Primary central nervous system vasculitis (PCNSV) represents a very rare and poorly understood disease. It is an autoimmune mediated disease affecting medium- to small-sized vessels only of the central nervous system. Clinical presentation is nonspecific and diagnosis difficult and invasive. The intent of this study was to analyse clinical features, diagnostic findings and genetic factors predictive or protective for PCNSV.
In 36,2% of suspected PCNSV the diagnosis was confirmed by predetermined diagnostic criteria based on biopsy (72%) or angiography (28%). 63,8% of the patients turned out to have other diagnoses. Clinical presentation was not able to discriminate between PCNSV and its differential diagnoses. However, a worse clinical outcome was associated with PCNSV (p = 0.005). Diagnostic results were congruent with previous data reported on PCNSV. Biopsy (p = 0.004), contrast enhancement (p = 0.000) or tumor-like mass lesion (p = 0.008) in magnetic resonance imaging (MRI), intrathecal IgG increase (p = 0.020), normal Duplex findings of cerebral arteries (p = 0.022) and conventional angiography (p = 0.010) were able to distinguish between the two cohorts statistically significant.
HLA frequencies within the PCNSV patient group versus general population could clearly show higher frequencies for the HLA-A*69 (p = 0.002) and the HLA-B*49 (p = 0.027) alleles in PCNSV. For the HLA-B*49 and DRB1*03 alleles a trend to a higher frequency in PCNSV could be observed, whereas a lower frequency for HLA-DRB1*01 in PCNSV might play a role as a protective factor. It remains to be seen whether HLA analysis will constitute an additional diagnostic tool in PCNSV in the future.
An investigation in a larger, multicentric cohort using uniform diagnostic standards is required, since our findings are limited by the small sample size.
|Advisor:||Kraemer , Markus|
|School:||Universitaet Duisburg-Essen (Germany)|
|Source:||DAI-C 81/1(E), Dissertation Abstracts International|
|Keywords:||Central nervous system vasculitis|
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