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.
Keywords: Autoimmunity; Neuropathic postural tachycardia syndrome; Small fiber neuropathy; Subcutaneous immunoglobulin.
© 2021 The Authors.