Helmholtz Gemeinschaft

Search
Browse
Statistics
Feeds

Complex regional pain syndrome and dysautonomia in a 14-year-old girl responsive to therapeutic plasma exchange

Item Type:Article
Title:Complex regional pain syndrome and dysautonomia in a 14-year-old girl responsive to therapeutic plasma exchange
Creators Name:Hendrickson, J.E. and Hendrickson, E.T. and Gehrie, E.A. and Sidhu, D. and Wallukat, G. and Schimke, I. and Tormey, C.A.
Abstract:Reflex sympathetic dystrophy, also known as complex regional pain syndrome (CRPS), has recently been shown to be associated with autoantibodies against {beta}2-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 {beta}2-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.
Keywords:CRPS, RSD, Therapeutic Plasma Exchange, Immunosuppression
Source:Journal of Clinical Apheresis
ISSN:0733-2459
Publisher:Wiley-Blackwell (U.S.A.)
Volume:31
Number:4
Page Range:368-374
Date:August 2016
Official Publication:https://doi.org/10.1002/jca.21407
PubMed:View item in PubMed

Repository Staff Only: item control page

Open Access
MDC Library