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)

In Sweden, clinicians at the Karolinska Hospital in Stockholm interviewed and took blood samples from 81 women aged 18-52 to examine the incidence of an insufficient response to activated protein C (APC), an increased level of antibodies to anionic phospholipids (Pla), and the presence of the mutation in the factor V gene in women who developed thrombosis while using oral contraceptives (OCs), in OC users who developed thrombosis during other risk situations (pregnancy or delivery, surgery, idiopathic), and in non-users who had a history of thrombosis. Women who had thrombosis during OC use had fewer pregnancies before developing thrombosis (p 0.05) and fewer recurrences after the thrombotic event than women in the other two groups. Non-users had the highest proportion of thrombotic recurrences (26%). Pulmonary embolism occurred more often as a result of the thrombotic event during OC use than during pregnancy, delivery, or surgery (p 0.01). APC resistance occurred in 27% of all women. The normalized ACP (nACP) ratio ranged between 0.41 and 1.48. Women who developed thrombosis during OC use had a significantly lower APC resistance than the other two groups (p 0.05). APC resistance increased as did the recurrence of thrombotic events (14-42%). The mean nACP ratio was highest among women who developed thrombosis during OC use and lowest in non-users (0.94 vs. 0.72). 40% of all women had mutation in the factor V gene. All but one woman was heterozygous. This mutation was present in relatively the same proportion in all three groups. The frequency of mutation was greater than that with laboratory-identified APC resistance in women with a history of thrombosis during OC use (38% vs. 14%; p 0.025). Coagulation and genetic analyses were highly correlated (p = 0.001). Pla were present in all three groups at essentially the same levels. Yet lupus anticoagulant activity was more common in OC users who developed thrombosis during other risk situations than the other two groups (p 0.05). 12% of all women had APC resistance and Pla. These findings show a flexible APC response.
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PMID:Thrombotic risk factors and oral contraception. 766 78

Pleuropulmonary complications of systemic lupus erythematosus (SLE) occur in 50-70% of patients and include pleuritis, pleural effusions, acute lupus pneumonitis, diffuse interstitial lung disease, atelectasis, diaphragmatic dysfunction and bronchiolitis obliterans. Additionally, a syndrome of acute reversible hypoxemia has recently been documented. This seems to occur in patients hospitalized for exacerbations of SLE and may be due to pulmonary leukoaggregation. It has become clear that other groups of pulmonary complications may be specifically associated with the antiphospholipid antibodies, both in patients with SLE and in those suffering from the "primary" antiphospholipid syndrome. These include pulmonary embolism and infarction, both thromboembolic and perhaps nonthromboembolic pulmonary hypertension, pulmonary arterial thrombosis, pulmonary microthrombosis, adult respiratory distress syndrome, intraalveolar pulmonary hemorrhage, as well as a postpartum syndrome.
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PMID:Review: antiphospholipid antibodies and the lung. 769 84

Antiphospholipid syndrome is a well-defined clinical and serological entity characterized by venous and arterial thrombosis, recurrent abortion and thrombocytopenia. The immunological feature shows the presence of anticardiolipin antibodies and lupus anticoagulant. Cardiac involvement includes pericardial effusion, myocardial dysfunction, valvular disease, coronary artery occlusion. The literature reports only a few cases of intracardiac thrombosis associated with primary antiphospholipid syndrome. Here we describe a case of a 54 year-old woman with thrombocytopenia and history of recurrent abortions. When she was 51 she presented a left deep iliac venous thrombosis, followed by recurrent pulmonary embolism; a caval filter was placed in the inferior vena cava. Transthoracic and transoesophageal echocardiogram showed 3 masses in the right atrium: the first one was connected with the atrial wall, hyperechogenic and extending to the right ventricle in diastole: th second was connected with the interatrial septum and less echogenic; the third was in connection with septum-tricuspid valve junction and it was floating in the atrium. The immunological feature showed the presence of lupus anticoagulant and antiphospholipid antibodies; the histological examination of the masses, surgically removed, proved they were thrombi coated by endothelial cells. The case reported is very uncommon and offers the opportunity to emphasize the difficulty of diagnostic differentiation of intracardiac masses using echocardiographic imaging: thrombotic masses may present similar characteristics of seat, morphology and echogenicity of other cardiac masses. For these reasons it can be useful to look for antiphospholipid antibodies and lupus anticoagulant in such clinical conditions.
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PMID:[Primary antiphospholipid syndrome and cardiac involvement. Description of a clinical case of right atrial thrombosis]. 770 May 44

The antiphospholipid syndrome (APS) is usually defined by the association of a clinical manifestation (venous or arterial thrombosis or miscarriage) with the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies). It frequently occurs in the course of systemic lupus erythematosus but is also encountered as a "primary" disease. APS is responsible for diverse respiratory manifestations. Pulmonary embolism is common. The site of the causal venous thrombosis is frequently unusual. Pulmonary hypertension may be a consequence of repeated embolism or may belong to the primary idiopathic variety. Pulmonary manifestations may also result from left-sided heart failure due to mitral or aortic valve abnormalities, myocardial infarction or a specific myocardiopathy. APS is probably involved in the occurrence of some cases of adult respiratory distress syndrome. Long term secondary prevention of recurrent thrombosis is a central point in the management of APS.
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PMID:[Antiphospholipid syndrome and the pneumologist]. 772 79

Pulmonary hemorrhage due to small vessel vasculitis is a well recognized complication of many autoimmune and collagen vascular disorders. The antiphospholipid antibody syndrome (APS) is a disorder associated with hypercoagulability, thrombocytopenia, and thromboembolic phenomena. Pulmonary manifestations include pulmonary embolism, pulmonary hypertension, and respiratory failure associated with diffuse pulmonary infiltrates. We describe a patient with the APS syndrome, without systemic lupus erythematosus, who developed pulmonary hemorrhage due to pulmonary capillaritis with evidence of perivascular immune complex deposition.
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PMID:Pulmonary capillaritis and alveolar hemorrhage associated with the antiphospholipid antibody syndrome. 778 81

The antiphospholipid syndrome is a thrombotic disorder which can occur in a primary form or more classically in systemic lupus erythematosus. An association between cryptogenic fibrosing alveolitis and the antiphospholipid syndrome has not previously been reported. We describe a patient with severe cryptogenic fibrosing alveolitis who developed pulmonary embolism and myocardial infarction in the presence of antiphospholipid antibody. The case also illustrates that worsening respiratory function may represent superimposition of one lung pathology on another, and may not simply be a deterioration of the pre-existing disease.
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PMID:Antiphospholipid syndrome in a patient with rapidly progressive fibrosing alveolitis. 778 86

Obstetrician-gynecologists reviewed patient records of women delivering during January 1986-December 1992 to determine the maternal mortality rate and trends and the causes of maternal deaths in the maternity ward at the National University of Singapore. There were 26,173 deliveries and 9 maternal deaths (a maternal mortality rate of 22.9/100,000). The causes of maternal deaths were pulmonary embolism (underlying condition, systemic lupus erythematosus [SLE]), hemorrhage from multiple sites (thrombotic thrombocytopenia), acute exacerbation of SLE with interstitial pneumonitis, pulmonary fibrosis (systemic sclerosis), fulminant hepatitis (prior hepatitis and liver disease), and cerebral embolism (rheumatic heart disease with mitral valve replacement). There were also three incidental maternal deaths bringing the maternal mortality rate up to 34.4/1000. The incidental causes of death included septicemia from perforated peptic ulcer (uncontrolled thyrotoxicosis), multiple metastases from lung cancer, and suicide (family dispute over adoption of newborn). A cesarean section preceded 4 (44%) of the 9 maternal deaths. Two of these deaths were incidental maternal deaths. Cesarean section was related to two of the remaining six (33%) deaths. These findings show that traditional direct causes of maternal death (hemorrhage, sepsis, embolism, or hypertension) were not responsible for the maternal deaths at this tertiary facility. Instead, the women tended to have medical conditions that placed them at high risk of death regardless of pregnancy status.
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PMID:Maternal mortality: evolving trends. 781 Nov 98

Chronic thromboembolic pulmonary hypertension (CT-E PH) is a rare and aberrant outcome of acute pulmonary embolism. Because it has become a potentially curable form of pulmonary hypertension, the frequency of recognized cases has increased. We report a case series of 72 patients with CT-E PH evaluated in our institution between 1984 and 1993, and discuss diagnostic clues and therapeutic approaches. All patients complained of dyspnea on exertion, a history of acute thromboembolic event, and lung murmurs were found in 60% and 17% of patients, respectively. The presence of a disorder of coagulation was found in 30% of the patients tested, the most common abnormality being lupus anticoagulant. The key noninvasive study for diagnosis was the lung perfusion scan which showed at least one segmental or larger perfusion defect in all patients. Pulmonary angiography confirmed the diagnosis in all cases, and sometimes associated to intravascular ultrasound imaging, established the feasibility of thromboendarterectomy. Medical therapy included the use of long-term oral anticoagulant, and in the case of lower limb venous thrombosis, inferior vena cava filtration. Finally two surgical procedures were discussed in selected patients: thromboendarterectomy and lung transplantation. Since 1988, eight patients have benefited from lung transplantation (six patients are still alive), and 11 patients underwent thromboendarterectomy which was successful in 9 patients with a dramatic functional and hemodynamic improvement.
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PMID:Surgical management of unresolved pulmonary embolism. A personal series of 72 patients. 781 30

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

We report a 14-year-old boy with systemic lupus erythematosus and anticardiolipin antibodies. He developed asymptomatic superior vena cava thrombosis and silent pulmonary embolism. Computed tomographic scan of his chest and abdomen showed features of long standing obstruction secondary to superior vena cava thrombosis. Asymptomatic thrombosis of the major vessels and silent pulmonary emboli are part of the disease spectrum of pediatric antiphospholipid syndrome.
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PMID:Asymptomatic superior vena cava thrombosis and pulmonary embolism in an adolescent with SLE and antiphospholipid antibodies. 803 93


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