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Patients with Ehlers Danlos syndrome and CRPS: a possible association?
Joan M Stoler1, Anne Louise Oaklander
1Genetics and Teratology Unit, Department of Pediatrics, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. jstoler@partners.org
Insights
Ehlers Danlos syndrome (EDS) may predispose individuals to complex regional pain syndrome (CRPS) after limb trauma. This association suggests EDS could impact nerve injury, connective tissue fragility, or surgical trauma in CRPS development.
Area of Science:
- Neuroscience
- Genetics
- Rheumatology
Background:
- Complex Regional Pain Syndrome (CRPS) is a debilitating condition often following limb trauma, with unknown predisposing factors.
- Genetic contributions to CRPS are suspected but not identified.
- Ehlers Danlos Syndrome (EDS) encompasses inherited connective tissue disorders.
Observation:
- Four patients diagnosed with CRPS also presented with classical or hypermobility types of Ehlers Danlos Syndrome (EDS).
- All observed patients had sustained joint injuries, with three undergoing surgical treatment.
- Standard diagnostic criteria were used for both CRPS and EDS diagnoses.
Findings:
- The co-occurrence of CRPS and EDS suggests a potential link between these conditions.
- Hypothesized mechanisms include nerve stretch injury in hypermobile joints, fragile nerve connective tissue in EDS, and increased surgical trauma.
- EDS may be a contributing factor to the development of CRPS.
Implications:
- Screening CRPS patients for EDS may be beneficial for clinical and research purposes.
- Understanding the role of EDS could lead to new diagnostic or therapeutic strategies for CRPS.
- Further research is warranted to elucidate the genetic and mechanistic links between EDS and CRPS.
Abstract:
Rare patients are left with chronic pain, vasodysregulation, and other symptoms that define complex regional pain syndrome (CRPS), after limb traumas. The predisposing factors are unknown. Genetic factors undoubtedly contribute, but have not yet been identified. We report four CRPS patients also diagnosed with the classical or hypermobility forms of Ehlers Danlos syndrome (EDS), inherited disorders of connective tissue. These patients had been diagnosed using standard diagnostic criteria for CRPS and for EDS. All had sustained joint injury; in three this had been surgically treated. The association of these two diagnoses leads us to hypothesize that EDS might contribute to the development of CRPS in one or more of the following ways: via stretch injury to nerves traversing hypermobile joints, increased fragility of nerve connective tissue, or nerve trauma from more frequent surgery. We review the clinical presentation of the different Ehlers Danlos syndromes and provide clinical criteria that can be used to screen CRPS patients for EDS for clinical or research purposes.
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