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)

Fifty-six patients, 49 females and 7 males, with the confirmed diagnosis of systemic lupus erythematosus were examined by M-mode, 2--D and Doppler echocardiography. Pericardial effusion was found in 15 patients (27%), while pericardial thickening was suspected in 6 additional patients (37.5% altogether). Two patients had the signs of a pericardial tamponade, but both of them were uraemic. Libman-Sacks endocarditis was suspected in 4 patients (7.5%) because of verrucous changes in the aortic or mitral valve and regurgitant jet. Slight to moderate left ventricular hypocontractility was present in 3 patients (5%), while 3 additional patients had borderline values of the left ventricular contractility parameters. Left ventricular hypertrophy, usually mild, was found in 21 patients (37.5%). Echocardiographic signs of pulmonary hypertension were present in 2 patients (3.6%). It has been concluded that pericardial affection is frequent during the course of systemic lupus erystematosus, while a diffuse myocardial involvement is rare, except the consequences of arterial hypertension and accelerated coronary atherosclerosis. Libman-Sacks endocarditis still represents a diagnostic problem. For a more precise definition of cardiac involvement in systemic lupus erythematosus, a comparative analysis of the disease activity and immunosuppressive therapy is needed.
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PMID:[Echocardiographic analysis of changes in the heart in patients with systemic lupus erythematosus]. 207 23

In order to detect diagnostic clues to identify patients with chronic immune thrombocytopenia which is likely to develop systemic lupus erythematosus (SLE), a retrospective study was conducted. Of 39 patients with chronic immune thrombocytopenia, 4 fulfilled the diagnostic criteria being between 4.5 and 14 years after the initial diagnosis of "idiopathic" thrombocytopenic purpura. The remaining 35 patients were found to be idiopathic thrombocytopenic purpura (ITP) after at least a 5-year follow-up. It was noted that in patients with SLE who had thrombocytopenia at presentation, only 4 or 5 findings of diagnostic criteria were observed. Of particular interest, all patients who developed SLE were positive for anti-SS-A antibody, in contrast none of the patients with ITP had this antibody. Furthermore, despite the few clinical manifestations, we noted lupus peritonitis in 1 patient and pulmonary hypertension in another patient. In conclusion, the anti-SS-A antibody is an excellent clue for the early detection of SLE occurring as "idiopathic" thrombocytopenic purpura.
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PMID:Thrombocytopenia subsequently develops systemic lupus erythematosus--can anti-SS-A antibody predict the next event? 208 72

The clinical and serological findings on 24 patients with pulmonary hypertension (PHT) seen at the Lupus Clinic of St. Thomas' Hospital, London are presented. Twenty-two patients had systemic lupus erythematosus (SLE), one other a primary antiphospholipid syndrome and another an SLE/progressive systemic sclerosis (PSS) overlap syndrome. In 21 of the 24 patients, the disease resembled the primary idiopathic variety with clear lung fields and no clinical evidence of pulmonary thromboembolism, although angiography and nuclear perfusion scans were not performed. Two patients clearly suffered from thromboembolic PHT, one with SLE and one with an antiphospholipid syndrome. One patient with SLE/PSS overlap syndrome developed pulmonary fibrosis. The frequency of antiphospholipid antibodies (lupus anticoagulant and antibodies to cardiolipin was 68% which appears to be higher than generally found in patients with SLE, and the clinical significance of this finding is unknown. Other associated features of the antiphospholipid syndrome in this group were uncommon. Death occurred in 13 of the 24 patients, 4 were lost to followup and 7 are known to be alive. The cause of death was circulatory failure in the majority; sudden death once this complication occurred was particularly common. One patient died from adult respiratory distress syndrome and one from hemorrhagic shock while undergoing heart/lung transplantation. Two patients underwent successful heart/lung transplantation. One, however, died of a mesenteric occlusion and bowel infarction following a second lung transplantation because of rejection of the first heart/lung transplantation after one year. The other patient is alive and well 2 years later.
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PMID:Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. 212 32

The prevalence of lupus anticoagulant (LAC) and its relation with reported clinical associations has been determined in 55 patients with systemic lupus erythematosus (SLE) from northern India who were studied prospectively. Kaolin clotting time was used to screen for LAC, which was detected in seven (13%) of the patients. Significant associations were found between LAC and thrombotic events, onset of disease at an early age, and disease of shorter duration. No statistically significant association could be found between LAC and recurrent abortions, pulmonary hypertension, thrombocytopenia, and neurological manifestations. It is concluded that LAC is a useful marker for a subset of patients with SLE at risk of thromboembolic events.
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PMID:Lupus anticoagulants in systemic lupus erythematosus: prevalence and clinical associations. 212 9

The description of a phospholipid syndrome in patients with systemic lupus erythematodes (SLE) filled up a gap in defining an "atypical" subgroup--quite often negative to DNA antibodies but suffering from arterial thromboses, thrombophlebitis, aseptic bone necroses, and pulmonary hypertension. 54 patients with SLE and 32 of their closet blood relatives were examined for lupus anticoagulation factor, anticardiolipin antibodies and Wassermann test. The results were interpreted in relation to the forms of the disease and its clinical manifestations. Clinically healthy relatives positive for circulating lupus anticoagulation factor or antiphospholipid antibodies are under close control.
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PMID:[The antiphospholipid syndrome in patients with systemic lupus erythematosus and in their closest blood relatives (a preliminary report)]. 212 71

Clinical features and prognosis of sixty patients with connective tissue disease accompanied by pulmonary hypertension (PH) (26 MCTD, 20 SLE, and 14 PSS) reported retrospectively by multi-institutions were compared. Though the obtained data were incomplete and lacking in uniformity, no significant difference in the clinical features among the three diseases were observed except high incidence of pulmonary fibrosis and low % VC in PSS and PH patients. Statistically significant difference, however, was observed between live and dead patients of three diseases gathered in post sternal pain, pulmonary diastolic murmur, right ventricular hypertrophy on ECG and mean pressure of pulmonary artery. Higher incidence of anti-nRNP antibody was observed in SLE with PH and PSS with PH patients than with the general population. A quicker occurrence of PH and shorter survival time were observed in MCTD patients with PH than in SLE and PSS patients with PH.
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PMID:Pulmonary hypertension in connective tissue disease. Clinical analysis of sixty patients in multi-institutional study. 233 48

A 52-year-old woman presenting with systemic lupus erythematosus (SLE) and a right recurrent laryngeal nerve palsy is described. The symptoms and signs resolved within days of initiating steroid therapy. This is the first reported case of recurrent laryngeal nerve palsy associated with SLE in the absence of pulmonary hypertension or laryngeal lupus.
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PMID:Systemic lupus erythematosus and right recurrent laryngeal nerve palsy. 237 50

The antiphospholipid syndrome--the association of venous and/or arterial thromboses, often accompanied by thrombocytopenia in the presence of the antiphospholipid antibodies ("lupus anticoagulant" antibodies to cardiolipin)--is seen mainly in patients with systemic lupus erythematosus (SLE) and the closely related "lupus-like" disease, i.e., lupus patients not conforming to the 1982 revised American Rheumatism Association classification for SLE. It is also seen in a group of patients who do not manifest any of the major clinical or serologic features of SLE, the majority of whom do not appear to progress to classical lupus. A multicenter study of 70 of these patients is documented and their major clinical and serologic characteristics examined: They have been characterized as suffering from a "primary" antiphospholipid syndrome and present typically with a history of deep vein thromboses, often accompanied by pulmonary thromboembolism, which in a few is complicated by thromboembolic pulmonary hypertension, arterial occlusions (most commonly strokes), or fetal loss. The events are often recurrent and may be accompanied by hemocytopenias (thrombocytopenia and less frequently Coombs positivity and/or hemolytic anemia). They are often antinuclear antibody-negative and are always negative for antibodies to dsDNA and to ENA, typical serologic features of SLE. There may be a family history of SLE or a familial clotting tendency in a minority. The group of patients presented appears to be closely related, but distinctly separate from SLE.
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PMID:The "primary" antiphospholipid syndrome: major clinical and serological features. 250 56

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

A 16-year-old boy had recurrent venous thromboses and pulmonary thromboembolism that caused him pulmonary hypertension. He also had livedo reticularis, thrombocytopenia and high titer IgG antiphospholipid (cardiolipin) antibodies. In the absence of clinical and laboratory evidence of SLE, he was considered to have a primary antiphospholipid syndrome. Coagulation studies revealed a functional deficiency of protein C although it was present in normal antigenic amounts. Since both his parents had normal functional and antigenic protein C findings, his deficiency was considered acquired. The reactivity of the anticardiolipin antibodies could be decreased in a dose dependent fashion when preincubated with increasing amounts of thrombomodulin, a protein required for protein C activation at the endothelial cell membrane. This interaction of antiphospholipid antibodies with thrombomodulin may help explain the occurrence of thrombosis in some patients with antiphospholipid antibodies, despite the behavior in vitro of these antibodies as circulating anticoagulants.
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PMID:Acquired protein C deficiency in a patient with primary antiphospholipid syndrome. Relationship to reactivity of anticardiolipin antibody with thrombomodulin. 254 43


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