Background Vasculitis of the cerebral arterioles and small arteries can lead to local ischaemia and to regressive changes in the brain. This process is more or less widespread and multifocal, depending on the number of involved vessels and is followed by phagocytosis and/or fibrosis, depending on the stages of the pathological process. Because of the recurrent nature of vasculitis with time these regressive changes accumulate within the brain and may cause or rather may contribute to an unexpected sudden death of the patient (1).
Objectives The aim of this study was to determine: the prevalence, and histological characteristics of vasculitis in the brain, and to follow the formal pathogenesis of multifocal brain necrosis due to arteritis and/or arteriolitis in rheumatoid arthritis (RA) patients complicated with systemic vasculitis (SV).
Methods A randomized autopsy population of 161 in-patients with RA was studied. SV complicated RA in 36 (22.36%) of 161 cases; tissue samples of brain were available for histologic evaluation in 35 of 36 patients with vasculitis.
Vasculitis and consecutive focal liquefaction and/or gliosis (glia scars) were characterized histologically and immunohistochemically.
Results Arteritis and/or arteriolitis were observed in the brain in 3 of 35 cases (1.86% of 161, 8.57% of 35).
Only non-specific vasculitis was observed; fibrinoid necrotic and granulomatous types of vasculitis (in contrast with other organs) were not found in the brain.
Only intracerebral arterioles and small arteries were involved, with varying incidence and severity of vasculitis, the accompanying venules and small veins were not.
Acute, subacute-subchronic and chronic stages of vasculitis were recognized histologically. Different stages of inflammation existed simultaneously in different vessels or combined in the same vessel.
The inflammatory infiltrates of vasculitis were dominated by CD3 T lymphocytes.
Arteritis and/or arteriolitis were followed by multifocal liquefied brain necrosis which led to death in 2 of 3 cases.
Vasculitis of the brain and vasculogenic, multifocal brain necrosis was not recognized clinically.
Conclusions Cerebral manifestations of systemic autoimmune vasculitis are relatively uncommon in RA. Cerebral arteritis and/or arteriolitis was rare (8.57 % of 35 RA patients with SV) and mild in our autopsy population but, because of its localization, it was often lethal. Cerebral vasculitis with multifocal brain necrosis led to death in 2 of 3 patients (in one case due to bronchopneumonia and in the second one due to femoral vein thrombosis, pulmonary embolism and septic infarction of the lung).
Because of the recurrent nature of autoimmune vasculitis the regressive changes accumulate within the brain and may lead to sudden death of the patient. Corresponding to the vascular lesions, multiple microscopic foci of necrosis exist side by side in different stages of liquefaction as hypodense, patchy changes on CT scan.
Vasculogenic microinfarcts in the brain may be clinically characterized by unexplained recurrent cerebral symptoms and spontaneous remissions which insidiously may lead to death (2).
Bély M: Krankheitsmodifizierende Faktoren bei chronischer Polyarthritis. D.Sc. Thesis, Budapest 1993.
Kiss G, Kelemen J, Bély M, Vértes P: Clinically diagnosed fatal cerebral vasculitis in long-standing juvenile rheumatoid arthritis. Virchows Arch 2005;448:381-383
Disclosure of Interest None Declared