Neurological involvement of relapsing polychondritis in Japan: An epidemiological study

Abstract

We conducted a large scale epidemiological study in Japan and revealed a high mortality rate in RP patients with neurological involvement. Japanese RP patients developed encephalitis/meningitis (12 out of 239 cases, 5.0%), cerebral infarct/bleeding (5 cases, 2.1%) and cerebral vasculitis (4 cases, 1.7%). The mortality rate was 18%, in contrast to 8.1% of RP patients without neurological involvement. We suggested that neurological involvement appeared to be a major determinant of disease severity in patients with RP.