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

• Published in: Journal of clinical apheresis Vol. 31; no. 4; pp. 368 - 374
• Main Authors: Hendrickson, Jeanne E., Hendrickson, Emma T., Gehrie, Eric A., Sidhu, Davinder, Wallukat, Gerd, Schimke, Ingolf, Tormey, Christopher A.
• Format: Journal Article
• Language: English
• Published: United States Blackwell Publishing Ltd 01.08.2016; Wiley Subscription Services, Inc
• Subjects: Adolescent; Autoantibodies - blood; Complex Regional Pain Syndromes - therapy; CRPS; Female; Humans; immunosuppression; Plasma Exchange - methods; Primary Dysautonomias - therapy; Receptor, Muscarinic M2 - immunology; Receptors, Adrenergic, beta-2 - immunology; Receptors, Nicotinic - immunology; RSD; therapeutic plasma exchange; immunosuppression; therapeutic plasma exchange; RSD; CRPS
• ISSN: 0733-2459; 1098-1101
• DOI: 10.1002/jca.21407

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.