Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

Although nephritis remains a very important clinical disease manifestation of systemic lupus erythematosus (SLE), the focus has shifted from issues of mortality to morbidity. An improved method for measuring renal function is presented, and the value of renal biopsy, specifically the chronicity index, is confirmed as a predictor of end-stage renal disease. Several studies detailing the generally favorable course in SLE patients with renal failure who have eventually required chronic dialysis or renal transplantation are discussed. A high frequency of cardiovascular manifestations, often subclinical, is documented, including abnormalities of ventricular and valvular function. The clinical course and response to treatment of SLE patients with diffuse interstitial lung disease and pulmonary hypertension are outlined. Cases of the unusual gastrointestinal presentation of protein-losing enteropathy in SLE have been described by several authors. The clinical correlates and significance of persistent rheumatoid-like arthritis and radiologic erosive and cystic bony lesions in SLE are discussed. Serologic markers of specific SLE disease manifestations and general predictors of disease activity are evaluated. Complications related to infection, malignancy, and pregnancy are described.
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PMID:Clinical manifestations of disease activity, its measurement, and associated morbidity in systemic lupus erythematosus. 175 10

The ultrastructure of pulmonary alveolar capillaries in idiopathic interstitial pneumonia (IIP) and collagen vascular diseases (CVD) were investigated. In IIP, the endothelial cells were only slightly swollen. The basement membrane (BM) was moderately thickened. In CVD, swelling of the endothelial cells and thickening of the BM were more severe than those of IIP. The injury of the endothelial cells seemed to be more severe than IIP. The patients with PSS and MCTD revealed degeneration of endothelial cells as well as lamellation of BM. The endothelial cell damage was most severe among CVD cases, and these changes were assumed to induce pulmonary hypertension. Microtubular structures were observed in the endothelium of SLE, dermatomyositis, polymyositis, PSS and MCTD patients. Weibel-Palade bodies and increase of pericytes were noticed in PSS and MCTD patients which seemed to be related to endothelial injuries. No electron-dense deposits were observed in any case.
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PMID:[An electron microscopic study of the pulmonary alveolar capillaries in idiopathic interstitial pneumonia and lungs of collagen vascular diseases]. 175 17

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

We describe a 26-year-old patient with systemic lupus erythematosus and pulmonary and portal hypertension associated with the presence of anticardiolipin antibodies. Postmortem histological examination revealed the presence of recent thromboses of pulmonary and portal vessels. Our findings further support the concept of pulmonary hypertension being part of the antiphospholipid syndrome and suggest that portal hypertension due to intrahepatic portal vein thrombosis might be another manifestation of this syndrome.
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PMID:Portal and pulmonary vessel thrombosis associated with systemic lupus erythematosus and anticardiolipin antibodies. 179 32

Pulmonary hypertension due to recurrent thromboembolism is a rare disease but life-threatening. We evaluated 18 patients (11 female, 7 male) with this pathology between 1973 and 1991. We compared clinical features and evolution of our patients with the ones of the literature. The mean interval between beginning of symptoms and diagnosis was 5 years (range 1-10 years) and the most frequent symptom was increasing dyspnoea. In 2 of our patients there were well definite predisposing causes for thromboembolism (intracardiac catheters), 6 of the others had a previous episode of acute pulmonary embolism. Mean pulmonary arterial pressure was 50 mmHg and low output was present in 8 of these. Lung perfusion scintigraphy was diagnostic in 98% of cases showing segmental defects and pulmonary angiography confirms diagnosis revealing abrupt cut-off of cases showing segmental defects and pulmonary angiography confirms diagnosis revealing abrupt cut-off a major pulmonary artery. Angiographic evaluation of thrombus extent and location was difficult. In a small number of patients was found lupus anticoagulant, deficiency of protein C, of protein S and of antithrombin III. Mortality in medical treatment was 39% at a mean follow-up of 4-5 years. Progression of pulmonary hypertension was due to recurrent pulmonary embolism only in 30-40% of cases. The role of caval filter is not well established. Thromboendarterectomy shows immediate good results at short time but the long-term results are not known.
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PMID:[Thromboembolic pulmonary hypertension]. 184 71

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

A 22 year-old-female had suffered from polyarthralgia and Raynaud's phenomenon since 1984. In 1986, she was diagnosed as systemic lupus erythematosus (SLE). In April 1988, she was admitted to Kawasaki Municipal Hospital because of fever and dyspnea on exertion (DOE). Physical examination showed high fever, butterfly rash, oral ulcer and elevation of heart sound IIp on auscultation. Laboratory findings revealed that erythrocyte sedimentation rate was elevated to 105 mm/hr. The following values were observed, anti DNA antibody 391 IU/ml, serum IgA 5mg/dl, anti IgA antibody weakly positive. Chest X ray showed CTR 65%. Echo cardiogram showed massive pericardial effusion. 201T1 myocardial SPECT revealed right ventricular pressure over loading. PSL 40 mg/day was started to administer for the massive pericardial effusion due to SLE activities. On 6th of June, right heart catheterization confirmed the pulmonary hypertension (PPA 22 mmHg, Pulmonary artery resistance (PAR) 1163 dyne/sec/cm-5/mm2). By the treatment with PSL, massive pericardial effusion was gradually improved but DOE clinically unchanged. Second right heart catheterization was done on 8th of August. PAR was improved to 895 dyne/sec/cm-5/mm2 but PPA was elevated to 26 mmHg. It is very interesting that PPA was elevated although PAR was improved by PSL therapy. It is considered that the increase in venous return which was caused by improvement of massive pericardial effusion induced conversely the elevation of PPA. Additionally she was complicated with IgA deficiency. It may occur not only by the immunogenetical disorder such as HLA or IgG subclass alteration but also by anti IgA antibody or lymphocytes dysfunction complicated with SLE.
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PMID:[A case of systemic lupus erythematosus complicated with pulmonary hypertension and massive pericardial effusion]. 192 98

Pregnancies complicated by collagen vascular diseases present a challenge to the obstetrician, internist, and other consulting physicians caring for the patient. Although most pregnancies complicated by these disorders result in satisfactory outcomes, a significant number develop manifestations sufficiently serious to require admission to an intensive care unit. Such manifestations include lupus nephritis, CNS lupus, ARDS, massive alveolar hemorrhage, scleroderma renal crisis, pulmonary hypertension, severe myositis, and diffuse vasculitis. Of the numerous therapeutic options available, many may be used to optimize maternal and perinatal outcome.
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PMID:Pregnancy complicated by collagen vascular disease. 194 52

Identical twins were simultaneously diagnosed with systemic lupus erythematosus (SLE) at the age of 12. Later, both developed pulmonary involvement of SLE including pleural effusions, pleuritis, and recurrent bronchopneumonia. In their last year of life, their pulmonary condition deteriorated and they died of severe pulmonary hypertension at the age of 20. At autopsy, small to large pulmonary arterial walls were markedly thickened. There was no SLE renal involvement. Pulmonary hypertension in SLE without pulmonary parenchyma involvement is extremely rare, with only 18 such cases reported. This is the first report of pulmonary hypertension observed in identical twins with a similar clinical course. This case may suggest the possible genetic fate of pulmonary involvement of SLE.
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PMID:Fatal pulmonary hypertension in identical twins with systemic lupus erythematosus. 200 51


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