Pain management strategies for neuropathic pain in Fabry disease - a systematic review

Abstract

BackgroundNeuropathic pain is one of the key features of (classical) Fabry disease (FD). No randomized clinical trials comparing effectiveness of different pain management strategies have been performed. This review aims to give an overview of existing pain management strategies.MethodsPubMed and Embase were searched up to September 2014 for relevant articles on treatment of neuropathic pain in FD.ResultsSeven-hundred-thirty-one articles were identified of which 26 were included in the analysis. Studies reported on 55 individuals in total, with group-sizes ranging from 1 to 8. Carbamazepine appeared most beneficial: complete pain relief in 5/25, partial relief in 17/25, and no benefit in 3/25 patients. Phenytoin resulted in complete relief in 1/27, partial relief in 12/27 and no benefit in 6/27 patients. In 8 patients a significant reduction in the frequency of pain attacks was described. Gabapentin caused partial relief in 6/7 and no relief in 1/7 patients. Little evidence was reported for SSNRI’s or treatment combinations. Adverse-effects were reported in all treatment strategies.ConclusionsOnly for carbamazepine, phenytoin and gabapentin there is evidence of effectiveness in neuropathic pain due to FD, but comparison of effectiveness between these drugs is lacking. In routine clinical practice adverse-effects may discourage use of carbamazepine and phenytoin in favor of second-generation antiepileptic drugs, but this is currently not supported by clinical evidence. This review suffers greatly from incomplete outcome reports and a predominance of case reports, which emphasizes the need for robust clinical trials and observational cohort studies.

European expert consensus statement on therapeutic goals in Fabry disease

“…of patients experience severe depression [134]. As patients often experience chronic neuropathic pain and there is a strong association of chronic pain with depression, it is also important to manage Fabry-related pain adequately, with regular assessment and use of appropriate pharmacological therapies [95,117,118]. [135].…”

“…Many patients still suffer from neuropathic pain despite ERT and therefore intensified adjunctive pain management should be considered. Exercising clinical caution in the selection of analgesics for symptomatic treatment of neuropathic pain is important to avoid undesirable side effects and potential addiction risks[94,118]. Based on currently available clinical evidence, tricyclic antidepressants, serotonin and noradrenaline reuptake inhibitors (e.g.…”

“…Based on currently available clinical evidence, tricyclic antidepressants, serotonin and noradrenaline reuptake inhibitors (e.g. duloxetine), carbamazepine, gabapentin, and pregabalin are suggested pain-management agents in Fabry disease, although treatment outcomes from several clinical trials are modest, and tricyclic antidepressant use may increase the risk of cardiac arrhythmia[118]. Lidocaine patches, high-strength capsaicin patches, and tramadol are second-line options, while strong opioids (preferably controlled-release formulations) should be reserved as a last resort third line of treatment, considering the reported chronic opioid abuse epidemic[119].…”

The neurological manifestations of Fabry disease. A review

“…Для профилакти-ки инсультов могут использоваться статины [88]. Для ку-пирования нейропатической боли эффективны карбама-зепин, фенитоин, габапентин [89].…”

Erratum to: Pain management strategies for neuropathic pain in Fabry disease - a systematic review

“…After publication of the original article [ 1 ], it came to the authors’ attention that Additional file 1 had been erroneously included as Appendix 1. As a result, the published article did not contain an option to download the Additional file; ‘Treatment options: A summary of the most commonly prescribed drugs in neuropathic pain in FD, with information on dosage, titration and precautions’ is available to download as Additional file 1 in this erratum.…”