Disseminated perivenous necrotizing encephalomyelitis in systemic lupus erythematosus: report of an autopsy case

The patient, a 22-year-old woman who had been treated for systemic lupus erythematosus (SLE) for 10 years, was hospitalized for arthralgia, melena, and difficulty in walking. CT examination of the brain showed grain-like high-density lesions scattered throughout the cerebral white matter and basal ganglia. At autopsy, multiple perivenous, well-demarcated foci of brownish discoloration were seen scattered throughout the cerebral white matter and basal ganglia. Histopathologically these lesions consisted of foci of coagulation necrosis surrounding the veins. The veins in the foci showed fibrous thickening of the walls, but there were no indications of vasculitis. At the periphery of the lesions, the axons were better preserved than their myelin sheaths. The neuropathological findings in the present case closely resemble those of acute disseminated (perivenous) encephalomyelitis, although an inflammatory cell infiltration had apparently already subsided. Although its pathogenesis remains unclear, this finding should not be regarded as an incidental complication but rather as a rare subtype of central nervous system lesion occurring with SLE.