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)

Hemodynamically significant valvular lesions have been rarely reported sequelae of Libman-Sacks endocarditis complicating systemic lupus erythematosus (SLE). Furthermore, embolic phenomena associated with these vegetations have not been clearly documented. We present a report of critical aortic stenosis associated with SLE in a patient who had received corticosteroid treatment for several years. An embolus, histologically identical with the aortic valve vegetation, was found in the left anterior descending artery at necropsy. There was no evidence of rheumatic heart disease, bacterial endocarditis or a bicuspid aortic valve. Recent reports suggest an increased incidence of significant valvular dysfunction in patients with SLE who have received long-term corticosteroid treatment.
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PMID:Aortic stenosis associated with systemic lupus erythematosus. 646 59

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discusses stenotic aortic valves and Part II will discuss causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, or rheumatic. Other rare causes of stenotic aortic valves include active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's, dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and diseases affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal defect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing pure aortic valve regurgitation.
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PMID:Pathology of aortic valve stenosis and pure aortic regurgitation. A clinical morphologic assessment--Part I. 816 31

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discussed stenotic aortic valves and Part II discusses causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, and rheumatic. Other rare causes included active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and disease affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal defect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing purely regurgitant aortic valves.
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PMID:Pathology of aortic valve stenosis and pure aortic regurgitation: a clinical morphologic assessment--Part II. 816 82

A 46-year-old woman was hospitalized for aortic valve stenosis (AS) associated with systemic lupus erythematosus (SLE) on April 12, 1996. She had a syncopal episode six months before admission. She was found to have thrombocytopenia, and was diagnosed with SLE by further examination. Irregular genital bleeding was also seen on admission while her SLE was being controlled with steroid therapy. Aortic valve replacement was performed after the steroids had been reduced to avoid excessive bleeding. The aortic valve was the bicuspid with raphe. There was much calcification on the cusps and the annulus, but there were no degenerative changes. The postoperative course was uneventful, and her SLE has been in remission two years after the operation. The management of steroid therapy for SLE patients complicated with cardiovascular disease is discussed.
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PMID:[A surgical case of aortic valve stenosis associated with systemic lupus erythematosus]. 921 96

The etiology of valvular heart diseases (VHD) has changed in the last 50 years in the industrialized countries. A significant reduction in the incidence of rheumatic fever and its sequelae, increase in life expectancy, recognition of new causes of VHD and advancement in technology are responsible for the metamorphosis of the etiology of VHD. Heritable disorders of connective tissue (marfan syndrome, Ehlers-Danlos syndrome, adult polycystic kidney disease, floppy mitral valve/mitral valve prolapse); congenital heart disease (bicuspid aortic valve); inflammatory/immunologic disorders (rheumatic fever, AIDS, Kawasaki disease, syphilis, seronegative spondyloarthropathies, systemic lupus erythematosus, antiphospholipid syndrome); endocardial disorders (nonbacteremic thrombotic endocarditis, infective endocarditis, endomyocardial fibroelastosis); myocardial dysfunction (ischemic heart disease, dilated cardiomyopathy, hypertrophic cardiomyopathy); diseases and disorders of other organs (chronic renal failure, carcinoid heart disease); aging (calcific aortic stenosis, mitral annular calcification); postinterventional valvular disease; drugs and physical agents are all clinical entities associated with VHD. It should be emphasized that VHDs still constitute a major health problem which will increase with the aging population.
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PMID:Etiology of valvular heart disease. 1503 Feb 51

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

The bicuspid aortic valve (BAV) and specific systemic autoimmune diseases are associated with cardiovascular manifestation, including aortic aneurysm. We reported a case of 64 year-old patient with BAV and a history of ankylosing spondylitis (AS) and systemic lupus erythematosus (SLE), and who developed ascending thoracic aortic aneurysm. The patient presented also the homozygosity for genetic variants of MMP9, ACE, MTHFR and PAI-1 genes. Gene-environmental interactions may represent an additional pathogenetic dimension in the still challenging management of the abnormalities of the aortic wall, including dilatation, aneurysm and dissection.
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PMID:Ascending aortic aneurysm in a patient with bicuspid aortic valve, positive history of systemic autoimmune diseases and common genetic factors: a case report. 1958 Jun 62

Non-bacterial thrombotic endocarditis (NBTE) is characterized by presence of sterile vegetations that develop from fibrin and platelets on heart valves. The main conditions predisposing to NBTE are malignancy, autoimmune diseases and other hypercoagulable states. The authors describe a case of a 25-year-old male, in whom NBTE was diagnosed on the bicuspid aortic valve. The presence of significant aortic regurgitation and dental caries were initially suggestive of infective endocarditis; although, serial blood culture were negative and procalcytonin concentration was within normal ranges. Empiric antibiotic therapy did not result in diminishing of vegetations, similarly to the anticoagulation treatment initiated when strongly positive lupus anticoagulant was detected in laboratory findings. Aortic valve replacement was necessary. Bacteriologic examination of the excised valve was negative. Widespread fibrin masses at different stages of organization on the leaflets confirmed NBTE in histopathologic assessment. Lupus anticoagulant was probably secondary to thyroid autoimmune disease.
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PMID:[Non-bacterial thrombotic endocarditis on the bicuspid aortic valve in a 25-year-old male with lupus anticoagulant]. 2708 1

Background: Alagille syndrome (AGS) is an autosomal-dominant, multisystem disorder caused by mutations in the JAG1 gene. Case Description: A 34-year-old man was referred to our service 10 years ago with focal seizures with impaired awareness and transient slurred speech. He had a 5-year history of intermittent left monocular low-flow retinopathy. He has a family history of AGS. General examination revealed mild hypertension, aortic regurgitation, and livedo reticularis. Neurological examination was normal. Investigations: He had mild hyperlipidaemia and persistently-positive lupus anticoagulant consistent with primary anti-phospholipid syndrome. Color Doppler ultrasound revealed low velocity flow in a narrowed extracranial left internal carotid artery (ICA). MR and CT angiography revealed a diffusely narrowed extracranial and intracranial left ICA. Formal cerebral angiography confirmed severe left ICA narrowing consistent with a left ICA "vasculopathy" and moyamoya phenomenon. Transthoracic echocardiogram revealed a bicuspid aortic valve and aortic incompetence. Molecular genetic analysis identified a missense mutation (A211P) in exon 4 of the JAG1 gene, consistent with AGS. Discussion: AGS should be considered in young adults with TIAs/stroke and unexplained extracranial or intracranial vascular abnormalities, and/or moyamoya phenomenon, even in the absence of other typical phenotypic features. Gene panels should include JAG1 gene testing in similar patients.
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PMID:Extracranial and Intracranial Vasculopathy With "Moyamoya Phenomenon" in Association With Alagille Syndrome. 3076 Oct 79