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)

Recent reports describe the association of antiphospholipid antibodies (aPL) with chorea or severe heart valve lesions in systemic lupus erythematosus, lupus-like disease, or the primary antiphospholipid antibody syndrome. We conducted a case series and a case-control investigation of patients with rheumatic fever with Sydenham chorea or other manifestations of rheumatic fever for anticardiolipin antibodies (aCL) during the acute attack and disease remission. Eighty percent of patients were positive for aCL during the rheumatic fever attack vs 40% when inactive (p = 0.035); IgG and IgM aCL increased significantly with disease activity. Individuals with or without Sydenham chorea were equally positive for aCL (76 and 83%, respectively). A significant association was found between IgM aCL and carditis: All patients with valvulitis had IgM aCL (100%) vs 37% of patients without valvular involvement (p = 0.02). aPL may play a role in the pathogenesis of some clinical manifestations of acute rheumatic fever.
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PMID:Anticardiolipin antibodies in acute rheumatic fever. 781 96

Severe aortic and mitral valvular insufficiency developed in a 52-year-old woman with systemic lupus erythematosis after previous treatment with corticosteroids, splenectomy, and cyclophosphamide for immune thrombocytopenia and hemolytic anemia. After aggressive management of the thrombocytopenia, simultaneous prosthetic replacement of both valves was satisfactorily performed; pathologic examination revealed myxoid degeneration. The patient was doing well with excellent valve function 26 months after the operation. The relationship of steroid therapy to lupus valvulitis is reviewed.
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PMID:Simultaneous aortic and mitral valve replacement for lupus endocarditis: report of a case and review of the literature. 327 37

We reviewed the medical records and autopsy reports of 50 patients with systemic lupus erythematosus to determine the clinical and neuropathological features of this disease. Neuropsychiatric disturbances were found in the majority (74%) of the patients, occurring as psychiatric illness only (5 patients), neurological disorders only (15 patients), and both together (17 patients). Central nervous system (CNS) lesions were present in half the patients; embolic brain infarcts (10 patients) and CNS infections (8 patients) were the most common. Cardiac sources of emboli were Libman-Sacks endocarditis (5 patients), chronic valvulitis (2 patients), and left side of heart mural thrombus (2 patients). There was no evidence of active CNS vasculitis. Clinical features of thrombotic thrombocytopenic purpura (TTP) developed during the terminal illness in 14 patients, 7 of whom also had pathological evidence of TTP. Correlation between neuropsychiatric disorders and brain lesions could be made in approximately half the patients. This study indicates that cardiac emboli from Libman-Sacks endocarditis and TTP are common pathogenetic factors of CNS disease in systemic lupus erythematosus, whereas CNS vasculitis is rare.
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PMID:Clinical and neuropathological findings in systemic lupus erythematosus: the role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura. 338 74

This paper describes a 47-years old patient with the history of lupus valvulitis for six and a half years, who had implantation of a Starr-Edwards prosthesis for a mitral insufficiency four years ago. This case represents the longest survival reported so far after mitral valve replacement for lupus valvulitis.
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PMID:Mitral valve replacement for lupus valvulitis. 382 24

In this review, the cardiac lesions which develop in association with the various collagen-vascular diseases are described. In rheumatoid arthritis, the most frequent lesions are: fibrous obliterative pericarditis, with pericardial deposits of calcium, fibrin, cholesterol, and rheumatoid granulomas; granulomatous or nonspecific myocarditis; valvulitis, vasculitis, and amyloid deposits. In ankylosing spondylitis, the lesions involve mainly the valves (aortic and mitral valves) and the aorta. In systemic lupus erythematosus, the predominant cardiovascular lesions are: pericarditis, Libman-Sacks endocarditis, nonspecific myocarditis, vasculitis with fibrinoid necrosis, and acceleration of atherosclerosis. In scleroderma, the main cardiac lesion is fibrosis with only scanty inflammatory cells; pericarditis and nonbacterial thrombotic endocarditis also occur. In dermatomyositis/polymyositis, fibrous or fibrinous pericarditis can occur, as well as myocarditis with infiltrates of lymphocytes and plasma cells and with degeneration and necrosis of myocytes; valvulitis is uncommon except when the disease is related to mucinous adenocarcinoma. In polyarteritis nodosa, various stages of necrotizing vasculitis involve all layers of the arterial walls; foci of myocardial necrosis of various sizes can occur in association with these lesions; cardiac hypertrophy related to hypertension and pericarditis related to uremia, may also be found. In Wegener's granulomatosis, pericarditis, inflammatory infiltrates, necrotizing granulomas, and vasculitis have been observed in the heart.
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PMID:Cardiovascular lesions in collagen-vascular diseases. 391 76

Valvulitis in systemic lupus erythematosus has been observed for many years. Fourteen cases of mitral valve replacement have been reported so far in the literature. In the light of all previously reported experiences, prosthetic valve related morbidity and mortality remain high in patients with systemic lupus erythematosus. We describe the first true instance of reconstructive approach in a patient who presented with severe mitral incompetence due to lupus valvulitis. The patient was clinically well with good valve function one year after surgery. Reconstructive mitral valve surgery may be preferable for the surgical management of mitral regurgitation in active lupus valvulitis since it eliminates the need for anticoagulation and avoids the disadvantages of different types of prostheses in these young patients who are under prolonged steroid therapy and have usually associated renal failure.
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PMID:Mitral valve repair in lupus valvulitis--report of a case and review of the literature. 855 85

A 37-year-old man with systemic lupus erythematosus, who underwent an aortic valve replacement with a Carpentier-Edwards porcine valve for severe aortic insufficiency, was admitted to the hospital with pulmonary edema. Transesophageal echocardiography revealed severe aortic insufficiency arising from partial dehiscence of the valve sewing ring, as well as centrally from the valve cusp. In addition, marked thickening of the mitral valve was observed with severe eccentric regurgitation. At surgery, valvulitis of the native mitral and bioprosthetic aortic valves was demonstrated, with a perforation of the porcine valve cusp. After replacement of both valves, the patient had a stormy postoperative course with recurrent communications between the left ventricle and atrium requiring multiple surgeries and eventually died. This case illustrates the severity of valvulopathy and ensuing complications that can affect patients with systemic lupus erythematosus and demonstrates that the valvulopathy can affect bioprosthetic valves, a finding that has significant implications as to the type of valve replacement in these patients.
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PMID:Valvulitis involving a bioprosthetic valve in a patient with systemic lupus erythematosus. 867 31

For many years, valvulitis in systemic lupus erythematosus has been known to occur. Our patient was a 17-year-old girl who presented with severe mitral incompetence and renal insufficiency due to lupus valvulitis. The patient was first treated by mitral valve repair, but follow-up disclosed precocious calcification of the valve, necessitating mitral valve replacement with a cryopreserved homograft. At follow-up after 1 year, echocardiography has shown the valve to be functioning normally. A reconstructive mitral valve operation would seem to be preferable. However, a conservative operation does not alter the natural history of the disease and the progression of valve thickening and calcification.
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PMID:Systemic lupus erythematosus valvulitis: mitral valve replacement with a homograft. 878 90

Valvular abnormalities develop in 36% and 35% of patients with primary antiphospholipid syndrome (PAPS) and with systemic lupus erythematosus (SLE) respectively, and in 48% of patients with SLE and antiphospholipid antibodies (aPL). Valvulopathy includes leaflet thickening, vegetations, regurgitation, and stenosis. A literature survey shows that significant morbidity from valvular dysfunction, mostly mitral regurgitation leading to congestive heart failure, occurs in 4% and 6% of SLE and PAPS patients, respectively. The pathogenesis of valvulopathy may involve interaction of aPL with antigens on the valve surface, resulting in valvulitis. Current therapy includes symptomatic measures and valve replacement. A novel approach for symptomatic antiphospholipid syndrome (APS) related valvulopathy involves treatment with systemic corticosteroid. We describe four such patients and their dramatic clinical and hemodynamic response to treatment with prednisone when symptomatic measures failed.
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PMID:Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment. 928 87

Three patients, aged five to 16 years, developed chorea as the only or main clinical manifestation of primary antiphospholipid syndrome. In two cases, complaints were self-limited five to eight months after onset. In one patient, the clinical course was complicated by valvulitis. Under corticosteroid treatment, chorea disappeared and cardiac involvement stabilised. Primary antiphospholipid syndrome is a probably under-recognised differential diagnosis of choreatic syndromes in childhood. Assessment of anticardiolipin antibodies and/or lupus anticoagulant should be an obligatory part of the diagnostic work-up of such patients. Early diagnosis of primary antiphospholipid syndrome may improve clinical management and prognosis.
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PMID:Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood. 1083 88


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