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16 Articles in Volume 19, Issue #2
Analgesics of the Future: Inside the Potential of Glial Cell Modulators
APPs as Leaders in Pain Management
Cases in Urine Drug Monitoring Interpretation: How to Stay in Control
Complex Chronic Pain Disorders
Efficacy of Chiropractic Care for Back Pain: A Clinical Summary
Hydrodissection for the Treatment of Abdominal Pain Caused by Post-Operative Adhesions
Letters: The Word "Catastrophizing;" AIPM Ceases Operations; Patient Questions
Management of Severe Radiculopathy in a Pregnant Patient
Managing Pain in Adults with Intellectual Disabilities
Pain in the Courtroom: An Excerpt
Q&A with Howard L. Fields: How Patients’ Expectations May Control Pain
Special Report: CGRP Monoclonal Antibodies for Chronic Migraine
The Management of Chronic Overlapping Pain Conditions
Vibration for Chronic Pain
What are the dangers of loperamide abuse?
When Patient Education Fails to Improve Outcomes: A Low Back Pain Case

Complex Chronic Pain Disorders

The pathophysiology of and approaches to 3 commonly seen pain conditions: CRPS, EDS, and SFN.
Pages 26-32
Page 2 of 4

Optimal treatment goals include pain reduction and restoration of normal limb function, best achieved with a multidisciplinary approach.11 A rehabilitation specialist, including physical and occupational therapy, is recommended. Physical therapy may be directed at improving range of motion and avoiding immobilization. Occupational therapy may include methods to reduce edema and improve function. A number of methods have claimed to promote pain desensitization in patients with CRPS, including stress-loading using scrubbing and carrying techniques, mirror visual feedback, pain-exposure physical therapy, and graded motor imagery.11

Multidisciplinary management should include mental health professionals. Individual counseling, cognitive behavioral therapy (CBT), and specific pain-coping and pain desensitization techniques may be useful. Co-existing major mood disturbances should be identified and treated.

There are limited data on the utility of pharmacotherapy in the treatment of CRPS, but a short course of corticosteroids, especially in early stages, and/or bisphosphonates may be recommended based on systematic reviews.11 Gabapentin, pregabalin, NSAIDs, tricyclics such as amitriptyline and similar medications, nasal calcitonin, as well as topical anesthetics and capsaicin have been useful in small trials. There have been reports suggesting improvement with ketamine infusions, memantine, intravenous immunoglobulin, epidural clonidine, clonidine, and injections of botulinum toxin A, but these interventions have not been tested in randomized clinical trials (RCTs) to date. In patients not responding to pharmacotherapy, peripheral sympathetic blockade, including lumbar/thoracic, stellate ganglion, brachial plexus blocks, sympathectomy, and spinal cord stimulation have been performed with mixed results and, again, a lack of RCTs.11 There are encouraging but limited data on the utility of transcranial magnetic stimulation in the treatment of CRPS.12 Although opioids were often used in the past to treat CRPS, there is no long-term data to support their efficacy and current recommendations do not support their use, other than for short-time duration.11

Although the outcome of patients with CRPS is highly variable, one study found that six years after the diagnosis, two-thirds of subjects still met criteria for CRPS.13 One-third were unable to work and disability and litigation issues were common. Recurrence of CRPS ranges from 10% to 30%.

(Source: 123RF)

Ehlers-Danlos/Joint Hypermobility

Diagnosis, Clinical Features

Ehlers-Danlos syndrome, or EDS, includes a group of genetic disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Although there are a number of subtypes of EDS, this review will focus on hypermobile EDS (hEDS), the most common type. The other types of EDS include those with cardiac and vascular manifestations. Most of these are rare and have specific genetic inheritance traits (eg, EDS with kyphoscoliotic, cornea, myopathic, and scleral abnormalities).

Classical EDS is characterized by joint hypermobility and skin hyperextensibility and has a specific genetic abnormality in DNA collagen sequencing. However, hEDS may have no major skin abnormalities and no known single genetic defect has been identified. hEDS overlaps clinically with Joint Hypermobility Syndrome (JHS), with some experts considering these two conditions to be the same disorder.14 However, JHS has no specific genetic basis or markers. The prevalence of hEDS ranges from 0.01% to 1% and this wide range is likely related to inclusion or exclusion of cases of JHS.14

Joint hypermobility is the most common clinical manifestation of hEDS.14 Hypermobility is best evaluated with the Beighton Hypermobility Scale (see Table VI). The diagnosis of hEDS is made clinically if the subject has generalized joint hypermobility and a number of other features with a positive family history. However, each of the EDS subtypes has different diagnostic criteria and, in certain types, genetic testing provides excellent sensitivity and specificity. Therefore, in all suspected cases of EDS, referral to an expert in clinical genetics may be recommended.

JHS is much more common than EDS and may be difficult to differentiate from hEDS.14,15 It is present in 5% to 10% of the general population. Clinical manifestations include joint hypermobility using the Beighton Hypermobility Scale, skin fragility, and chronic widespread pain. There is also often a family history of joint hypermobility.

There is a significant clinical overlap of hEDS and JHS with fibromyalgia/CWP.16 More than 90% of 466 adults with EDS reported joint pain and 42% had a diagnosis of fibromyalgia.17 Joint hypermobility was a strong risk factor for CWP.17 Both EDS and JHS have been associated with chronic fatigue, chronic abdominal and pelvic pain, high rates of irritable bowel syndrome, temporomandibular joint disorder, and vulvodynia.

Pathophysiology

Genetic alterations vary in each of the EDS subtypes, most being autosomal dominant. In classic EDS, mutations have been identified in two collagen genes, but in hEDS, the exact mutations have not been located.15 The cardiac and vascular EDS subtypes have genetic mutations involving different forms of procollagen and collagen.

Joint hypermobility may lead to overuse injury, joint subluxations, traumatic arthritis, and secondary osteoarthritis. These peripheral factors interact with central factors in patients with EDS/JHS. Patients with EDS exhibit hyperalgesia and allodynia. Pressure pain thresholds (PPTs) in hEDS subjects were significantly lower than in controls.18 Patients with either hEDS or JHS exhibited reduced cold and heat pain thresholds and increased wind-up.18 EDS/JHS has also been associated with a high prevalence of psychiatric disorders, including depression, anxiety, and eating disorders.19 Neuroimaging has demonstrated increased reactivity in pain and emotion processing. Autonomic nervous system dysfunction, including dysautonomia, has also been common.

Treatment

Last updated on: March 4, 2019
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