Longitudinally extensive transverse myelitis with neuro-lymphatic protein expressionin systemic lupus erithematosus patient. An autopsy case report

Abstract

Abstract Acute transverse myelitis (inflammation across one or more segments of spinal cord) is a rare complication of systemic lupus erythematosus (SLE) although its frequency is greater than in the general population. Even less common is longitudinal extensive transverse myelitis (LETM), (inflammation affects three or more vertebral segments). The pathogenesis of LETM is unclear and the management uncertain. We present a case of a 34-year-old woman with SLE and LETM of the whole spine, with rapid progression despite intensive treatment. Autopsy revealed a spine with liquefactive necrosis; some vessels showed fibrinoid necrosis and there were thrombi and an infiltration of lymphocytes and neutrophils in both the grey and white matter. Histological examination of brain revealed necrosis and oedema in the cortex and around the lateral ventricles. The immunohistochemistry showed CD3-positive T-lymphocytes in the wall of the spinal blood vessels, and a prominent D2-40 immunostaining, mainly localized at perivascular inflammatory regions.