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

150 cases of chronic inflammatory lung diseases of unknown aetiology and assumed hyperergic (immuno-reactive) pathogenesis were examined for hypertensive pulmonary arterial lesions and for chronic cor pulmonale. Hypertensive lesions of the small pulmonary arteries were found in more than half of the cases with chronic disorders of long duration, but were inconspicuous in diseases of acute progressive character. Hypertensive lesions were found regularly in chronic interstitial pneumonia, frequently in scleroderma and rheumatoid arthritis and occasionally in dermatomyositis and disseminated lupus erythematosus. Chronic Cor pulmonale occurred in 16% of the cases with hypertensive arterial lesions of grade I (hypertrophy of media) and in 50% of grade II/III (hypertrophy of media and intimal fibrosis). Interstitial lung fibrosis plays an important role in the pathogenesis of cor pulmonale: two thirds of the cases with interstitial lung fibrosis had developed cor pulmonale and all the cases with cor pulmonale also had interstitial lung fibrosis. Hypertensive arterial lesions of grade IV-VI according to Heath and Edwards (angiitis, plexogenic and angiomatoid lesions) have been described in severe cases of pulmonary hypertension (congenital cardiac shunts, primary pulmonary hypertension). In secondary forms of pulmonary hypertension, as represented by our material, these changes are of little importance.
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PMID:[Hypertensive lesions of pulmonary arteries in chronic inflammatory lung diseases (author's transl)]. 15 72

A young woman with systemic lupus erythematosus (SLE) had clinical evidence of acute cor pulmonale. Autopsy disclosed vascular lesions in the lungs resembling those seen in advanced pulmonary hypertension. This case illustrates that severe pulmonary vascular disease may complicate SLE and mimic pulmonary thromboembolic disease.
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PMID:Severe pulmonary vascular disease in systemic lupus erythematosus. 47 99

A case of systemic lupus erythemaosus with an unusual complication of pulmonary hypertension leading to cor pulmonale is reported. Lung biopsy showed an interstitial pneumonitis with pulmonary vascular narrowing causing pulmonary hypertension. These changes appeared to be immunologically mediated by the utilization of IgG and C3. Microtubular virus-like particles were present in the endothelial cell cytoplasm of both lung and kidney.
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PMID:Lupus cor pulmonale with electron microscope and immunofluroescent antibody studies. 124 Dec 63

Patients with mixed connective tissue disease (MCTD) exhibit clinical features of systemic lupus erythematosus (SLE), progressive systemic sclerosis or scleroderma (PSS), and polymyositis-dermatomyositis (PM-DM). In their sera is an unusually high titer of a circulating antinuclear antibody with specificity for a nuclear ribonucleoprotein antigen. Pleuropulmonary manifestations are common in MCTD and the incidence varies from 20% to 85%. The pleuropulmonary complications include pleural effusion, interstitial pulmonary processes, pulmonary arterial hypertension (PAH), pulmonary vasculitis, pulmonary thromboembolic phenomena, aspiration pneumonia, and hypoventilatory failure. Pulmonary vascular pathology with progressive PAH and cor pulmonale is the most serious complication of MCTD. The pleuropulmonary manifestations in MCTD are similar to the respiratory problems well recorded in SLE, PSS, and PM-DM. Even though the pleuropulmonary complications are common in MCTD, they may remain clinically inapparent until fatal complications ensue.
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PMID:Lungs in mixed connective tissue disease. 157 26

Although systemic lupus erythematosus (SLE) no longer has the very poor prognosis that it had 50 years ago, there remains a significant mortality. We were able to determine the causes of death in 27 of the 29 patients with SLE who died over the period 1985 to 1989. This represents one and five year mortality of six and 24% respectively. In common with other studies, sepsis was a major factor, being implicated in the deaths of 37% of our patients. However, sepsis (i) occurred almost exclusively in patients with active SLE; (ii) often occurred after prolonged hospitalisation; and (iii) was a terminal event in otherwise fatal SLE in several patients. Overall, active disease was determined to be a cause of death in 67% of the patients. An unexpected observation was the finding that active cardiopulmonary disease accounted for 37% of deaths. Although late mortality from degenerative vascular disease is being increasingly reported with the modern prolonged SLE survivorship, it was identified in only one patient. We conclude that active disease remains the most important factor in mortality in SLE.
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PMID:Mortality in systemic lupus erythematosus: active disease is the most important factor. 158 Aug 64

(SLE) One hundred patients suffering from systemic lupus erythematosus (SLE) were examined by clinical, non invasive cardiological, radiological and laboratory methods. Valve diseases were revealed by M-mode echocardiographic examination in 17 patients. 14 patients had various conduction disturbances. Out of the remaining 83 patients not having any valve disease, systolic dysfunction of the myocardium was detectable in 17 persons and pericardial effusion in 3 persons. Spirometric alterations have been found in 64 persons and cor pulmonale has been diagnosed in 8 persons.
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PMID:[Cardiac and cardiopulmonary changes in systemic lupus erythematosus]. 264 7

The patient, a 30 year-old Caucasian female with a 6-year history of systemic lupus erythematosus was suspected of having pulmonary hypertension following chest X-Ray and routine echocardiography. Whilst awaiting further cardiological investigations she developed acute respiratory distress accompanied by gross signs of cor pulmonale and died despite full intropic and ventilatory support, in addition to intravenous "Pulse" methyl prednisolone. Postmortem findings showed typical features of the adult respiratory distress syndrome ('ARDS') but minimal vascular changes attributable to the pulmonary hypertension. The 'ARDS' was presumably associated with an acute 'flare' of the lupus.
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PMID:Systemic lupus erythematosus, pulmonary hypertension and adult respiratory distress syndrome (ARDS). 318 May 53

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.
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PMID:Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature. 360 12

We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor.
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PMID:Pulmonary arterial hypertension as a manifestation of lupus erythematosus. 365 44

The occurrence of cardiac manifestations and their relationship with the lupus anticoagulant (LA) in SLE was studied in 74 patients who were followed up for 22 years (median), of which 16 years were after the initial LA testing. Pericarditis was the most common cardiac event occurring in 16 (22%) patients but it did not correlate with LA. Valvular heart disease, coronary artery disease, left ventricular failure and/or cor pulmonale were observed in 16 (22%) patients. Taken together, their occurrence was associated with a history of leg ulcers (odds 3.8, P = 0.028) but not with LA or other common clinical manifestations of the antiphospholipid syndrome. Valvular heart disease in five patients was significantly associated with LA (P = 0.05). Cor pulmonale due to chronic pulmonary embolism was present in two patients with LA. Myocardial infarctions in five patients occurred late in the course of disease but in relatively young patients (mean 43 years). Fatal myocardial infarction in the absence of atherosclerosis in two LA-positive patients supports a pathogenetic role for LA in these cases. In conclusion, of the various cardiac complications in SLE, valvular heart disease and cor pulmonale appear to be connected with the antiphospholipid syndrome. Both conditions should be actively sought in patients with LA to decrease possible adverse events (arterial emboli and right ventricular failure) affecting the patients' prognosis.
Lupus 1994 Jun
PMID:Lupus anticoagulant and cardiac manifestations in systemic lupus erythematosus. 795 2


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