CNS Vasculitis in Association with Connective Tissue Disorders

The following disorders fall into this category: systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (scleroderma), and Sjogren’s syndrome. SLE is the best recognized for association with CNS vasculitis although alternative mechanisms can be related to coexistent antiphospholipid antibody syndrome, resulting in a hypercoagulable state, as well as potential cardio-embolic events from Libman-Sacks endocarditis. The most common CNS presentation is a multifactorial encephalopathy which can include cognitive impairment with psychosis, seizures, headache, and chorea. The autoimmune-mediated pathology tends to be non-thrombotic in such a presentation but instead is a combination of deposition of immune complexes and vasculitis. SLE is prominently listed in the differential diagnosis of stroke in the young [91]. There can be vascular occlusion with ischemic stroke as well as hemorrhagic insults which can be related to vasculitic mediated aneurysm formation [92]. Involvement of the CNS is relatively common in SLE, but the exact frequency of involvement varies considerably among reported studies [54, 93].

Systemic sclerosis is not commonly associated with CNS vasculitis but is recognized for potential PNS effects. Amaral et al. [94] reported on 180 studies of systemic sclerosis and identified the following pattern of potential CNS involvement: headache in 23.73%, seizures in 13.56%, and cognitive impairment in 8.47% with anxiety/depression also commonly seen.

Rheumatoid arthritis (RA), like lupus, is now a much more indolent disease with advances in immunosuppression. Furthermore, the incidence of RA is reported to be declining [54]. Cerebral infarction can be seen related to vasculitis. Of note, there is the potential for atlantoaxial subluxation with resultant ischemia or compression to the brainstem. It is reported that this is a not uncommon cause of death attributable to RA [95].

Sjogren’s syndrome, also known as keratoconjunctivitis sicca, can be associated with vasculitis of the CNS. It is reported that the vascular insult may be related to the presence of anti-Ro and antineuronal antibodies [54]. There have been reports of both ischemic and hemorrhagic strokes with this disorder. However, the risk appears to be quite small and, as mentioned previously, may be part of an autoimmune overlap such as with coexistent ANCA antibodies [74].

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