Complex regional pain syndrome, prototype of a novel kind of autoimmune disease

• Published in: Autoimmunity reviews Vol. 12; no. 6; pp. 682 - 686
• Main Authors: Goebel, Andreas, Blaes, Franz
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01-04-2013
• Subjects: Allergy and Immunology; Animals; Autoimmune Diseases - drug therapy; Autoimmune Diseases - immunology; Chronic Pain - drug therapy; Chronic Pain - immunology; Complex regional pain syndrome; Complex Regional Pain Syndromes - drug therapy; Complex Regional Pain Syndromes - immunology; CRPS; Humans; Immunoglobulin; Immunoglobulins, Intravenous - therapeutic use; Immunologic Factors - therapeutic use; IVIG; Neuropathic pain; IVIG; Immunoglobulin; CRPS; Complex regional pain syndrome; Neuropathic pain
• ISSN: 1568-9972; 1568-9972; 1873-0183
• DOI: 10.1016/j.autrev.2012.10.015

Abstract Complex regional pain syndrome (CRPS) is a painful condition, which arises in a limb after trauma. CRPS can profoundly affect patients' quality of life, and there is no cure. CRPS is associated with limb-confined sensory, motor, skin, bone and autonomic abnormalities. Recent research has shown that some patients respond to treatment with immunoglobulins, and that a majority have IgG serum-autoantibodies directed against, and activating autonomic receptors. CRPS serum-IgG, when transferred to mice elicits abnormal behaviour. These results suggest that CRPS is associated with an autoantibody-mediated autoimmune process in some cases. CRPS has unusual features, including a non-destructive, and regionally-confined course. We propose that CRPS constitutes a prototype of a new kind of autoimmunity, which we term ‘IRAM’ (injury-triggered, regionally-restricted autoantibody-mediated autoimmune disorder with minimally-destructive course). Understanding autoimmune contribution to CRPS should allow the exploration of novel treatment modalities in the future. Additional ‘functional’ disorders, painful or painless may be autoimmune in nature.