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We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (GBM) disease accompanied by systemic lupus erythematosus (SLE) presenting central nervous system involvement in a 32-year-old Japanese male. He was admitted to our hospital because of a 3-week history of fever and rapidly failing renal function requiring hemodialysis (HD). Laboratory tests showed anti-GBM antibody elevation with a value of 16,385 units/ml. On day 85, he had generalized tonic–clonic seizure. Brain magnetic resonance T2 Flair imaging showed multiple high intensity lesions in a broad area. We made a diagnosis of central nervous system involvement in SLE based on positivity for antinuclear and anti-DNA antibodies, hypocomplementemia, and discoid skin rash. After combined therapy consisting of plasma exchange, HD, and steroid pulse, the patient made a good …