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.

Long COVID: Association of Functional Autoantibodies against G-Protein-Coupled Receptors with an Impaired Retinal Microcirculation

“…There is evidence that reduction, elimination or neutralization of the GPCR-AAbs goes along with the improvement of clinical features in patients with a seropositivity of those GPCR-AAbs. Either therapeutic plasma exchange (TPE) [57], immunoadsorption [25,58], immunoglobulin therapy (IVIg) [59] or the aptamer BC 007 [12] were observed to improve the patients' symptoms in each clinical entity. It can be assumed that GPCR-AAbs have a harmful impact on diseases with autoimmune impacts (e.g., POTS, ME/CFS, Long COVID).…”

Cardiovascular dysautonomia in postacute sequelae of SARS‐CoV‐2 infection

“…48 However, intravenous immunoglobulin (IVIG) appears poorly tolerated in POTS, although side effects appear to regress after reducing the infusion rate and additional treatment cycles, as well as by premedication with dexamethasone and intravenous hydration. 49,50 Imbalance in sympathetic/parasympathetic nervous system A systematic review of heart rate variability in patients with PASC compared to healthy controls has been compiled. 51 Most studies show persistent PASC symptoms to be associated with reduced standard deviation of the RR intervals, a marker of increased sympathetic activity and reduced vagal tone.…”

“…Patients with POTS, unresponsive to conventional treatment, show improvement in autonomic symptoms after subcutaneous immunoglobulin or plasmapheresis, with 85% of patients able to reduce or discontinue oral medications for POTS 48 . However, intravenous immunoglobulin (IVIG) appears poorly tolerated in POTS, although side effects appear to regress after reducing the infusion rate and additional treatment cycles, as well as by premedication with dexamethasone and intravenous hydration 49,50 …”

Small fiber neuropathy

“…In a 35-year-old woman with autoimmune-mediated, neurogenic POTS, intravenous, respectively, subcutaneous immunoglobulins reduced symptoms and improved quality of life. 76 In a study of 40 patients with cryptogenic SFN, 55% had either antibodies against the TS-HDS or the FGFP-3 receptor. 48 Eight of the seropositive patients improved on IVIGs, with a 42% reduction in pain scores (p = 0.02), a 44% reduction in the Utah Neuropathy Score, and improved IENFD post-treatment.…”

“…Immunoglobulins have been successfully administered in several types of immune‐mediated SFN, either in monotherapy or in combination with immunosuppressive drugs, respectively, pain killers. In a 35‐year‐old woman with autoimmune‐mediated, neurogenic POTS, intravenous, respectively, subcutaneous immunoglobulins reduced symptoms and improved quality of life 76 . In a study of 40 patients with cryptogenic SFN, 55% had either antibodies against the TS‐HDS or the FGFP‐3 receptor 48 .…”