Autoimmunity in postural orthostatic tachycardia syndrome: Current understanding

Abstract

There is growing interest in the role of autoimmunity in postural orthostatic tachycardia syndrome (POTS). In recent years, investigators have described an increased rate of co-morbid autoimmune disease, and the presence of several neural receptor autoantibodies and non-specific autoimmune markers in POTS. Case reports on the efficacy of immunotherapy in highly selected POTS patients continue to appear in the literature, while no prospective clinical trials have occurred to date. This article summarizes the current state of knowledge on the role of autoimmunity in POTS, the clinical implications of these findings, and prospects for future research.

Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management

“…In recent years, search for the presence of a hypothetical immunological fingerprint in POTS has resulted in a discovery of diverse autoantibodies with a proposed causative role in the syndrome . A specific focus was given cardiovascular G‐protein‐coupled membrane complexes, such as adrenergic, muscarinic and angiotensin II type‐1 receptors The symptoms of POTS, especially tachycardia, could be evoked by the direct action on sinus rate controlling system (via adrenergic and muscarinic receptors) or through a compensatory mechanism responding to peripheral vasodilation (via adrenergic, angiotensin and other possible vasoactive receptors) (Fig.…”

“…This attractive hypothesis awaits verification in larger and independent patient populations and well‐selected control groups. An interested reader is recommended to consult some of recently published reviews on the subject . Other autoantibodies with a proposed aetiological role in POTS include antibodies against nicotinic acetylcholine‐receptor in autonomic ganglia and Sjögren autoantibodies , detected in POTS through antibody screening programmes.…”

How We Treat Autoimmune Small Fiber Polyneuropathy with Immunoglobulin Therapy

“…In some patients, dysautonomia may occur in association with primary antineuronal autoimmunity, including antibodies to the adrenergic and muscarinic receptors, ion channels, the ganglionic acetylcholine receptor, the NMDA receptor, antibodies against the N-type or P/Q-type voltage gated calcium channels (Lambert-Eaton Syndrome), and others [3]. In other patients, it may occur in the context of systemic autoimmune disease [3,4], and autonomic neuropathy may be the initial manifestation of Sjogren syndrome [5] or the antiphospholipid syndrome [6], which may coexist. Dysautonomia may also occur in association with most other autoimmune diseases, including some cases of Hashimoto thyroiditis [4], rheumatoid arthritis, [7] spondyloarthropathy [8], lupus [9], systemic sclerosis [10], celiac disease [11], inflammatory bowel disease [12], myasthenia gravis [13], and multiple sclerosis [1].…”

“…SFPN may manifest clinically as postural tachycardia syndrome, orthostatic intolerance, orthostatic hypotension, inappropriate sinus tachycardia, gastrointestinal dysmotility, complex regional pain syndrome, and/or neurogenic bladder [2]. In some patients, dysautonomia may occur in association with primary antineuronal autoimmunity, including antibodies to the adrenergic and muscarinic receptors, ion channels, the ganglionic acetylcholine receptor, the NMDA receptor, antibodies against the N-type or P/Q-type voltage gated calcium channels (Lambert-Eaton Syndrome), and others [3]. In other patients, it may occur in the context of systemic autoimmune disease [3,4], and autonomic neuropathy may be the initial manifestation of Sjogren syndrome [5] or the antiphospholipid syndrome [6], which may coexist.…”

Is postural tachycardia syndrome an autoimmune disorder? And other updates on recent autonomic research

“…However, some patients with POTS also tested positive with low titers [13]. Autonomic experts now agree that positivity for ganglionic nicotinic antibodies at low titer (i.e., below 0.2 nmol/L) has no clinical relevance for POTS, as these can be detected in up to 5% of healthy controls [9,12,14].…”