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)

Systemic lupus erythematosus (SLE) is frequently associated with cardiovascular manifestations, but rarely complicated with aortic disease. We report a 28-year-old female patient with a 14-year history of SLE and a 3-year history of hypertension. She had suffered from palpitation and chest tightness for 1 month before admission. Heart echo showed thoracic to low abdominal level with low flow. A computed tomography (CT) scan confirmed aneurysms of the descending thoracic and upper abdominal aorta, down to the renal level. Diagnosis of aortic aneurysm should be considered in patients with SLE, especially those who have a history of hypertension, prolonged steroid use, palpitation and chest pain. Current imaging modalities, such as cardiac echo, CT and magnetic resonance angiography may provide earlier detection of subclinical disease, which may aid in preventing these fatal complications. It is important to control hypertension aggressively in patients with SLE. In addition to decreasing steroid doses, early use of immunosuppressive agents and accurate noninvasive image modalities may allow us to prevent severe damage to the aorta and avoid the fatal complications.
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PMID:Aortic aneurysm in systemic lupus erythematosus. 1549 14

Patients with antiphospholipid syndrome (APS) are prone to excessive postoperative morbidity and mortality after cardiovascular surgery because of its thromboembolic derangements. We present a case of coronary artery bypass grafting (CABG) in a patient with primary APS. He suffered from repetitive coronary occlusion after percutaneous transluminal coronary angioplasty (PTCA). Since his lupus anticoagulant level was found to be 217 s (normal, <50 s), he was diagnosed as the primary APS. He received steroid pulsation therapy with 1000 mg of prednisolone, double-filtration plasmapheresis (DFPP) and 50 mg of cyclophosphamide to attenuate the antibody activity. Four months after the last PTCA, he experienced chest pain and approximately 90% of stenosis in the left anterior descending (LAD) lesion was apparent, although the titer for the lupus anticoagulant was reduced to the normal range. He had drug allergy to ticlopidine hydrochloride and aspirin. Taken together, his disease was found to be resistant to these medical treatments, and surgical treatment was considered. Since cardiopulmonary bypass is known to exaggerate its coagulatory and fibrinolytic complications, off-pump CABG (OPCAB) was feasible in this case. The left internal thoracic artery (ITA) was anastomosed to the LAD using the off-pump technique. The procedure was successful, and the postoperative course for 3 years has been satisfactory without any cardiovascular complaints.
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PMID:Off-pump coronary artery bypass in a patient with the antiphospholipid syndrome. 1575 83

Although patients undergoing cardiac surgery often present with diverse comorbidities, those with coagulation derangements are especially challenging. The present report describes the management of a patient who presented with a Factor V Leiden mutation, lupus anticoagulant, and acquired activated protein C resistance. A 42-year-old female presented with acute shortness of breath and chest pain. She was otherwise healthy 1 month prior to admission when she presented with dysfunctional uterine bleeding, resulting in the transfusion of three units of packed red blood cells. Coagulation evaluation revealed that the patient had lupus anticoagulant, factor V Leiden mutation and an activated protein C resistance. The patient presented with an acute myocardial infarction and was found to have 90% stenosis of her left main coronary artery, moderate mitral and tricuspid regurgitation, and a left ventricular ejection fraction of 25%. An emergent off-pump coronary artery bypass procedure with placement of a vein graft to the left anterior descending artery was completed. Intraoperative thrombophilia was encountered as evidenced by both an elevated thromboelastograph coagulation index (+3.6) and an acquired antithrombin-III deficiency. Postoperatively, the patient was placed on low molecular weight heparin, but developed heparin-induced thrombocytopenia and was switched to a direct thrombin inhibitor, argatroban. The following case report describes the coagulation management of this patient from the time of admission to discharge 43 days later, and the unique challenges this combination of hemostatic defects present to the clinicians.
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PMID:Coagulation management of a patient with factor V Leiden mutation, lupus anticoagulant, and activated protein C resistance: a case report. 1591 49

A 39-year-old woman with a history of systemic lupus erythematosus developed chest pain and conduction abnormalities. An echocardiographic examination revealed systolic dysfunction and ventricular thickening. Because of the unclear nature of her cardiac disease, right ventricular endomyocardial biopsy was performed. Light microscopy showed diffuse myocyte vacuolization without myocarditis, and transmission electron microscopy demonstrated sarcoplasmic myelinoid and curvilinear bodies, diagnostic of hydroxychloroquine toxicity. Among patients with autoimmune connective disorders, cardiac dysfunction may be a result of the disease or occasionally of its treatment. Although the nature of the cardiac disease (myocardial, valvular, or pericardial) can generally be evaluated echocardiographically, endomyocardial biopsy may be indicated to rule out diseases with a specific microscopic appearance, such as myocarditis or hydroxychloroquine cardiotoxicity.
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PMID:Hydroxychloroquine-induced cardiotoxicity in a 39-year-old woman with systemic lupus erythematosus and systolic dysfunction. 1615 29

Scleroderma heart involvement (SHI) is often manifest, and virtually always present when accurately searched and holds a significant prognostic value. Myocardial involvement by patchy fibrosis (secondary to both repeated ischaemia and immunoinflammatory damage) leads to ventricular diastolic dysfunction, whereas right ventricle overload and failure may complicate pulmonary hypertension. Left ventricular systolic dysfunction is present in a minority of patients, namely those presenting atherosclerotic coronary artery disease and/or arterial hypertension, sometimes triggered by sclerodermic renal involvement. Dysrhythmias and conduction disturbances are considered an hallmark of SHI, facilitated by autonomic dysfunction. SHI is frequently linked to parenchimal and/or vascular lung disease; they determine symptom occurrence, particularly dyspnoea, fatigue, palpitations and chest pain when pericardium is affected. Accurate cardiologic baseline screening and subsequent follow-up are mandatory in all patients, initially consisting in some noninvasive diagnostic procedures: visit, electrocardiogram (EKG), chest X-ray, Doppler-echocardiography. When needed, these examinations should be integrated by EKG Holter-monitoring, cardiopulmonary stress tests, cardiac magnetic resonance imaging, nuclear studies of myocardial function and perfusion, cardiac catheterization to better estimate pulmonary hypertension, and cardiac natriuretic hormone evaluation. Several vasodilator approaches (prostacycline or NO/endothelin) may counteract the microvascular dysfunction at peripheral and cardiopulmonary level, and fight the sequelae of pulmonary hypertension.
Lupus 2005
PMID:Heart involvement and systemic sclerosis. 1621 71

We report on a rare case of a late-onset drug-induced lupus erythematosus. A 35 year old male patient complained about dyspnea, chest pain and reduced physical activity for three months. His medical history consisted of epilepsy treated with carbamazepine for 20 years. After diagnosis of a large pericardial effusion and percardiocentesis (1200 ml) the diagnosis of viral perimyocarditis was suspected. Under antiphlogistic treatment the symptoms vanished initially. Four weeks later the pericardial effusion recurred and a livedo reticularis became evident. A structural or infectious heart disease, in particular viral myocarditis, was ruled out invasively. Serologic testing revealed antinuclear antibodies and antibodies against histones without presence of antibody against ds-DNA, thereby confirming the diagnosis of carbamazepine-induced lupus erythematodes. After discontinuation of carbamazepine and immunosuppressive medication the patient recovered completely.
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PMID:[Chest pain, reduced physical activity, and polyserositis in a 35-year old patient with anticonvulsive medication]. 1628 38

To study the prevalence of connective tissue disorders and identify the epidemiological profile of the population from the northern and northeastern regions of Brazil. All patients admitted to the general medical ward at Getulio Vargas Hospital were screened through history and physical examination for the existence of known, suspected or possible connective tissue disorder. A total of 120 patients were enrolled in the study - 61 (50.8%) with systemic lupus erythematosus (SLE), 41 (34.2%) with rheumatoid arthritis (RA), 16 (13.3%) with systemic sclerosis (SS), one (0.8%) with mixed connective tissue disease (MCTD) and one (0.8%) with polymyositis (PM). Women accounted for (104) 86.7% of the participants and (83) 70.9% were non-Caucasians. The mean age was 36.8 years. Alopecia was the most frequent symptom (55/46.6%), followed by other cutaneous lesions (50/42.4%), dyspnea (47/39.8%) and chest pain (39/33.0%). The most frequent rheumatologic disorder encountered in our hospital-based study population was systemic lupus erythematosus, which is contrary to findings of the majority of studies performed in this country.
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PMID:Epidemiological study of patients with connective tissue diseases in Brazil. 1635 68

The patient was a 37-year-old female, who was diagnosed as having systemic lupus erythematosus (SLE) with nephrotic syndrome in 1991. SLE has been well controlled with a combination therapy of prednisolone, cyclophosphamide and mizoribine. She was admitted to our hospital for chest pain on exertion in June 2002. A grade of 2 systolic murmur was heard along left sternal border and edema in the both lower legs was present. Laboratory findings showed proteinuria and anemia. Serological tests did not show decrease in complements and was negative for autoantibodies including anti-ds-DNA antibody. The serum level of brain natriuretic peptide was 651 pg/ml. On chest X-ray films, there were no remarkable findings. An electrocardiogram showed a pattern of left ventricular hypertrophy with inverted T wave. The heart ultrasonic test recognized asymmetric hypertrophy of the septum, being more prominent in the apex, but there was no obstruction of the left ventricular outflow tract. Examination of an endomyocardial biopsy specimen showed disarray and mild hypertrophy of myocardial cells, which were compatible with hypertrophic cardiomyopathy (HCM), but there were no pathological findings specific for SLE. Additional treatment with beta-blocker under a diagnosis of HCM resulted in a favorable response. Although 7 SLE patients with HCM have been reported, endomyocardial biopsy was not performed. There appears to have been a chance association between SLE and HCM, considering the clinical courses in reported cases and the pathological findings in our case.
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PMID:[A case of systemic lupus erythematosus associated with hypertrophic cardiomyopathy]. 1639 45

Mounting evidence from a growing body of epidemiologic studies demonstrates that patients with systemic lupus erythematosus (SLE) are at increased risk for the development of premature cardiovascular disease (CVD). However, awareness of accelerated atherosclerosis in young SLE patients, albeit growing, is still limited, as documented by the brief case presented. Inflammation is thought to play an important role in both the pathogenesis of SLE, as well as atherosclerotic vascular disease. Inflammatory processes that are shared by SLE and atherosclerotic disease include immune complex deposition and fixation, autoantibody binding, complement activation and CD40-CD40 ligand interaction. By examining the inflammatory mechanisms in common between SLE and atherosclerotic disease, we can come to a better understanding of the pathophysiology of the accelerated atherosclerotic process seen in patients with SLE and can gain insights into developing and instituting preventative and treatment strategies. In this article, we present a case of a young woman with SLE who presents with chest pain, followed by a review of inflammation-based pathogenic mechanisms that are shared by SLE and atherosclerotic cardiovascular disease.
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PMID:Premature atherosclerotic disease in systemic lupus erythematosus--role of inflammatory mechanisms. 1643 36

We herein present a rare case of severe aortic valve stenosis with a bicuspid valve in a patient with systemic lupus erythematosus. The symptoms resulted from aortic valve stenosis, such as chest pain, dyspnea and syncope, which subsided after the insertion of an intra-aortic balloon pump. Thereafter, a calcified bicuspid aortic valve was successfully replaced with a mechanical valve. The pathological findings of the resected valve included irregular fibrotic thickening and marked calcification without any vegetation or thrombus formation. The efficacy of an intra-aortic balloon pump for the relief of symptoms associated with severe aortic valve stenosis indicates its usefulness for such critically ill patients prior to undergoing valvular surgery.
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PMID:Stenosis of the bicuspid aortic valve with systemic lupus erythematosus. 1648 31


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