Long-term cardiovascular autonomic and clinical changes after immunoglobulin G immunoadsorption therapy in autoimmune autonomic ganglionopathy.
: A 63-year-old male was diagnosed with autoimmune autonomic ganglionopathy based on the finding of plasma antibodies to the nicotinic acetylcholine receptor (nAChR) of autonomic ganglia. He complained of mouth and eye dryness, dysphagia, severe constipation, erectile dysfunction, urgency, frequent urination, habitual orthostatic syncope and presyncope. A remarkable symptomatic orthostatic hypotension without changes in heart rate was present. We here describe the 3-year time course of the changes in spectral indices of cardiovascular autonomic control LF/HF and LFSAP, dysautonomia symptoms intensity and anti-nAChR antibodies following repetitive selective immunoadsorptions. During the follow-up, the reduction of anti-nAChR antibodies produced by immunoadsorption was associated with a diminished orthostatic hypotension, a restored capability to increase LF/HF, LFSAP and norepinephrine in upright position, a decline in the intensity of autonomic symptoms and an improvement of life quality. Spectral parameters LF/HF and LFSAP may represent noninvasive, low-cost biomarkers suitable for autoimmune autonomic ganglionopathy patients' clinical follow-up.
aInternal Medicine 4, Humanitas Clinical and Research Center, Milano bNeuroimmunology and Neuromuscular Diseases Unit, I.R.C.C.S. Istituto Neurologico 'C. Besta' cI.R.C.C.S Istituti Clinici Scientifici Maugeri dHumanitas University, Rozzano, Milan, Italy.
Franca
F
Barbic
Franca
F
Dipaola
Francesca
F
Andreetta
Enrico
E
Brunetta
Laura
L
Dalla Vecchia
Renato
R
Mantegazza
Raffaello
R
Furlan
Carlo
C
Antozzi
D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D001341:Autonomic Nervous System / Q000187:drug effects* / Q000276:immunology / Q000503:physiopathology; D001342:Autonomic Nervous System Diseases / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D005725:Ganglia, Autonomic / Q000187:drug effects* / Q000276:immunology / Q000503:physiopathology; D006339:Heart Rate / Q000187:drug effects / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000494:pharmacology / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009638:Norepinephrine / Q000097:blood; D011978:Receptors, Nicotinic / Q000276:immunology*; D016896:Treatment Outcome
D001323:Autoantibodies; D007074:Immunoglobulin G; D011978:Receptors, Nicotinic; D009638:Norepinephrine