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Query: UMLS:C0409974 (lupus)
 22,386 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

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

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

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

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

The Committee reviewed cardiac involvement in the antiphospholipid antibody syndrome. The Committee's recommendations are: Valve abnormalities: anticoagulation is recommended for symptomatic patients with valvulopathy. Prophylactic antiplatelet therapy may be appropriate for asymptomatic patients (recommended by 13/17 experts in an independent review). Committee members disagreed whether corticosteroid therapy is helpful, but agree that distinguishing among presumptive valvulitis (valve thickening on echocardiogram), valve deformity and vegetations is important, as treatment implications may differ. Occlusive arterial disease (angina, myocardial infarction): the Committee recommends aggressive treatment of all risk factors for atherosclerosis (hypertension, hypercholesterolaemia, smoking) and liberal use of folic acid, B vitamins and cholesterol-lowering drugs (preferably statins). Hydroxychloroquine for cardiac protection in APS patients may be considered. The Committee also recommends warfarin anticoagulation for those who have suffered thrombosis in the absence of atherosclerosis, but recognizes that developing data may support the use of antiplatelet agents instead. Intracardiac thrombi: the Committee recommends intensive warfarin anticoagulation, and consultation with cardiac surgeons when appropriate. Ventricular dysfunction: the Committee has no recommendations on this aspect of cardiac disease. Pulmonary hypertension: the Committee recommends intensive anticoagulation with warfarin and clinical trials of bosentan, epoprostenol and other new agents.
Lupus 2003
PMID:Cardiac disease in the antiphospholipid syndrome: recommendations for treatment. Committee consensus report. 1289 91

Hemodynamically significant lupus valvulitis, requiring valve replacement, is rare: 21 cases have been reported so far in the literature, and only 2 of these have involved double valve replacement. We describe an additional case of double valve replacement in a patient with systemic lupus erythematosus. The histopathologic and clinical features of this case suggest that valvular involvement resulted from both acute and chronic disease processes. Medical success in the treatment of systemic lupus erythematosus, especially that achieved through prolonged or high-dose steroid therapy, may cause chronic valvular disease to become a more common surgical problem. A review of the literature supports this contention.
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PMID:Double valve replacement for lupus valvulitis: report of a case and review of the literature. 1522 91

The heart can be involved in vasculitides but the frequency of its involvement and the manifestations vary according to the vasculitis. Cardiovascular manifestations include cardiomyopathy (specific or resulting from myocardial infarctions), coronary arteritis (with risk of aneurysms, stenoses and thrombosis formation or rupture), pericarditis, valvulitis, conduction-tissue involvement (with heart blocks), arrhythmias (mainly supraventricular) and/or dissection of the aorta (and/or its proximal branches). As many of these manifestations are clinically silent, at least during their early stages, heart function should be systematically assessed in vasculitis patients, with at least ECG and echocardiography, and more invasive exploratory procedures when the former reveal abnormalities or symptoms become manifest. Specific cardiomyopathy has been identified as a factor of poor outcome in small and medium-sized vessel vasculitides (five-factor score). Therefore, in addition to symptomatic treatments, prescription of corticosteroids and immunosuppressants (mainly cyclophosphamide) is considered mandatory. This regimen has dramatically improved the overall prognosis of affected patients.
Lupus 2005
PMID:Cardiac involvement in small and medium-sized vessel vasculitides. 1621 74

Cardiac involvement is fairly common in patients with systemic lupus erythematosus (SLE). It may involve all layers of the heart and coronary arteries as well as the heart valves. We report an extremely rare presentation of valvulitis and valvular dysfunction associated with systemic lupus erythematosus. This is the first case of lupus valvulitis which required three mechanical prosthetic valve replacements with disease recurrence leading to a fatal outcome. This is, in our point of view, the consequence of aggressive natural history of the disease and perhaps late diagnosis and treatment of underlying SLE which was unsuccessful.
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PMID:Triple Valvulopathy and Jaccoud's Arthropathy: A Case Report and Literature Review. 2743 66


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