Peripheral sensorimotor and autonomic neuropathy associated with systemic lupus erythematosus: Clinical, pathological and immunological features

The clinical features and pathological findings in the sural nerves are described of 7 patients with peripheral neuropathy; in 4 cases the criteria for diagnosis of systemic lupus erythematosus (SLE) were satisfied and in 3 other cases there was serological evidence of an undifferentiated connective tissue disease, most probably SLE. The peripheral neuropathy was of a chronic sensorimotor type with predominantly sensory features and gradual onset. In 2 cases the presentation was asymmetric. One patient had autonomic dysfunction. The pathological findings in the biopsied sural nerves were those of axonal degeneration and vasculitis. In 6 nerves there was increased expression of Class II (Ia) antigen within the nerve fascicle, perineurium and within endothelial cells.