Valvular heart disease is common in patients with systemic lupus erythematosus, frequently changes over time, appears to be temporally unrelated to other clinical features of lupus, and is associated with substantial morbidity and mortality.

Aortic root disease and valve disease are common in patients with AKS, are unrelated to clinical features of AKS, can resolve or progress over time and are associated with clinically important cardiovascular morbidity.

To compare the diagnostic value of transesophageal and transthoracic echocardiography in infective endocarditis, paired transesophageal and transthoracic echocardiograms were obtained prospectively for 66 episodes of suspected endocarditis in 62 patients. Echocardiographic results were compared with the presence or absence of endocarditis determined by pathologic or nonechocardiographic data from the subsequent clinical course. All echocardiograms were interpreted by an observer told only that the studies were from patients in whom the diagnosis of endocarditis was suspected. The diagnosis of endocarditis was eventually made in 16 of the 66 episodes of suspected endocarditis (14 by pathologic and 2 by clinical criteria). In 7 of 16 transthoracic and 15 of 16 transesophageal echocardiograms, endocarditis was diagnosed at a probability level of "almost certain," giving a sensitivity of 44% and 94%, respectively (p less than 0.01). For the remaining episodes, 49 of 50 transthoracic and all transesophageal studies yielded normal results, giving a specificity of 98% and 100%, respectively. This study suggests that transesophageal echocardiography is highly sensitive and specific for the diagnosis of infective endocarditis and significantly more sensitive than transthoracic echocardiography. Although echocardiography cannot rule out endocarditis, the high diagnostic sensitivity of transesophageal echocardiography results in a low probability of the disease when the study yields negative results in a patient with an intermediate likelihood of the disease.

Objective Magnetic resonance imaging (MRI) often demonstrates brain lesions in neuropsychiatric systemic lupus erythematosus (NPSL). The present study compared post-mortem histopathology with pre-mortem MRI in NPSL. Methods 200 subjects with NPSLE were studied prospectively with MRI over a 10-year period during which 22 subjects died. In 14 subjects, a brain autopsy with histopathology that permitted direct comparison with pre mortem MRI was successfully obtained. Surface anatomy was used to determine the approximate location of individual lesions. Results Pre mortem MRI findings in fatal NPSLE were small focal white matter lesions (100%), cortical atrophy (64%), ventricular dilation (57%), cerebral edema (50%), diffuse white matter abnormalities (43%), focal atrophy (36%), cerebral infarction (29%), acute leukoencephalopathy (25%), intracranial hemorrhage (21%), and calcifications (7%). Microscopic findings in fatal NPSLE included global ischemic changes (57%), parenchymal edema (50%), microhemorrhages (43%), glial hyperplasia (43%), diffuse neuronal/axonal loss (36%), resolved cerebral infarction (33%), microthomboemboli (29%), blood vessel remodeling (29%), acute cerebral infarction (14%), acute macrohemorrhages (14%), and resolved intracranial hemorrhages (7%). Cortical atrophy and ventricular dilation seen by MRI predicted brain mass at autopsy (r = -0.72, p = 0.01, and r = -0.77, p =0.01, respectively). Cerebral autopsy findings, including infarction, cerebral edema, intracranial hemorrhage, calcifications, cysts, and focal atrophy were also predicted accurately by pre mortem MRI. Conclusion Brain lesions in NPSLE detected by MRI accurately represent serious underlying cerebrovascular and parenchymal brain injury on pathology.

Objective To determine whether Libman-Sacks endocarditis is a pathogenic factor for cerebrovascular disease (CVD) in systemic lupus erythematosus (SLE). Background A cardioembolic pathogenesis of SLE CVD manifested as 1) neuropsychiatric SLE (NPSLE) including stroke and transient ischemic attacks (TIA), 2) neurocognitive dysfunction, and 3) MRI focal brain lesions has not been established. Methods A 6-year study of 30 patients with acute NPSLE (27 women, age 38±12 years), 46 age-and-sex matched SLE controls without NPSLE (42 women, age 36±12 years), and 26 age-and-sex matched healthy controls (22 women, age 34±11 years) who underwent clinical and laboratory evaluations, TEE, carotid duplex, transcranial Doppler, neurocognitive testing, and brain MRI/MRA. NPSLE patients were re-evaluated after 4.5 months of therapy. All patients were followed clinically for a median of 52 months. Results Libman-Sacks vegetations (87%), cerebromicroembolism (27% with 2.5 times more events per hour), neurocognitive dysfunction (60%), and cerebral infarcts (47%) were more common in NPSLE than in SLE (28%, 20%, 33%, and 0%) and healthy controls (8%, 0%, 4%, and 0%, respectively) (all p≤0.009). Patients with vegetations had 3 times more cerebromicroemboli per hour, lower cerebral blood flow, more stroke/TIA and overall NPSLE events, neurocognitive dysfunction, cerebral infarcts, and brain lesion load than those without (all p≤0.01). Libman-Sacks vegetations were independent risk factors of NPSLE (OR=13.4, p<0.001), neurocognitive dysfunction (OR=8.0, p=0.01), brain lesions (OR=5.6, p=0.004), and all 3 outcomes combined (OR=7.5, p<0.001). Follow-up re-evaluations in 18 (78%) of 23 surviving NPSLE patients demonstrated improvement of vegetations, microembolism, brain perfusion, neurocognitive dysfunction, and lesion load (all p≤0.04). Finally, patients with vegetations had reduced event free survival time to stroke/TIA, cognitive disability, or death (p=0.007). Conclusion The presence of Libman-Sacks endocarditis in patients with SLE is associated with higher risk for embolic CVD. This suggests that Libman-Sacks endocarditis may be a source of cerebral emboli.

Valve excrescences are common on the left-sided heart valves of normal subjects and patients regardless of gender and age; they persist unchanged over time and do not appear to be a primary source of cardioembolism.

Lupus erythematosus valve disease is frequent (74%) regardless of the presence or absence of antiphospholipid antibodies. Therefore antiphospholipid antibodies may not be a primary pathogenetic factor. The characteristic appearance of leaflet thickening and masses in patients with lupus erythematosus may be unique.

BackgroundNeuropsychiatric systemic lupus erythematosus (NPSLE) is associated with increased morbidity and mortality.MethodsWe used Diffusion Tensor Imaging (DTI) to assess white matter abnormalities in seventeen NPSLE patients, sixteen SLE patients without NPSLE, and twenty age- and gender-matched controls.ResultsNPSLE patients differed significantly from SLE and control patients in white matter integrity of the body of the corpus callosum, the left arm of the forceps major and the left anterior corona radiata.ConclusionsSeveral possible mechanisms of white matter injury are explored, including vascular injury, medication effects, and platelet or fibrin macro- or microembolism from Libman-Sacks endocarditis.