title: Complex regional pain syndrome and dysautonomia in a 14-year-old girl responsive to therapeutic plasma exchange.
abstract: Reflex sympathetic dystrophy, also known as complex regional pain syndrome (CRPS), has recently been shown to be associated with autoantibodies against β2-adrenergic and muscarinic M2 receptors. In addition to pain and sudomotor/vasomotor symptoms, dysautonomia is also observed in a subset of CRPS patients. Despite its severity, there are few effective therapies for CRPS described to date. We report a case of a 14-year-old girl with CRPS of her right leg and dysautonomia (gastroparesis, postural tachycardia) refractory to multiple therapies, successfully treated with therapeutic plasma exchange (TPE) with albumin replacement. The patient, who has serum anti β2-adrenergic and muscarinic M2 receptor autoantibodies in addition to nicotinic acetylcholine receptor ganglionic autoantibodies, underwent an initial course of five TPEs over a 2-week period. She demonstrated a clinical response to TPE as manifested by a rapid improvement in her fatigue and gastroparesis, with a gradual yet significant improvement in her leg pain and sudomotor/vasomotor flares. Following the loading procedures, the patient was treated with rituximab. She continues to require periodic TPE to maintain a remission, with additional immunosuppression being considered long term. Although further studies are needed, TPE (in combination with immunosuppression) may be an appropriate therapy for CRPS patients with detectable autoantibodies, as it is for better characterized diseases with autoantibodies against neuronal surface receptors such as myasthenia gravis or Lambert Eaton myasthenic syndrome. J. Clin. Apheresis 31:368-374, 2016. © 2015 Wiley Periodicals, Inc.
authors.affiliation: Department of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut.|Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: Jeanne E
authors.initials: JE
authors.lastname: Hendrickson
authors.affiliation: Adams School, Guilford, Connecticut.
authors.forename: Emma T
authors.initials: ET
authors.lastname: Hendrickson
authors.affiliation: Department of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: Eric A
authors.initials: EA
authors.lastname: Gehrie
authors.affiliation: Department of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: Davinder
authors.initials: D
authors.lastname: Sidhu
authors.affiliation: Berlin Cures GmbH, Berlin, Germany.|Max Delbrück Center for Moleculare Medicine, Berlin, Germany.
authors.forename: Gerd
authors.initials: G
authors.lastname: Wallukat
authors.affiliation: Berlin Cures GmbH, Berlin, Germany.
authors.forename: Ingolf
authors.initials: I
authors.lastname: Schimke
authors.affiliation: Department of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut.|VA Connecticut Healthcare System, West Haven, Connecticut.
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Tormey
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D020918:Complex Regional Pain Syndromes / Q000628:therapy*; D005260:Female; D006801:Humans; D010951:Plasma Exchange / Q000379:methods*; D054969:Primary Dysautonomias / Q000628:therapy*; D043585:Receptor, Muscarinic M2 / Q000276:immunology; D018343:Receptors, Adrenergic, beta-2 / Q000276:immunology; D011978:Receptors, Nicotinic / Q000276:immunology
chemical_list: D001323:Autoantibodies; D043585:Receptor, Muscarinic M2; D018343:Receptors, Adrenergic, beta-2; D011978:Receptors, Nicotinic
keywords: CRPS; RSD; immunosuppression; therapeutic plasma exchange