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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

Two cases of hypertensive pulmonary vascular disease with Raynaud's phenomenon are reported. One is a 32-year-old housewife of primary pulmonary hypertension with some immunological disorders and the other is a 28-year-old female of systemic lupus erythematosus. It may be considered that some immunological process could play a role in the etiology of some cases of primary pulmonary hypertension and the relationship of pathogenesis between these 2 cases was discussed.
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PMID:Case report. Two cases of pulmonary hypertension with Raynaud's phenomenon. Primary pulmonary hypertension and systemic lupus erythematosus. 116 Jan 86

The pulmonary vascular changes in systemic lupus erythematosus (SLE) have been investigated from 20 autopsies performed at the Mount Sinai Hospiral from 1964 to 1973. Acute lesions included fibrinoid necrosis and vasculitis. Chronic lesions consisted of intimal fibrosis, medial hypertrophy, alteration of elastic laminae, periadventitial fibrosis, and, in one case, aneruysmal dilatation. These changes were found variously in arterioles, arteries and veins. The fibrotic and occlusive vascular lesions may account for the syndrome of "unexplained breathlessness" that occurs in SLE. These lesions may progress in certain individuals to overt pulmonary hypertension; the concept of coexisting primary pulmonary hypertension and SLE should be re-examined.
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PMID:Pulmonary vascular disease in systemic lupus erythematosus. 125 21

A 24-year-old West Indian woman with a four-year history of systemic lupus erythematosus presented with progressive dypsnoea due to primary pulmonary hypertension. Despite showing a response to vasodilators, these failed to control the condition. Her pulmonary hypertension increased in severity, eventually resulting in her death. We believe primary pulmonary hypertension to be an unusual complication of systemic lupus erythematosus. We suggest that this diagnosis should be considered in all patients with systemic lupus erythematosus and progressive dypsnoea, as optimum benefit can only be obtained by early institution of vasodilator therapy.
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PMID:Primary pulmonary hypertension associated with systemic lupus erythematosus. 179 Jun 44

Recently pulmonary hypertension (PH) has been recognized as one of life threatening complications which determine the prognosis of patients with systemic lupus erythematosus (SLE). Clinical aspects and pathology in patients with SLE complicated by PH (SLE-PH) have been reported to have a close similarity to those in patients with primary pulmonary hypertension (PPH), the prognosis of which is very poor in general. However, the long-term prognosis for patients with SLE-PH is not clear yet. Mainly because of some technical limitations for determining the severity of the PH, long-term follow-up studies have been inconclusive. In this study, 7 patients with SLE-PH and 6 patients with PPH were studied by using two-dimensional echocardiography. The interventricular septal curvature (R) was measured from the parasternal short axis view and the reciprocal value (1/R) was applied as a severity index of the PH. In 4 patients with SLE-PH and 6 with PPH, pulmonary arterial pressure was measured by cardiac catheterization concomitantly. There were no significant differences in 1/R and pulmonary arterial pressure between the two groups when PH was diagnosed for the first time. In the PPH group, the mean period of follow-up study was 25.3 months and all of the patients died during that period. In SLE-PH group, the mean period of follow-up study was 50.6 months, and only 3 patients (43%) died during that period. No patients in the PPH group showed improvement in 1/R, but 3 patients with SLE-PH improved in 1/R during a long follow-up period (60, 96 and 103 months respectively). Thus, the prognosis for SLE-PH was better than that for PPH, and there were some differences in the course and worsening behavior of PH in SLE-PH compared with that in PPH during the long-term follow-up study with reference to the echocardiographic investigation using 1/R value.
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PMID:[Clinical course and prognosis in SLE patients with pulmonary hypertension: a comparative study with primary pulmonary hypertension]. 192 4

Human plasma contains an inhibitor of tissue factor-initiated coagulation known as the lipoprotein-associated coagulation inhibitor (LACI) or also known as the extrinsic pathway inhibitor (EPI). A competitive fluorescent immunoassay was developed to measure the plasma concentration of LACI in samples from normal individuals and patients with a variety of diseases. The LACI concentration in an adult control population varied from 60% to 160% of the mean with a mean value corresponding to 89 ng/mL or 2.25 nmol/L. Plasma LACI levels were not decreased in patients with severe chronic hepatic failure, warfarin therapy, primary pulmonary hypertension, thrombosis, or the lupus anticoagulant. Plasma LACI antigen was decreased in some, but not all patients with gram-negative bacteremia and evidence for disseminated intravascular coagulation. Plasma LACI levels were elevated in women undergoing the early stages of labor (29%), in patients receiving intravenous tissue-type plasminogen activator (45%), and in patients receiving intravenous heparin (375%). A radioligand blot of the pre- and post-heparin plasma samples shows the increase to be in a 40-Kd form of LACI. Very low levels of plasma LACI antigen were found in patients with homozygous abetalipoproteinemia and hypobetalipoproteinemia, diseases associated with low plasma levels of apolipoprotein B containing lipoproteins. Following the injection of heparin into one patient with homozygous abetalipoproteinemia, the plasma LACI antigen level increased to a level comparable with that in normal individuals after heparin treatment.
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PMID:Plasma antigen levels of the lipoprotein-associated coagulation inhibitor in patient samples. 207 76

Although pulmonary involvement commonly occurs in systemic lupus erythematosus (SLE), pulmonary vascular hypertension has rarely been reported. Two patients with SLE had pulmonary hypertension without underlying pulmonary parenchymal or cardiac disease. The first patient's condition was initially diagnosed as primary pulmonary hypertension (PPH), and full-blown SLE subsequently developed. The second patient had well-established SLE when respiratory symptoms secondary to underlying pulmonary hypertension developed. There is a possible relationship between connective tissue diseases and PPH.
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PMID:Systemic lupus erythematosus and pulmonary vascular hypertension. 670 30

In many cases of chronic microembolic pulmonary hypertension the source of the emboli is unknown. Disease or injury to the sinus node is usually accompanied by thrombus formation within the antrum atrii dextri, and this region is not often inspected carefully during necropsy. In the present study the hearts of 14 patients with right ventricular hypertrophy or documented pulmonary hypertension showed disease or injury in the sinus node, and each had both old and recent thrombosis in the antrum atrii dextri. In all 14 cases both lungs were available for histological examination. The group included six cases of rheumatic heart disease with mitral stenosis, four cases of disseminated lupus erythematosus, and four cases of so-called primary pulmonary hypertension. Every lung contained both new and old microemboli, as well as a wide variety of other histological abnormalities narrowing the small pulmonary arteries. Many of these narrowing lesions were recognisable as the consequence of prior microembolisation. For future postmortem examination of cases of chronic microembolic pulmonary hypertension, mural thrombi within the antrum atrii dextri should be considered as one important potential source for recurring microembolisation to the lungs.
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PMID:Thrombi in antrum atrii dextri of human heart as clinically important source for chronic microembolisation to lungs. 682 32

Primary pulmonary hypertension is an irreversible and fatal disorder. Every effort should therefore be made to discover all the other treatable diseases which may be associated with pulmonary hypertension. The association of systemic lupus erythematosus and pulmonary hypertension was rarely reported in the past. We add another case in which pulmonary hypertension was the presenting symptom of systemic lupus erythematosus (SLE). In contrast to the previously reported cases, our patient responded well to corticosteroids. It is assumed that this favorable response was due to the relatively early stage of the disease, when the histopathologic pulmonary changes were still in the reversible inflammatory stage.
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PMID:Corticosteroid responsive pulmonary hypertension in systemic lupus erythematosus. 718 37

Non-resolved chronic pulmonary thromboembolism is a frequent cause of pulmonary hypertension. In long-standing disease hypertension is progressive due to intimal and medial changes in the perfused vessels. Non-resolution of thromboemboli is often associated with underlying coagulopathies; the presence of a lupus anticoagulant may pose a significant problem in the peri-operative management of these patients. Pulmonary thrombendarterectomy presents an efficient option of treatment which is feasible in the majority of patients. By means of pulmonary angiography and computed tomography operability is verified by the often difficult recognition of thromboembolic changes in the central pulmonary arteries. Patients with solely peripheral thromboembolic changes or primary pulmonary hypertension must be excluded. In presence of significant exertional dyspnea and/or pulmonary pressure elevation surgery is indicated. Mortality is high and mainly related to unrelieved pulmonary hypertension or pulmonary complications; pulmonary reperfusion edema, respiratory failure or pneumonia and sepsis. In all survivors the reduction of pulmonary hypertension is highly significant and persistent. Thromboembolic pulmonary hypertension may be treated curatively in most patients by thrombendarterectomy. Correct selection of surgical candidates is mandatory, and the patients should preferably be diagnosed and undergo surgery in an early stage of their disease.
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PMID:[Surgical treatment of thromboembolism-induced pulmonary hypertension]. 786 94


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