Autoimmune postural orthostatic tachycardia syndrome

Abstract

The aim of this study was to evaluate the association between postural orthostatic tachycardia syndrome (POTS) and circulating antiganglionic acetylcholine receptor (gAChR) antibodies. We reviewed clinical assessments of Japanese patients with POTS, and determined the presence of gAChR antibodies in serum samples from those patients. Luciferase immunoprecipitation systems detected anti‐gAChR α3 and β4 antibodies in the sera from POTS (29%). Antecedent infections were frequently reported in patients in POTS patients. Moreover, autoimmune markers and comorbid autoimmune diseases were also frequent in seropositive POTS patients. Anti‐gAChR antibodies were detectable in significant number of patients with POTS, and POTS entailed the element of autoimmune basis.

Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management

“…Importantly, symptoms of orthostatic intolerance and number of syncopal events do not well discriminate between POTS and other syncope‐related dysautonomic conditions, orthostatic hypotension and recurrent vasovagal syncope . The onset of POTS may be precipitated by typical immunological stressors such as viral infection, frequently of the upper respiratory or gastrointestinal tract, vaccination, trauma, pregnancy, surgery or even a period of intensive psychosocial stress . The antecedent history of suspected viral infection is reported by between 20% and 50% of all patients .…”

“…The onset of POTS may be precipitated by typical immunological stressors such as viral infection, frequently of the upper respiratory or gastrointestinal tract, vaccination, trauma, pregnancy, surgery or even a period of intensive psychosocial stress . The antecedent history of suspected viral infection is reported by between 20% and 50% of all patients . However, a substantial number of POTS patients do not report any triggering event and experience rather slowly progressing or even stationary symptoms over a long period of time .…”

“…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.…”

“…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. A brief summary of autoimmune activity found in different POTS cohorts is presented in Table .…”

Association of small-fiber polyneuropathy with three previously unassociated rare missense SCN9A variants

“…22 Other iiSFN appears to develop as part of ill-defined systemic dysimmune/inflammatory conditions, with selective immunoglobulin deficiencies particularly common, 1 and reports have associated autoantibodies with dysautonomic SFN symptoms including POTS. 23,24 In a small passive transfer study that injected sera from patients with "idiopathic" painful small fiber polyneuropathy to reproduce symptoms, physiology and pathology in mice provided direct support of the hypothesis that some cases of iiSFN are B cell mediated. 25 However, the largest pool of evidence comes from the many cases and case series reporting efficacy of two immunotherapies effective for large fiber immune neuropathies: corticosteroids and/or pooled intravenous immunoglobulin (IVIg) for iiSFN.…”