Long-lasting clinical and autonomic effects of plasma ganglionic acetylcholine antibodies removal and immunosuppressive therapy in a patient with autoimmune Pure Autonomic Failure

      Anti alfa-3 and alfa-7 ganglionic cholinergic receptors antibodies (anti-AChRAbs) plasma removal acutely improved dysautonomia symptoms in case reports of Pure Autonomic Failure (PAF). It is unknown whether a long lasting treatment based on repetitive anti-AChRAbs removal and combined immunosuppressive therapy might durably improve both cardiovascular autonomic functioning and clinical features. A 50-years-old male affected by PAF with anti-AChRAbs was studied before anti-AChRAbs removal (Baseline) and one (T1), two (T2) and three (T3) years from Baseline. Based on the worsening of symptoms the patient underwent 17 immunoabsorption procedures over the 36 months. Prednisone was administered starting with 50 mg/day and progressively reduced up to 5 mg/die since T2. We evaluated the time course of plasma anti-AChRAbs, the corresponding changes produced by a gravitational stimulus (75° head-up tilt) in heart rate (ΔHR), systolic arterial pressure (ΔSAP), spectral indices of cardiac (ΔLF/HF) and vascular (ΔLFSAP) sympathetic control and plasma norepinephrine (ΔNE). Twelve dysautonomia symptoms were quantified by a visual analogue scale (VAS, score:0 = no-symptom; 10 = highest-intensity) and averaged (total VAS).
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