Kompleksni regionalni bolni sindrom [Complex regional pain syndrome]

Blažeković, Ivan and Bilić, Ervina and Žagar, Marija and Anić, Branimir (2015) Kompleksni regionalni bolni sindrom [Complex regional pain syndrome]. Liječnički vjesnik, 137 (9-10). pp. 297-306. ISSN 0024-3477

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Abstract

Complex regional pain syndrome (CRPS) represents a state of constant and often disabling pain, affecting one region (usually hand) and often occurs after a trauma whose severity does not correlate with the level of pain. The older term for this condition of chronic pain associated with motor and autonomic symptoms is reflex sympathetic dystrophy or causalgia. The aim of this review, based on contemporary literature, is to show the epidemiology and etiology, proposed pathophysiological mechanisms, method of diagnosis and treatment options, prevention and mitigation of this under-recognized disease. CRPS I occurs without known neurological damage, unlike CRPS II, where the history of trauma is present and in some cases damage to the peripheral nervous system can be objectively assessed using electromyoneurography. New diagnostic methods, such as quantitative sensory testing (CST), challenge this division because the CST findings in patients with CRPS I can suggest damage to Adelta peripheral nerve fibers. Except for distinguishing type I and type II disease, it is important to bear in mind the diversity of clinical presentation of CRPS in acute and chronic phase of the disease. This regional pain syndrome typically includes the autonomic and motor signs and thus differs from other peripheral neuropathic pain syndromes. The complexity of the clinical presentation indicates the likely presence of different pathophysiological mechanisms underlying this disease. Previous studies have demonstrated the autonomic dysfunction, neurogenic inflammation and neuroplastic changes. The diagnosis of CRPS is based on anamnesis and clinical examination on the basis of which the disease can be graded according to the Budapest Criteria. A valuable aid in differentiating subtypes of the disease is electromyoneurography. The treatment of CRPS is as complex as the clinical picture and the pathophysiology of the disease and requires interdisciplinary cooperation and individual approach. The pharmacological approach is mainly symptomatic, including analgesics, glucocorticoids, baclofen, bisphosphonates and prophylactic administration of vitamin C. Physical therapy besides preventing atrophy and contractures reduces the use of analgesic therapy. Invasive approach includes stimulation of the spinal cord, peripheral nerve catheters with anesthetic and amputation that patients in severe condition gladly accept. Further research is needed to better understand the disease and more effective therapies.

Abstract in Croatian

Kompleksni regionalni bolni sindrom (engl. complex regional pain syndrome – CRPS) stanje je trajne i često onesposobljavajuće boli, zahvaća jednu regiju (najčešće ruku) i često se javlja nakon traume koja se težinom ne poklapa s razinom boli. Stariji naziv za ovo stanje kronične boli s pridruženim motoričkim i autonomnim simptomima jest refleksna simpatička distrofija ili kauzalgija. Cilj je ovoga preglednog rada temeljem literaturnih podataka prikazati epidemiologiju i etiologiju, predložene patofiziološke mehanizme, način dijagnosticiranja te mogućnosti liječenja, prevencije i smanjenja posljedica ove nedovoljno prepoznate bolesti. Oblik CRPS-a I nastaje bez poznatoga neurološkog oštećenja, za razliku od CRPS-a II, gdje u anamnezi nalazimo podatke o traumi, a u nekim slučajevima možemo elektromioneurografski objektivizirati oštećenje perifernoga živčanog sustava. Nove dijagnostičke metode, poput kvantitativnoga senzornog testiranja (KST), izazov su za ovu podjelu jer se u nalazu KST-a i u bolesnika s CRPS-om I može naći oštećenje A-delta-vlakana perifernih živaca. Osim što razlikujemo tip I i tip II bolesti, važno je imati na umu i kliničke različitosti CRPS-a u akutnoj i kroničnoj fazi bolesti. Ovaj regionalni bolni sindrom redovito obuhvaća i autonomne i motoričke znakove i time se razlikuje od drugih sindroma periferne neuropatske boli. Složenost kliničke slike upućuje na vjerojatnu prisutnost različitih patofizioloških mehanizama u podlozi ove bolesti. U dosadašnjim istraživanjima dokazane su autonomna disfunkcija, neurogena upala i neuroplastične promjene. Dijagnoza CRPS-a osniva se na anamnezi i kliničkom pregledu temeljem kojih se bolest može stupnjevati sukladno tzv. Budimpeštanskim kriterijima. Dragocjena pomoć u razlikovanju podtipova bolesti jest elektromioneurografija. Liječenje CRPS-a složeno je kao i sama klinička slika i patofiziologija bolesti te zahtijeva ­interdisciplinarnu suradnju i individualni pristup. Farmakološki je pristup pretežito simptomatski, uključuje analgetike, glukokortikoide, baklofen, bisfosfonate te profilaktičko davanje vitamina C. Fizikalna terapija osim sprječavanja atrofije i kontrakture uda smanjuje i uporabu analgetske terapije. Invazivni pristup obuhvaća stimulaciju kralježnične moždine, periferne živčane katetere s anestetikom te konačno amputaciju koju pacijenti u teškom stanju prihvaćaju. Daljnja su istraživanja potrebna radi boljeg razumijevanja bolesti i učinkovitije terapije.

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