Massive Bitemporal Hemorrhages in Antineutrophil Cytoplasmic Autoantibodies Vasculitis

ANCA-associated small vessel vasculitides are systemic diseases characterized by inflammation and necrosis of small-sized blood vessels.1 They are characterized by the presence of antineutrophil cytoplasmic antibodies (ANCAs), which primarily target the kidney, lung, skin and peripheral nervous system.2 In rare cases, they can affect the central nervous system (CNS) and cause cerebrovascular events.3 We present a patient with ANCA vasculitis who developed fatal massive bitemporal hemorrhages during the course of immunosuppressive therapy. [...]a recent meta-analysis has evaluated the specificity of elevated c-ANCA levels in the serum at 96.3% (CI [94.1-98.5]),4 which, combined with the high pretest probability of vasculitis (mononeuritis multiplex, acute renal failure, inflammatory anemia, multifocal brain infarcts), made a diagnosis of ANCA vasculitis highly probable even without a gold-standard pathological diagnosis. Figure 1 Axial view of a head CT showing massive bitemporal haemorrhages with significant mass effect.