|ECR 2013 / C-1597|
|Analysis of central nervous system findings on MR imaging in patients with neurolupus|
To study the frequency and the vascular cerebral pattern of central nervous system involvement in patients with neurolupus.
The prevalence of systemic lupus erythematosus (SLE) is up to 150/100.000, depending principally on the gender and race (5-10 times more frequent in women and in Afro-Caribbean and Asian population) [1, 2].
The diagnostic of SLE is based on the American College of Rheumatology (ACR) diagnostic criteria, requiring a minimum of 4 out of 11 .
Neurolupus has a prevalence of 14-90% of all the SLE patients during the entire course of their disease, with a generally accepted average of 30-40%. Also it represents the cause of death in 19% of SLE patients [1, 2].
According to the ACR criteria, neurolupus includes 12 syndromes, divided in neurological (aseptic meningitis, cerebrovascular lesions, demyelinating syndromes, headache, movement disorders, myelopathy, epilepsy) and neuropsichiatrical (acute confusional states, anxiety disorder, cognitive dysfunction, affective disorders) .
The average age of the patients with cerebral infarct due to SLE is 35 years, with a recurrence in 35-60% of the patients associated to the presence of antiphospholypidic antibodies [1, 2].
One of the most debilitating complications of neurolupus is the myelopathy. It has a prevalence of 1-5% of the SLE patients. It usually develops early in the evolution of the disease leading to an unfavorable prognosis. In 39% of the patients with lupus related myelopathy it constitutes the presenting symptom of SLE, and in another 42% occurs during the first 5 years after the diagnostic [2, 3].
Pathology of cerebral lesions in SLE:
- microangiopathic disease (the most frequent neuropathological finding; due to intimal hyperplasia, erythrocytes extravasation and development of fibrin thrombi). It typically produce a multifocal involvement, sometimes with multiples microinfarcts
- macroscopic infarcts (less frequently, principally explained by secondary coagulopathy due to antiphosfolipidic antibodies or by embolic phenomena due to Libman–Sacks endocarditis)
- accelerated atherosclerosis (steroid treatment, vasculitis and microhemorrhages playing a role)
- direct autoimmune neural alteration, demyelination, embolisms [1-3].
In the medical literature there is a lack of studies with large number of neurolupus patients describing the characteristics and prevalence of brain lesions in this population.
The two most frequent MR findings reported in SLE patients were: multiple small lesions (8-70%) and brain atrophy signs (9-67%). They are commonly found in patients with long course disease and in those with neurolupus [2, 3].
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