Highly efficacious, long-term, triglyceride lowering with rituximab therapy in a patient with autoimmune hypertriglyceridemia.

We report the first case of an autoimmune hypertriglyceridemia successfully treated with rituximab, an anti-CD20-targeted monoclonal antibody. A 45-year-old man, with prior autoimmune conditions, developed severe, acquired hypertriglyceridemia resistant to traditional triglyceride lowering therapies. After the elimination of secondary or genetic causes, we detected the presence of anti-LPL-IgG by immunoblot. After 3 infusions of rituximab, we observed a marked improvement of his hypertriglyceridemia, concomitant with a reduction in plasma anti-LPL antibody titer and B-lymphocytes counts. The patient has been receiving rituximab maintenance therapy for 5 years without any serious adverse events and with excellent control of his previous, marked hypertriglyceridemia.