Cited by 237Connective tissue disorders researchMusculoskeletal pain and rehabilitationPediatric Pain Management Techniques

The DOI matches its Crossref record; 26 of the 26 references it cites were found in a registry or an index.

Pain in Ehlers-Danlos Syndrome Is Common, Severe, and Associated with Functional Impairment

Open access

Voermans, Nicol C., Knoop, Hans, Bleijenberg, Gijs, van Engelen, Baziel G.·2010·Journal of Pain and Symptom Management

DOI 10.1016/j.jpainsymman.2009.12.026

Open this paper in CoChat — free account Open PDF

Abstract

Context The Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Musculoskeletal pain is mentioned in the diagnostic criteria and described as early in onset, chronic, and debilitating. However, systematic research on pain in EDS is scarce.Objectives We investigated prevalence and impact of pain and associated features in a large group of EDS patients.Methods We performed a study among members of the Dutch EDS patient organization (n=273) and included the McGill Pain Questionnaire to investigate various aspects of pain, the Sickness Impact Profile to study functional impairment, the Symptom Checklist subscale sleep to evaluate sleep disturbances, and the Checklist Individual Strength subscale fatigue to determine fatigue severity.Results The results of this study show that 1) chronic pain in EDS is highly prevalent and associated with regular use of analgesics; 2) pain is more prevalent and more severe in the hypermobility type than in the classic type; 3) pain severity is correlated with hypermobility, dislocations, and previous surgery; 4) pain is correlated with low nocturnal sleep quality; and 5) pain contributes to functional impairment in daily life, independent of the level of fatigue.Conclusion From this large cohort of EDS patients, we conclude that pain is common and severe in EDS. Pain is related to hypermobility, dislocations, and previous surgery and associated with moderate to severe impairment in daily functioning. Therefore, treatment of pain should be a prominent aspect of symptomatic management of EDS.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Integrity signals

Registry

Crossref · 10.1016/j.jpainsymman.2009.12.026

Cites

26 of 26 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

SF-36 health survey: Manual and interpretation guide
Ware, J. (2003) · Medical Entomology and Zoology · Cited by 11,424
The McGill Pain Questionnaire: Major properties and scoring methods
Melzack, R. (1975) · Pain · doi:10.1016/0304-3959(75)90044-5 · Cited by 6,873
Systematic review and meta-analysis of randomized controlled trials of cognitive behaviour therapy and behaviour therapy for chronic pain in adults, excluding headache
Morley, S., Eccleston, C., & Williams, A. (1999) · Pain · doi:10.1016/s0304-3959(98)00255-3 · Cited by 1,597
Dimensional assessment of chronic fatigue syndrome
Vercoulen, J. H. M. M., Swanink, C. M. A., Fennis, J. F. M., Galama, J. M. D., van der Meer, J. W. M., & Bleijenberg, G. (1994) · Journal of Psychosomatic Research · doi:10.1016/0022-3999(94)90099-x · Cited by 1,306
Self-citation
Psychological aspects of persistent pain: Current state of the science
Keefe, F. J., Rumble, M. E., Scipio, C. D., Giordano, L. A., & Perri, L. M. (2004) · The Journal of Pain · doi:10.1016/j.jpain.2004.02.576 · Cited by 954
Connective Tissue and Its Heritable Disorders
Heinegård, D., Aspberg, A., Franzén, A., & Lorenzo, P. (2002) · Wiley eBooks · doi:10.1002/0471221929 · Cited by 946
A recessive form of the Ehlers-Danlos syndrome caused by tenascin-X deficiency
Schalkwijk, J., Zweers, M. C., Steijlen, P. M., Dean, W. B., Taylor, G., van Vlijmen, I. M., van Haren, B., Miller, W. L., & Bristow, J. (2001) · New England Journal of Medicine · doi:10.1056/nejmoa002939 · Cited by 391
Tenascin-X deficiency is associated with Ehlers-Danlos syndrome
Burch, G. H., Gong, Y., Liu, W., Dettman, R. W., Curry, C. J., Smith, L., Miller, W. L., & Bristow, J. (1997) · Nature Genetics · doi:10.1038/ng0997-104 · Cited by 366
The Ehlers‐Danlos Syndrome
Steinmann, B., Royce, P. M., & Superti‐Furga, A. (2002) · Connective Tissue and Its Heritable Disorders · doi:10.1002/0471221929.ch9 · Cited by 358
Estimating the clinical effectiveness of cognitive behavioural therapy in the clinic: Evaluation of a CBT informed pain management programme
Morley, S., Williams, A., & Hussain, S. (2008) · Pain · doi:10.1016/j.pain.2008.02.025 · Cited by 194

Show all 26 references

Research works printed but not linked: 4

Baakman WBE. [Quality of life in patients with Ehlers-Danlos syndrome]. [Dutch]. Thesis. Utrecht: University of Utrecht, Health Psychology, 2002.
Arrindell, 1986, Lisse: Swets & Zeitlinger
Berger, 1981, [German]. Z Psychosom Med Psychoanal, 27, 45
Jacobs, 1990, Ned Tijdschr Geneeskd, 134, 1950

Sorted by citations

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history
Tinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31538 · Cited by 499
Chronic diseases in the European Union: the prevalence and health cost implications of chronic pain
Leadley, R. M., Armstrong, N., Lee, Y. C., Allen, A., & Kleijnen, J. (2012) · Journal of Pain & Palliative Care Pharmacotherapy · doi:10.3109/15360288.2012.736933 · Cited by 282
Neurological and spinal manifestations of the Ehlers–Danlos syndromes
Henderson, F. C., Austin, C., Benzel, E., Bolognese, P., Ellenbogen, R., Francomano, C. A., Ireton, C., Klinge, P., Koby, M., Long, D., et al. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31549 · Cited by 238
Self-citation
Ehlers-Danlos Syndrome, hypermobility type: an underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations
Castori, M. (2012) · ISRN Dermatology · doi:10.5402/2012/751768 · Cited by 222
Pain management in the Ehlers–Danlos syndromes
Chopra, P., Tinkle, B., Hamonet, C., Brock, I., Gompel, A., Bulbena, A., & Francomano, C. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31554 · Cited by 209
Ehlers-Danlos syndrome, classical type
Bowen, J. M., Sobey, G. J., Burrows, N. P., Colombi, M., Lavallee, M. E., Malfait, F., & Francomano, C. A. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31548 · Cited by 184
Small fibre neuropathy
Cazzato, D., & Lauria, G. (2017) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000472 · Cited by 160
Re‐writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type
Castori, M., Morlino, S., Celletti, C., Ghibellini, G., Bruschini, M., Grammatico, P., Blundo, C., & Camerota, F. (2013) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36315 · Cited by 159
Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome, hypermobility type): Principles and proposal for a multidisciplinary approach
Castori, M., Morlino, S., Celletti, C., Celli, M., Morrone, A., Colombi, M., Camerota, F., & Grammatico, P. (2012) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.35483 · Cited by 162
Muscle mass, muscle strength, functional performance, and physical impairment in women with the hypermobility type of Ehlers‐Danlos syndrome
Rombaut, L., Malfait, F., Wandele, I. D., Taes, Y., Thijs, Y., Paepe, A. D., & Calders, P. (2012) · Arthritis Care & Research · doi:10.1002/acr.21726 · Cited by 137
Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes
Cazzato, D., Castori, M., Lombardi, R., Caravello, F., Bella, E. D., Petrucci, A., Grammatico, P., Dordoni, C., Colombi, M., & Lauria, G. (2016) · Neurology · doi:10.1212/wnl.0000000000002847 · Cited by 122
Medication, surgery, and physiotherapy among patients with the hypermobility type of Ehlers-Danlos syndrome
Rombaut, L., Malfait, F., De Wandele, I., Cools, A., Thijs, Y., De Paepe, A., & Calders, P. (2011) · Archives of Physical Medicine and Rehabilitation · doi:10.1016/j.apmr.2011.01.016 · Cited by 124
Modest association of joint hypermobility with disabling and limiting musculoskeletal pain: results from a large-scale General population-based survey
Mulvey, M. R., Macfarlane, G. J., Beasley, M., Symmons, D. P. M., Lovell, K., Keeley, P., Woby, S., & McBeth, J. (2013) · Arthritis Care & Research · doi:10.1002/acr.21979 · Cited by 115
Balance, gait, falls, and fear of falling in women with the hypermobility type of Ehlers‐Danlos syndrome
Rombaut, L., Malfait, F., Wandele, I. D., Thijs, Y., Palmans, T., Paepe, A. D., & Calders, P. (2011) · Arthritis Care & Research · doi:10.1002/acr.20557 · Cited by 114
Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome hypermobility type) in 82 Italian patients
Castori, M., Morlino, S., Dordoni, C., Celletti, C., Camerota, F., Ritelli, M., Morrone, A., Venturini, M., Grammatico, P., & Colombi, M. (2012) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.35506 · Cited by 106
Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type
Di Stefano, G., Celletti, C., Baron, R., Castori, M., Di Franco, M., La Cesa, S., Leone, C., Pepe, A., Cruccu, G., Truini, A., et al. (2016) · European Journal of Pain · doi:10.1002/ejp.856 · Cited by 100
Gastrointestinal and nutritional issues in joint hypermobility syndrome/ehlers–danlos syndrome, hypermobility type
Castori, M., Morlino, S., Pascolini, G., Blundo, C., & Grammatico, P. (2015) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31431 · Cited by 98
Ehlers-Danlos Syndrome in Orthopaedics
Shirley, E. D., DeMaio, M., & Bodurtha, J. N. (2012) · Sports Health A Multidisciplinary Approach · doi:10.1177/1941738112452385 · Cited by 101
Ehlers–Danlos Syndrome—Hypermobility Type: A Much Neglected Multisystemic Disorder
Gazit, Y., Jacob, G., & Grahame, R. (2016) · Rambam Maimonides Medical Journal · doi:10.5041/rmmj.10261 · Cited by 94
Ehlers–Danlos syndrome, hypermobility type: A characterization of the patients' lived experience
Murray, B., Yashar, B. M., Uhlmann, W. R., Clauw, D. J., & Petty, E. M. (2013) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36293 · Cited by 92
Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: A comparative study with fibromyalgia and rheumatoid arthritis
Rombaut, L., Malfait, F., De Paepe, A., Rimbaut, S., Verbruggen, G., De Wandele, I., & Calders, P. (2011) · Arthritis & Rheumatism · doi:10.1002/art.30337 · Cited by 91
Symptom and joint mobility progression in the joint hypermobility syndrome (Ehlers-Danlos syndrome, hypermobility type).
(2011) · PubMed · Cited by 91
Gastrointestinal disorders in joint hypermobility syndrome/Ehlers‐Danlos syndrome hypermobility type: A review for the gastroenterologist
Beckers, A. B., Keszthelyi, D., Fikree, A., Vork, L., Masclee, A., Farmer, A. D., & Aziz, Q. (2017) · Neurogastroenterology & Motility · doi:10.1111/nmo.13013 · Cited by 90
Clinical heterogeneity in patients with the hypermobility type of Ehlers-Danlos Syndrome
De Wandele, I., Rombaut, L., Malfait, F., De Backer, T., De Paepe, A., & Calders, P. (2013) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2012.11.018 · Cited by 89
Chronic pain in patients with the hypermobility type of Ehlers-Danlos syndrome: Evidence for generalized hyperalgesia
Rombaut, L., Scheper, M., De Wandele, I., De Vries, J., Meeus, M., Malfait, F., Engelbert, R., & Calders, P. (2015) · Clinical Rheumatology · doi:10.1007/s10067-014-2499-0 · Cited by 91
Multidisciplinary treatment of disability in ehlers–danlos syndrome hypermobility type/hypermobility syndrome: A pilot study using a combination of physical and cognitive‐behavioral therapy on 12 women
Bathen, T., Hångmann, A. B., Hoff, M., Andersen, L. Ø., & Rand‐Hendriksen, S. (2013) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36060 · Cited by 86
Psychiatric disorders in Ehlers-Danlos syndrome are frequent, diverse and strongly associated with pain
Hershenfeld, S. A., Wasim, S., McNiven, V., Parikh, M., Majewski, P., Faghfoury, H., & So, J. (2016) · Rheumatology International · doi:10.1007/s00296-015-3375-1 · Cited by 81
Neurological manifestations of Ehlers-Danlos syndrome(s): A review.
Castori, M., & Voermans, N. C. (2014) · Europe PMC (PubMed Central) · Cited by 82
Self-citation
Both pain and fatigue are important possible determinants of disability in patients with the Ehlers-Danlos syndrome hypermobility type
Voermans, N. C., & Knoop, H. (2011) · Disability and Rehabilitation · doi:10.3109/09638288.2010.531373 · Cited by 81
Self-citation
Temporomandibular joint anatomy, function and clinical relevance
Wilkie, G., & Al‐Ani, Z. (2022) · BDJ · doi:10.1038/s41415-022-5082-0 · Cited by 76
Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type
Celletti, C., Castori, M., La Torre, G., & Camerota, F. (2013) · BioMed Research International · doi:10.1155/2013/580460 · Cited by 81
Joint protection and physical rehabilitation of the adult with hypermobility syndrome
Keer, R. J., & Simmonds, J. V. (2011) · Current Opinion in Rheumatology · doi:10.1097/bor.0b013e328342d3af · Cited by 71
Oral and mandibular manifestations in the Ehlers–Danlos syndromes
Mitakides, J., & Tinkle, B. T. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31541 · Cited by 66
Connective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain
Castori, M., Morlino, S., Ghibellini, G., Celletti, C., Camerota, F., & Grammatico, P. (2015) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.31426 · Cited by 69
The functional consequences of generalized joint hypermobility: a cross-sectional study
Scheper, M. C., de Vries, J. E., Juul-Kristensen, B., Nollet, F., & Engelbert, R. H. (2014) · BMC Musculoskeletal Disorders · doi:10.1186/1471-2474-15-243 · Cited by 65
Understanding the psychosocial impact of joint hypermobility syndrome and Ehlers–Danlos syndrome hypermobility type: a qualitative interview study
Bennett, S., Walsh, N. E., Moss, T., & Palmer, S. (2019) · Disability and Rehabilitation · doi:10.1080/09638288.2019.1641848 · Cited by 73
Neuropathic pain is a common feature in Ehlers-Danlos syndrome
Camerota, F., Celletti, C., Castori, M., Grammatico, P., & Padua, L. (2011) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2010.09.012 · Cited by 61
Shoulder function, pain and health related quality of life in adults with joint hypermobility syndrome/Ehlers–Danlos syndrome-hypermobility type
Johannessen, E. C., Reiten, H. S., Løvaas, H., Maeland, S., & Juul-Kristensen, B. (2016) · Disability and Rehabilitation · doi:10.3109/09638288.2015.1102336 · Cited by 61
Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know
Thwaites, P. A., Gibson, P. R., & Burgell, R. E. (2022) · Journal of Gastroenterology and Hepatology · doi:10.1111/jgh.15927 · Cited by 59
Exercise beliefs and behaviours of individuals with Joint Hypermobility syndrome/Ehlers–Danlos syndrome – hypermobility type
Simmonds, J. V., Herbland, A., Hakim, A., Ninis, N., Lever, W., Aziz, Q., & Cairns, M. (2019) · Disability and Rehabilitation · doi:10.1080/09638288.2017.1398278 · Cited by 60
Fatigue in adults with Marfan syndrome, occurrence and associations to pain and other factors
Bathen, T., Velvin, G., Rand‐Hendriksen, S., & Robinson, H. S. (2014) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36574 · Cited by 59
The Roles of Tenascins in Cardiovascular, Inflammatory, and Heritable Connective Tissue Diseases
Matsumoto, K., & Aoki, H. (2020) · Frontiers in Immunology · doi:10.3389/fimmu.2020.609752 · Cited by 58
Musculoskeletal Conditions in a Pediatric Population with Ehlers-Danlos Syndrome
Stern, C. M., Pepin, M. J., Stoler, J. M., Kramer, D. E., Spencer, S. A., & Stein, C. J. (2017) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2016.10.078 · Cited by 56
Pain in hypermobile Ehlers‐Danlos syndrome: New insights using new criteria
Bénistan, K., & Martinez, V. (2019) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.61175 · Cited by 57
Recognizing the tenascin‐X deficient type of Ehlers–Danlos syndrome: a cross‐sectional study in 17 patients
Demirdas, S., Dulfer, E., Robert, L., Kempers, M., van Beek, D., Micha, D., van Engelen, B. G., Hamel, B., Schalkwijk, J., Loeys, B., et al. (2017) · Clinical Genetics · doi:10.1111/cge.12853 · Cited by 60
Self-citation
Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review
Gilliam, E. A., Hoffman, J. D., & Yeh, G. Y. (2019) · Clinical Genetics · doi:10.1111/cge.13624 · Cited by 58
Cognitive, emotional, and behavioral considerations for chronic pain management in the Ehlers–Danlos syndrome hypermobility-type: a narrative review
Baeza-Velasco, C., Bulbena, A., Polanco-Carrasco, R., & Jaussaud, R. (2019) · Disability and Rehabilitation · doi:10.1080/09638288.2017.1419294 · Cited by 51
Proprioceptive precision is impaired in Ehlers-Danlos syndrome
Clayton, H. A., Jones, S. A. H., & Henriques, D. Y. P. (2015) · SpringerPlus · doi:10.1186/s40064-015-1089-1 · Cited by 50
Current Diagnosis and Treatment of Painful Small Fiber Neuropathy
Farhad, K. (2019) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-019-1020-1 · Cited by 47
Physical therapy treatment of hypermobile Ehlers–Danlos syndrome: A systematic review
Reychler, G., Backer, M. D., Piraux, E., Poncin, W., & Caty, G. D. (2021) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62393 · Cited by 50
Pain in the Ehlers–Danlos syndromes: Mechanisms, models, and challenges
Malfait, F., Colman, M., Vroman, R., De Wandele, I., Rombaut, L., Miller, R. E., Malfait, A., & Syx, D. (2021) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31950 · Cited by 41
Pain in Ehlers-Danlos syndromes: manifestations, therapeutic strategies and future perspectives
Castori, M. (2016) · Expert Opinion on Orphan Drugs · doi:10.1080/21678707.2016.1238302 · Cited by 46
A study of migraine characteristics in joint hypermobility syndrome a.k.a. Ehlers–Danlos syndrome, hypermobility type
Puledda, F., Viganò, A., Celletti, C., Petolicchio, B., Toscano, M., Vicenzini, E., Castori, M., Laudani, G., Valente, D., Camerota, F., et al. (2015) · Neurological Sciences · doi:10.1007/s10072-015-2173-6 · Cited by 49
Resistance to local anesthesia in people with the Ehlers-Danlos Syndromes presenting for dental surgery
Schubart, J. R., Schaefer, É., Janicki, P., Adhikary, S. D., Schilling, A. L., Hakim, A. J., Bascom, R., Francomano, C. A., & Raj, S. R. (2019) · Journal of Dental Anesthesia and Pain Medicine · doi:10.17245/jdapm.2019.19.5.261 · Cited by 48
A study exploring the prevalence of Joint Hypermobility Syndrome in patients attending a Musculoskeletal Triage Clinic
Connelly, E., Hakim, A. J., Davenport, S., & Simmonds, J. V. (2015) · Physiotherapy Practice and Research · doi:10.3233/ppr-140046 · Cited by 46
Management of chronic pain in Ehlers–Danlos syndrome: Two case reports and a review of literature
Zhou, Z., Rewari, A., & Shanthanna, H. (2018) · Medicine · doi:10.1097/md.0000000000013115 · Cited by 46
Chapter 18, The Spectrum of Pediatric Osteoporosis
Bianchi, M. L., & Glorieux, F. H. (2011) · Cited by 46
Satisfaction with life in adults with Marfan syndrome (MFS): associations with health-related consequences of MFS, pain, fatigue, and demographic factors
Velvin, G., Bathen, T., Rand‐Hendriksen, S., & Geirdal, A. Ø. (2016) · Quality of Life Research · doi:10.1007/s11136-015-1214-1 · Cited by 44
Pain Phenotypes in Rare Musculoskeletal and Neuromuscular Diseases
Tucker-Bartley, A., Lemme, J. D., Gomez-Morad, A. D., Shah, N. A., Veliu, M., Birklein, F., Storz, C., Rutkove, S. B., Kronn, D., Boyce, A. M., et al. (2021) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2021.02.009 · Cited by 45
Ehlers-Danlos syndrome, hypermobility type
INSERM (2020) · Definitions · doi:10.32388/0ekfj6 · Cited by 39
Is pain the only symptom in patients with benign joint hypermobility syndrome?
Gezer, İ. A., Yılmaz, H., Akkurt, H. E., Sallı, A., & Karaca, G. (2014) · Clinical Rheumatology · doi:10.1007/s10067-014-2610-6 · Cited by 38
Entrapment neuropathies and polyneuropathies in joint hypermobility syndrome/Ehlers-Danlos syndrome
Granata, G., Padua, L., Celletti, C., Castori, M., Saraceni, V. M., & Camerota, F. (2013) · Clinical Neurophysiology · doi:10.1016/j.clinph.2012.12.051 · Cited by 37
Pain control methods in use and perceived effectiveness by patients with Ehlers–Danlos syndrome: a descriptive study
Arthur, K. B., Caldwell, K. L., Forehand, S., & Davis, K. M. (2015) · Disability and Rehabilitation · doi:10.3109/09638288.2015.1092175 · Cited by 37
Work participation in adults with Marfan syndrome: Demographic characteristics, MFS related health symptoms, chronic pain, and fatigue
Velvin, G., Bathen, T., Rand‐Hendriksen, S., & Geirdal, A. Ø. (2015) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.37370 · Cited by 37
The Upper Limb Hypermobility Assessment Tool: A novel validated measure of adult joint mobility
Nicholson, L. L., & Chan, C. (2018) · Musculoskeletal Science and Practice · doi:10.1016/j.msksp.2018.02.006 · Cited by 40
Myopathy in a 20‐year‐old female patient with D4ST‐1 deficient Ehlers‐Danlos syndrome due to a homozygous CHST14 mutation
Voermans, N. C., Kempers, M., Lammens, M., van Alfen, N., Janssen, M. C., Bönnemann, C., van Engelen, B. G., & Hamel, B. C. (2012) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.35232 · Cited by 36
Self-citation
Psychosocial Influence of Ehlers–Danlos Syndrome in Daily Life of Patients: A Qualitative Study
Palomo-Toucedo, I., Leon-Larios, F., Reina-Bueno, M., Vázquez-Bautista, M., Munuera-Martínez, P., & Domínguez-Maldonado, G. (2020) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph17176425 · Cited by 36
COL6A5variants in familial neuropathic chronic itch
Boneschi, F. M., Colombi, M., Castori, M., Devigili, G., Eleopra, R., Malik, R. A., Ritelli, M., Zoppi, N., Dordoni, C., Sorosina, M., et al. (2016) · Brain · doi:10.1093/brain/aww343 · Cited by 36
High Frequency of Neuropathic Pain in Ehlers-Danlos Syndrome: An Association with Axonal Polyneuropathy and Compression Neuropathy?
Voermans, N. C., Knoop, H., & van Engelen, B. G. M. (2011) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2011.02.006 · Cited by 35
Self-citation
Ehlers–Danlos Syndrome and Hypermobility Syndrome Compared with Other Common Chronic Pain Diagnoses—A Study from the Swedish Quality Registry for Pain Rehabilitation
Molander, P., Novo, M., Hållstam, A., Löfgren, M., Stålnacke, B., & Gerdle, B. U. C. (2020) · Journal of Clinical Medicine · doi:10.3390/jcm9072143 · Cited by 36
Ehlers–Danlos syndrome and neurological features: a review
Savasta, S., Merli, P., Ruggieri, M., Bianchi, L., & Spartà, M. V. (2010) · Child s Nervous System · doi:10.1007/s00381-010-1256-1 · Cited by 33
High rate of dyspareunia and probable vulvodynia in Ehlers–Danlos syndromes and hypermobility spectrum disorders: An online survey
Glayzer, J. E., McFarlin, B. L., Castori, M., Suarez, M. L., Meinel, M., Kobak, W. H., Steffen, A. D., & Schlaeger, J. M. (2021) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.31939 · Cited by 33
Joint Hypermobility and Headache: Understanding the Glue That Binds the Two Together – Part 1
Neilson, D., & Martin, V. T. (2014) · Headache The Journal of Head and Face Pain · doi:10.1111/head.12418 · Cited by 32
Ehlers–Danlos syndrome(s) mimicking child abuse: Is there an impact on clinical practice?
Castori, M. (2015) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.31460 · Cited by 30
Pain due to Ehlers-Danlos Syndrome Is Associated with Deficit of the Endogenous Pain Inhibitory Control
Leone, C. M., Celletti, C., Gaudiano, G., Puglisi, P. A., Fasolino, A., Cruccu, G., Camerota, F., & Truini, A. (2020) · Pain Medicine · doi:10.1093/pm/pnaa038 · Cited by 30
Exploring the underlying mechanism of pain-related disability in hypermobile adolescents with chronic musculoskeletal pain
van Meulenbroek, T., Huijnen, I. P. J., Simons, L. E., Conijn, A. E. A., Engelbert, R. H. H., & Verbunt, J. A. (2021) · Scandinavian Journal of Pain · doi:10.1515/sjpain-2020-0023 · Cited by 30
“In G-HSD/hEDS joint pain is one of the most common complaints [23, 24] and recurrent musculoskeletal pain is an essential part of the classification of G-HSD/hEDS [9, 10].”
Background
Refining patterns of joint hypermobility, habitus, and orthopedic traits in joint hypermobility syndrome and Ehlers–Danlos syndrome, hypermobility type
Morlino, S., Dordoni, C., Sperduti, I., Venturini, M., Celletti, C., Camerota, F., Colombi, M., & Castori, M. (2017) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.38106 · Cited by 29
Sleep disorders and their management in children with Ehlers-Danlos syndrome referred to sleep clinics
Domany, K. A., Hantragool, S., Smith, D. F., Xu, Y., Hossain, M., & Simakajornboon, N. (2018) · Journal of Clinical Sleep Medicine · doi:10.5664/jcsm.7058 · Cited by 29
Use of complementary therapies for chronic pain management in patients with reported Ehlers‐Danlos syndrome or hypermobility spectrum disorders
Demes, J. S., McNair, B., & Taylor, M. R. G. (2020) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.61837 · Cited by 30
Placing joint hypermobility in context: traits, disorders and syndromes
Morlino, S., & Castori, M. (2023) · British Medical Bulletin · doi:10.1093/bmb/ldad013 · Cited by 32
Ehlers-Danlos Syndrome, Hypermobility Type -- GeneReviews(®)
Ra, P., Adam, M. P., Hh, A., Se, W., Amemiya, A., Ljh, B., Bird, T. D., Ct, F., Mefford, H. C., Rjh, S., et al. (2016) · Europe PMC (PubMed Central) · Cited by 29
Adaptation of balance reactions following forward perturbations in people with joint hypermobility syndrome
Bates, A. V., McGregor, A. H., & Alexander, C. M. (2021) · BMC Musculoskeletal Disorders · doi:10.1186/s12891-021-03961-y · Cited by 29
Exercise and Rehabilitation in People With Ehlers-Danlos Syndrome: A Systematic Review
Buryk-Iggers, S., Mittal, N., Mina, D. S., Adams, S. C., Englesakis, M. F., Rachinsky, M., Lopez-Hernandez, L., Hussey, L., McGillis, L., McLean, L., et al. (2022) · Archives of Rehabilitation Research and Clinical Translation · doi:10.1016/j.arrct.2022.100189 · Cited by 43
A connective tissue disorder may underlie ESSENCE problems in childhood
Baeza-Velasco, C., Grahame, R., & Bravo, J. F. (2017) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2016.10.011 · Cited by 26
Diseased Filum Terminale as a Cause of Tethered Cord Syndrome in Ehlers-Danlos Syndrome: Histopathology, Biomechanics, Clinical Presentation, and Outcome of Filum Excision
Klinge, P. M., Srivastava, V., McElroy, A. W., Leary, O. P., Ahmed, Z., Donahue, J. E., Brinker, T., Vloo, P. D., & Gokaslan, Z. L. (2022) · World Neurosurgery · doi:10.1016/j.wneu.2022.03.038 · Cited by 29
Kinematics and kinetics of people who are hypermobile. A systematic review
Bates, A. V., & Alexander, C. M. (2015) · Gait & Posture · doi:10.1016/j.gaitpost.2015.01.009 · Cited by 26
Small fiber neuropathy in hypermobile Ehlers Danlos syndrome/hypermobility spectrum disorder
Fernandez, A., Aubry‐Rozier, B., Vautey, M., Berna, C., & Suter, M. R. (2022) · Journal of Internal Medicine · doi:10.1111/joim.13539 · Cited by 27
Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond
Pezaro, S., Pearce, G., & Reinhold, E. J. (2018) · British Journal of Midwifery · doi:10.12968/bjom.2018.26.4.217 · Cited by 26
Symptom complaints and impact on functioning in youth with hypermobile Ehlers–Danlos syndrome
Tran, S. T., Jagpal, A., Koven, M. L., Turek, C. E., Golden, J. S., & Tinkle, B. T. (2020) · Journal of Child Health Care · doi:10.1177/1367493519867174 · Cited by 25
Efficacy of an out-patient pain management programme for people with joint hypermobility syndrome
Rahman, A., Daniel, C., & Grahame, R. (2014) · Clinical Rheumatology · doi:10.1007/s10067-014-2539-9 · Cited by 26
Use of prescription opioid and other drugs among a cohort of persons with Ehlers–Danlos syndrome: A retrospective study
Schubart, J. R., Schilling, A., Schaefer, E., Bascom, R., & Francomano, C. (2019) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.61031 · Cited by 26
Neuromuscular Activation Differences During Gait in Patients With Ehlers‐Danlos Syndrome and Healthy Adults
Robbins, S. M. K., Cossette‐Levasseur, M., Kikuchi, K., Sarjeant, J., Shiu, Y., Azar, C., & Hazel, E. M. (2019) · Arthritis Care & Research · doi:10.1002/acr.24067 · Cited by 23
Use of Cluster Analysis to Delineate Symptom Profiles in an Ehlers-Danlos Syndrome Patient Population
Schubart, J. R., Schaefer, É., Hakim, A. J., Francomano, C. A., & Bascom, R. (2019) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2019.05.013 · Cited by 27
Gastrointestinal symptoms and nutritional issues in patients with hypermobility disorders: assessment, diagnosis and management
Lam, C., Amarasinghe, G., Zarate-Lopez, N., Fikree, A., Byrne, P., Kiani-Alikhan, S., Gabe, S., & Paine, P. (2023) · Frontline Gastroenterology · doi:10.1136/flgastro-2022-102088 · Cited by 23
Pain Symptomatology and Management in Pediatric Ehlers–Danlos Syndrome: A Review
Feldman, E. C. H., Hivick, D. P., Slepian, P. M., Tran, S. T., Chopra, P., & Greenley, R. N. (2020) · Children · doi:10.3390/children7090146 · Cited by 24
Physical therapy interventions in generalized hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome: a scoping review
Brittain, M. G., Flanagan, S., Foreman, L., & Teran-Wodzinski, P. C. (2023) · Disability and Rehabilitation · doi:10.1080/09638288.2023.2216028 · Cited by 23
Ehlers–Danlos syndrome hypermobility type: a possible unifying concept for various functional somatic syndromes
Castori, M., Celletti, C., & Camerota, F. (2011) · Rheumatology International · doi:10.1007/s00296-011-2275-2 · Cited by 22
Primary Total Hip Arthroplasty in Patients With Ehlers-Danlos Syndrome: A Retrospective Matched-Cohort Study
Guier, C., Shi, G. G., Ledford, C. K., Taunton, M. J., Heckman, M. M., & Wilke, B. K. (2020) · Arthroplasty Today · doi:10.1016/j.artd.2020.05.006 · Cited by 22
Functional adaptation of tendon and skeletal muscle to resistance training in three patients with genetically verified classic Ehlers Danlos Syndrome
Møller, M., Kjær, M., Andersen, R. L., Magnusson, S. P., & Nielsen, R. B. (2019) · Muscles Ligaments and Tendons Journal · doi:10.32098/mltj.03.2014.09 · Cited by 21
Pain-related behaviors and abnormal cutaneous innervation in a murine model of classical Ehlers-Danlos syndrome
Syx, D., Miller, R. E., Obeidat, A. M., Tran, P. B., Vroman, R., Malfait, Z., Miller, R. J., Malfait, F., & Malfait, A.-M. (2020) · Pain · doi:10.1097/j.pain.0000000000001935 · Cited by 19

Authors: Voermans, N. C., Knoop, H., Bleijenberg, G., & van Engelen, B. G.
Affiliations: Radboud University Medical Center (NL)
Corresponding author: Voermans, Nicol C.
ORCID: Voermans, Nicol C.
Knoop, Hans
Bleijenberg, Gijs
Venue: Journal of Pain and Symptom Management
Publisher: Elsevier BV
Published: 2010-09
Volume: 40
Issue: 3
Pages: 370-378
ISSN: 0885-3924 (ISSN-L)
DOI: 10.1016/j.jpainsymman.2009.12.026
OpenAlex: W2152518735
Cited by: 237
References: 26
Citations by year: 2026: 11 · 2025: 9 · 2024: 15 · 2023: 15 · 2022: 20 · 2021: 28 · 2020: 17 · 2019: 16 · 2018: 10 · 2017: 12 · 2016: 17 · 2015: 15 · 2014: 13 · 2013: 11 · 2012: 11
FWCI: 7.02
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 3421 d)
Locations: Journal of Pain and Symptom Management (publishedVersion)
PubMed (publishedVersion)
Radboud Repository (Radboud University) (submittedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Health Sciences › Health Professions › Physical Therapy, Sports Therapy and Rehabilitation › Musculoskeletal pain and rehabilitation
Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Pediatric Pain Management Techniques
Keywords: Ehlers-Danlos syndrome, chronic pain, pain prevalence, pain intensity, functional disability, joint hypermobility, dislocations, previous surgery, sleep disorders, fatigue, analgesic use, hereditary connective tissue disorders, McGill Pain Questionnaire, Sickness Impact Profile, Checklist Individual Strength
MeSH terms: Activities of Daily Living, Adolescent, Adult, Aged, Aged, 80 and over, Analgesics, Cross-Sectional Studies, Ehlers-Danlos Syndrome, Fatigue, Female, Humans, Male, Middle Aged, Pain, Pain Measurement, Sleep Wake Disorders, Young Adult

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

Pain in Ehlers-Danlos Syndrome Is Common, Severe, and Associated with Functional Impairment

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Self-check

Citations

Cites

Cited by

Related

Metadata

Record

About these checks