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.