Pain management in the Ehlers–Danlos syndromes – American Journal of Medical Genetics Part C: Seminars in Medical Genetics – February 2017 – free full-text article
All the authors are internationally recognized EDS specialists: Brad Tinkle, Claude Hamonet, Isabelle Brock, Anne Gompel, Antonio Bulbena, Clair Francomano
Chronic pain is one of the major symptoms presented by patients with hEDS [Sacheti et al., 1997; Voermans et al., 2010].
It often presents as diffuse body pain affecting almost every part of the body.
It is common and may be severe [Voermans et al., 2009].
In one study, the prevalence of chronic pain was 90% in patients with various types of EDS, with the highest scores on severity of pain found in hEDS [Voermans et al., 2009].
Pain and fatigue have a high prevalence in EDS, frequently manifesting as the predominant symptoms and as the most disabling features [Rombaut et al., 2011].
Clinical examination, pain questionnaires, quantitative sensory testing, and neurophysiological responses disclosed no somatosensory nervous system damage.
In a study of 206 female patients with EDS, the impact of pain and functional impairment was similar to fibromyalgia but worse than that of rheumatoid arthritis [Rombaut et al., 2011].
Any form of pain be it nociceptive or neuropathic may be a secondary or even tertiary effect of underlying causes
Chronic pain in the Ehlers–Danlos syndromes (EDS) is common and may be severe. According to one study, nearly 90% of patients report some form of chronic pain.
Pain, which is often one of the first symptoms to occur, may be widespread or localized to one region such as an arm or a leg.
Studies on treatment modalities are few and insufficient to guide management.
The following is a discussion of the evidence regarding the underlying mechanisms of pain in EDS.
The causes of pain in this condition are multifactorial and include
- joint subluxations and dislocations,
- previous surgery,
- muscle weakness,
- proprioceptive disorders, and
- vertebral instability.
- Affected persons may also present with
- generalized body pain,
- gastrointestinal pain,
- temporomandibular joint pain,
- dysmenorrhea, and
Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint, proprioceptive disorder) and minimizing the sensation of pain.
Management strategies for chronic pain in EDS includes physical therapy, medications, as well as durable medical equipment such as cushions, compressive garments, and braces. The different modalities are discussed in this paper.
Pain is common in Ehlers–Danlos syndrome (EDS) and may correlate with
- frequency of subluxations and dislocations,
- soft tissue injury,
- history of previous surgery,
- myalgias, and
- may become chronic.
Pain may be musculoskeletal and/or widespread.
It may be acute and/or chronic.
The pain may interfere with socialization and activities of daily living.
Table I. Review of Literature of Types of Pain in hEDS
|Manifestations||Number of patients studied||Incidence (%)||References|
|Generalized body pain||>800 (cumulative)||90||Jerosch and Prymka ; Camerota et al. ; Hamonet et al. [2012, 2014]; Hamonet and Brock ; Scheper et al. ; Voermans and Knoop, 2011|
|Soft-tissue pain||>800 (cumulative)||90||Hudson et al. ; Hamonet et al. [2012, 2014]; Scheper et al. |
|Dislocations||>800 (cumulative)||78||Voermans et al. ; Hamonet et al. [2012, 2014]|
|Joint pain||28*||Elbow (43)*||Moore et al. ; Aktas et al. *; Sacheti et al. ; Tubiana ; Berglund et al. ; McCulloch and Redmond ; Hamonet et al. ^; Hamonet et al. ; Hamonet and Brock, 2015#]; Christopherson and Adams ; Scheper et al. |
|Fatigue||644 [cumulative]||95||Gulbahar et al. ; Voermans et al. [2009, 2010]; *Celletti et al. ; Hamonet et al. |
|11 [cumulative]||6 (55)*|
|Bone loss||23||16 (70)||Gulbahar et al. |
|Neuropathic pain||29*||68*||DeGraaf ; Kass and Kayed ; Stoler and Oaklander *; Camerota et al. ; Voermans et al. |
|Loss of proprioception||18*, 32#, 22^||Significant P-value||Helliwell *; Ferrell et al. ; Fatoye et al. ; #Rombaut et al. ; Zarate et al. ; Celletti et al. ; Galli et al. ; ^Clayton et al. ; Smith et al. ; Deparcy |
|Headaches||28*||75*||Sansur et al. ; Schievink et al. ; DeCoster et al. ; Henderson et al. [2005a]; Gulbahar et al. ; Milhorat et al. *; Bendik et al. ; Rozen ; Hamonet and Brock |
|Gastrointestinal pain||21*||85.7*||Douglas and Douglas ; Petros and Swash ; Castori et al. *; Zarate et al. ; Dordoni et al. ; Hamonet and Brock ; Mohammed et al., 2010|
|Temporomandibular joint pain||42*||71.4*||*DeCoster et al. [2004, 2005]; Hagberg et al. |
The article goes on to delve into a detailed description of the kinds of pain resulting from EDS: Pain management in the Ehlers–Danlos syndromes