Intravenous Immunoglobulin Therapy in Refractory Autoimmune Dysautonomias: A Retrospective Analysis of 38 Patients

American Journal of Therapeutics
Jill R Schofield, Kamal R Chemali

Abstract

Intravenous immunoglobulin (IVIG) has recognized efficacy in autoimmune peripheral nerve disorders, but there has been limited study of the use of IVIG in autoimmune dysautonomias. To determine the efficacy and safety of IVIG in patients with disabling, refractory autoimmune dysautonomias, including patients with postural tachycardia syndrome and gastrointestinal dysmotility. Patients with one or more autonomic disorder(s) and persistent serological evidence for autoimmunity who were unable to work or attend school despite usual treatments for dysautonomia were treated with IVIG for at least 3 months at a dose of at least 1 gm/kg monthly. Outcome measures included the composite autonomic symptom scale 31 survey and a functional ability score. There were 38 patients, 84% female and mean age of 28.4 years. Of patients, 83.5% improved on IVIG as defined by at least 20% improvement in the composite autonomic symptom scale 31 and/or functional ability score. The mean pretreatment functional ability score was 21% (mostly bedridden), which improved to a mean of 74% (nearing able to return to work/school) for responsive patients after at least 1 year of IVIG. The mean time to the first sign of response was 5.3 weeks. There were no seri...Continue Reading

References

Jan 6, 1996·Lancet·M T HeafieldA C Williams
Apr 1, 1997·Mayo Clinic Proceedings·A A SmitW Wieling
May 1, 1997·Journal of Neurology, Neurosurgery, and Psychiatry·R A Mericle, W J Triggs
Dec 16, 1998·The Netherlands Journal of Medicine·P J BarendregtA J Man In 't Veld
Jan 10, 2001·JAMA : the Journal of the American Medical Association·P C RoweS E Straus
Jul 18, 2003·The American Journal of Medicine·Yael GazitGiris Jacob
Jan 20, 2005·Journal of Internal Medicine·C J Czura, K J Tracey
Mar 19, 2005·Journal of Neurology, Neurosurgery, and Psychiatry·C H Gibbons, R Freeman
Apr 3, 2007·Journal of Neurology·Yusuf A RajaballyRichard J Abbott
Feb 13, 2008·Archives of Neurology·Christopher H GibbonsRoy Freeman
Apr 28, 2009·Autoimmunity Reviews·Ljudmila Stojanovich
May 16, 2009·Internal Medicine·Akihiro UedaTatsuro Mutoh
Mar 24, 2010·International Journal of Medical Sciences·Khalil KanjwalBlair P Grubb
Dec 12, 2012·Mayo Clinic Proceedings·David M SlettenWolfgang Singer
Dec 19, 2012·Autonomic Neuroscience : Basic & Clinical·Ivan AdamecMario Habek
Feb 28, 2014·Journal of the American Heart Association·Hongliang LiDavid C Kem
May 9, 2014·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Barbara BarunMario Habek
Jul 22, 2014·Neurogastroenterology and Motility : the Official Journal of the European Gastrointestinal Motility Society·E P FlanaganS J Pittock
Dec 30, 2014·European Journal of Pain : EJP·P KarlssonT S Jensen
Feb 11, 2015·Gastroenterology·Ciarán P KellyDaniel A Leffler
Jun 24, 2017·The Neurologist·Brent P GoodmanLucinda A Harris
Sep 5, 2017·Clinical Pediatrics·Jill R Schofield, Jeanne E Hendrickson
Feb 7, 2018·Therapeutic Advances in Neurological Disorders·Xiaolei LiuAnne Louise Oaklander
Feb 7, 2014·International Journal of Cardiology. Heart & Vessels·Rachel Nicoll, Michael Y Henein

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Citations

Sep 25, 2019·Journal of the American Heart Association·Amanda J Miller, Taylor A Doherty
Aug 5, 2020·Journal of the American Heart Association·Isabella KharrazihaViktor Hamrefors
Jul 29, 2020·Canadian Journal of Pain = Revue Canadienne De La Douleur·Mary A Kelley, Anne Louise Oaklander
Jan 1, 2021·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Belén RodriguezWerner J Z'Graggen
Apr 3, 2021·Journal of Autoimmunity·Gilad HalpertYehuda Shoenfeld
Nov 13, 2021·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Christopher J MathiasAlan Hakim

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