretreatment inhibited procainamide hydroxylamine-induced methemoglobin formation. These observations are consistent with the participation of hemoglobin in a redox cycle with procainamide hydroxylamine, generating products which are primarily responsible for its cytotoxicity in blood.
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PMID:Procainamide hydroxylamine lymphocyte toxicity--I. Evidence for participation by hemoglobin. 247 7

Twelve consecutive patients with systemic lupus erythematosus (SLE) and chest symptoms of at least 3 months' duration were investigated with spirometry, lung mechanics at rest and exercise, diffusion capacity and right heart catheterization. Vital capacity (88% of predicted, p less than 0.05), and FEV1 (84%, p less than 0.01) were decreased in the study group, but spirometric and diffusion capacity abnormalities were moderate compared with previous studies. The single breath CO2 test showed, in six patients, ventilation-perfusion mismatch with patterns typical for either bronchial obstruction or vascular disease. Non-respiratory factors were responsible for reduction of working capacity (on average 68% of predicted normal values (p less than 0.001]. Two patients with pulmonary hypertension were identified by right heart catheterization. One of them had overlap features with the CREST syndrome. Both these patients had abnormal SBT-CO2 test and diffusion capacity, along with diffuse perfusion defects on perfusion scintigraphy. The low frequency of pulmonary function abnormalities in this study suggests that irreversible pulmonary damage is uncommon in SLE.
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PMID:Lung function in patients with systemic lupus erythematosus and persistent chest symptoms. 251 98


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