Maintenance therapy with subcutaneous immunoglobulin in a patient with immune-mediated neuropathic postural tachycardia syndrome.

ABSTRACT

AIMS: We describe the disease course of a 35-year-old female with an autoimmune mediated neuropathic postural tachycardia syndrome (PoTS), who responded to immunoglobulin therapy and stabilized on maintenance therapy with subcutaneous immunoglobulin (SCIg). METHODS: We provide longitudinal data of clinical scores, tilt-table results and antibody titers. RESULTS: Initial treatment with intravenous immunoglobulin caused infusion-related side-effects whereas SCIg was well tolerated and improved clinical symptoms and quality of life. Clinical improvement correlated with the reduction of serum antibody titers 22 months after first infusion. CONCLUSIONS: These findings suggest that autoimmune-mediated neuropathic PoTS can be treated sufficiently with IVIg whereas SCIg minimizes side-effects.