Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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

A 36-year-old woman was admitted for recurring chest pain and hemoptysis. Blood pressure in the right and left arms was equal, and no murmurs or bruits were heard. Body temperature was normal on admission and remained within the normal range during the hospital stay. C-reactive protein was slightly elevated (2.3 mg/dL) and lupus anticoagulant was positive. Angiography showed no abnormality of the aorta or its branches, but the left pulmonary artery showed occlusion at the proximal portion. Computed tomography (CT) revealed segmental wall thickening of the thoracic aorta. Fluorine-18-fluorodeoxyglucose positron emission tomography (18FDG PET) showed high uptake in the proximal portion of the left pulmonary artery and in the thoracic aorta with wall thickening on CT. Based on these findings, a diagnosis of Takayasu's arteritis associated with antiphospholipid syndrome was made and high-dose steroid therapy (prednisolone 30 mg/day) was started. Two months later, the C-reactive protein level had decreased from 2.3 mg/dL to 1.1 mg/dL, and both the focal wall thickening and (18)FDG uptake of the thoracic aorta were decreased. 18FDG PET was useful for evaluating the efficacy of the steroid therapy in addition to making a diagnosis of Takayasu's arteritis associated with antiphospholipid syndrome.
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PMID:Usefulness of fluorine-18-fluorodeoxyglucose positron emission tomography in a patient with Takayasu's arteritis associated with antiphospholipid syndrome. 1660 57

Myocardial infarction in patients with systemic lupus erythematosus (SLE) is most commonly a consequence of atherosclerosis. Coronary vasculitis with aneurysms is a rare cause of myocardial ischemia in SLE. We present a case of a 22-year-old woman with a 4-year history of SLE who was admitted with acute onset of chest pain. Although initially treated for lupus pericarditis, she was subsequently found to have an acute myocardial infarction. Cardiac catheterization revealed multiple areas of aneurysmal coronary dilatation and only moderate stenoses of the secondary branches.In view of the angiographic findings, coronary revascularization was not indicated. Anticoagulant therapy was initiated as a result of the presence of large aneurysmal coronary dilatations, which are predisposed to in situ thrombosis and distal embolization. The coronary vasculitis was treated with immunosuppressive therapy. Measures aimed at secondary prevention of coronary artery disease, including optimization of lipid profile, blood pressure control, and prevention of left ventricular postinfarct remodeling, were initiated and continued indefinitely.
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PMID:Coronary vasculitis with acute myocardial infarction in a young woman with systemic lupus erythematosus. 1704 68

Systemic lupus erythematous (SLE) is a chronic inflammatory autoimmune disorder that primary affects women and may affect any organ system. Pleural inflammation is a common feature of SLE; however, as an initial presentation in SLE is rare. The author report the case of a 23-year-old woman with chest pain, dyspnea and without fever for 1 week. On physical examination, fine crackles were heard and vocal fremitus was decreased at the base of the left-side of lung. Patient had no symptoms/signs that can meet the SLE criteria; however, immunological workup showed positive response of ANA-speckle, anti-dsDNA, and anti-ENA in patient serum and pleural fluid. Lupus pleuritis with effusions was confirmed by the above investigation. A 1-month course of oral prednisolone-combined oral methrotreate was beneficial in relieving the pleuritis and pleural effusions.
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PMID:Pleuritis and pleural effusion as the initial presentation of systemic lupus erythematous in a 23-year-old woman. 1849 95

Diffuse alveolar haemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). We present the case of a 24-year-old Cambodian woman with SLE followed in the Brigham and Women's Hospital Lupus Center in Boston, Massachusetts. She presented with dyspnoea and chest pain and was found to have DAH that required a prolonged hospitalization that was complicated by recurrent DAH episodes and multiple infections. We discuss the diagnostic approach and management of patients with SLE-associated DAH as well as treatment options for refractory disease. Emerging therapies include plasmapheresis, the anti-CD20 monoclonal antibody rituximab and recombinant activated Factor VII therapy. In addition, we review the literature to date and compile what is known about the epidemiology, presenting features, diagnostic findings, management and outcomes in this condition. We found that DAH has been reported in 1.9% of patients with SLE. These patients were mostly female (88%) and young (mean age 30.2 years). Common presenting features included dyspnoea (94%), anaemia (97%) and new radiographic chest infiltrate (99%). Bronchoscopy, when performed, identified DAH in 90% of cases. Corticosteroids were the mainstay of care, and usage of cyclophosphamide varied by report. Despite recent advances in therapy, mortality has not improved substantially (48% overall survival versus 53% survival in reports published since 1993).
Lupus 2009 Aug
PMID:Dyspnoea in a young woman with active systemic lupus erythematosus. 1957 1

We report a 36-year-old woman with a 1-year history of systemic lupus erythematosus who was admitted with acute onset of dyspnea and chest pain. She presented with a classic medical history of antiphospholipid antibody syndrome, including spontaneous abortion, deep venous thrombosis, and clinical manifestations of lupus activation. The differential diagnosis was made after a detailed history and examinations with transthoracic/transesophageal echocardiography, deep venous ultrasonography, chest computed tomography, and coronary angiography. This case demonstrates a left ventricular apical thrombus in angiographically normal coronary arteries and also deep vein thrombosis causing acute pulmonary thromboembolism. Antiaggregant and anticoagulant therapies were initiated as a result of the presence of a left ventricular apical thrombus and deep venous thrombosis, which is predisposed to recurrent pulmonary or systemic embolization. Control echocardiography demonstrated resolution of apical thrombus and normalized left ventricular systolic function after aspirin, warfarin, and immunosuppressive therapy for 2 months.
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PMID:Antiphospholipid antibody syndrome: coexistence of left ventricular apical thrombus and deep vein thrombosis causing pulmonary thromboembolism in a patient with systemic lupus erythematosus. 2011 28

The prevalence of systemic lupus erythematosus (SLE) is 28 per 100,000. The disease is most common in people of Caribbean or Asian descent. SLE mainly affects adults and is common in women between the ages of 20 and 40 years, with a female to male ratio of 9:1. The pathogenesis is multifactorial and encompasses multiple immunological, vascular and inflammatory processes. Diagnosing SLE can be challenging because of the myriad of clinical features and substantial variability between patients. Cutaneous involvement is present in about 60% of cases and typically manifests as a malar or butterfly rash. Joint involvement is inflammatory in nature with arthralgia, arthritis and/or tendinitis and occurs in about 90% of patients with SLE. Cardiorespiratory symptoms are common with chest pain on inspiration due to lupus-induced pleurisy or pericarditis, which may be associated with effusions. Lupus glomerulonephritis is one of the most important systemic complications, occurring in about 30% of patients with SLE in the UK. Careful screening tests for renal disease need to be undertaken as it is asymptomatic. The diagnosis of SLE is traditionally based on a combination of clinical features and laboratory findings and any patient with suspected clinical features of lupus should be investigated for the presence of autoantibodies. Treatment often includes corticosteroids, by various routes, at different points in disease management. In addition, some experts advocate the use of hydroxychloroquine, an antimalarial, as a principal drug in all SLE patients. It is beneficial in the management of mucocutaneous, musculoskeletal, serosal and constitutional symptoms.
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PMID:GPs have key role in shared care of patients with SLE. 2012 Aug 28


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