Joint hypermobility syndrome (JHS) is a complex connective tissue disorder characterized by excessive joint mobility and chronic musculoskeletal pain. The condition extends beyond the joints and can affect various systems such as the skin, gastrointestinal tract, neurological system, and cardiovascular system, necessitating a multidisciplinary approach to optimize care. Notably, the prevalence of JHS is higher in young individuals, females, and those of Asian descent. Despite being a common condition, JHS is frequently underdiagnosed, resulting in persistent pain and disability. In order to diagnose JHS, it is necessary to rule out the presence of any consistent feature that indicates the existence of other connective tissue disorders that partially overlap with it, including Marfan and Ehlers-Danlos syndromes. A thorough clinical assessment, coupled with the application of the Brighton Criteria, can aid in making an accurate diagnosis. Management of JHS poses significant challenges and typically involves symptomatic treatment, including physiotherapy, rehabilitation, and pharmacological therapy. Additionally, a multidisciplinary approach, including collaboration with different specialists, is vital. Further research is needed to improve our understanding of JHS and develop interventions based on solid evidence. However, with comprehensive management strategies, individuals with JHS can achieve better pain control and improved function, enabling them to lead more fulfilling lives.
Published in | American Journal of Internal Medicine (Volume 11, Issue 3) |
DOI | 10.11648/j.ajim.20231103.12 |
Page(s) | 35-40 |
Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
Copyright |
Copyright © The Author(s), 2023. Published by Science Publishing Group |
Hypermobility Syndrome, Ehlers-Danlos Syndrome, Joint Pain, Chronic Pain
[1] | Fikree A, Aziz Q, Grahame R. Joint hypermobility syndrome. Rheum Dis Clin North Am. 2013; 39 (2): 419-430. |
[2] | Carbonell-Bobadilla N, Rodríguez-Álvarez A, Rojas-García G, Barragán-Garfias J, Orrantia-Vertiz M, Rodríguez-Romo R. Joint hypermobility syndrome. Acta Ortopedica Mexicana. 2020; 34 (6): 441-9. |
[3] | Tinkle BT, Levy HP. Symptomatic Joint Hypermobility: The Hypermobile Type of Ehlers-Danlos Syndrome and the Hypermobility Spectrum Disorders. Med Clin North Am. 2019; 103 (6): 1021-1033. |
[4] | Grahame R. Hypermobility: an important but often neglected area within rheumatology. Nat Clin Pract Rheumatol. 2008; 4 (10): 522-524. |
[5] | Kumar B, Lenert P. Joint Hypermobility Syndrome: Recognizing a Commonly Overlooked Cause of Chronic Pain. Am J Med. 2017; 130 (6): 640-647. |
[6] | Tinkle BT. Symptomatic joint hypermobility. Best Pract Res Clin Rheumatol. 2020; 34 (3): 101508. |
[7] | Hakim AJ, Cherkas LF, Grahame R, Spector TD, MacGregor AJ. The genetic epidemiology of joint hypermobility: a population study of female twins. Arthritis Rheum. 2004; 50 (8): 2640-2644. |
[8] | Remvig L, Jensen DV, Ward RC. Epidemiology of general joint hypermobility and basis for the proposed criteria for benign joint hypermobility syndrome: review of the literature. J Rheumatol. 2007; 34: 804–9. |
[9] | Syx D, De Wandele I, Rombaut L, Malfait F. Hypermobility, the Ehlers-Danlos syndromes and chronic pain. Clin Exp Rheumatol. 2017; 35 Suppl 107 (5): 116-122. |
[10] | Atwell K, Michael W, Dubey J, et al. Diagnosis and Management of Hypermobility Spectrum Disorders in Primary Care. J Am Board Fam Med. 2021; 34 (4): 838-848. |
[11] | Tinkle B, Castori M, Berglund B, et al. Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history. Am J Med Genet C Semin Med Genet. 2017; 175 (1): 48-69. |
[12] | Pacey V, Tofts L, Wesley A, Collins F, Singh-Grewal D. Joint hypermobility syndrome: a review for clinicians. J Paediatr Child Health. 2015; 51 (4): 373-380. |
[13] | Colombi M, Dordoni C, Chiarelli N, Ritelli M. Differential diagnosis and diagnostic flow chart of joint hypermobility syndrome/ehlers-danlos syndrome hypermobility type compared to other heritable connective tissue disorders. Am J Med Genet C Semin Med Genet. 2015; 169C (1): 6-22. |
[14] | Beighton P, Solomon L, Soskolne CL. Articular mobility in an African population. Ann Rheum Dis. 1973; 32: 413–8. |
[15] | Malek S, Reinhold EJ, Pearce GS. The Beighton Score as a measure of generalised joint hypermobility. Rheumatol Int. 2021; 41 (10): 1707-1716. |
[16] | Grahame R, Bird HA, Child A. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol. 2000; 27 (7): 1777-1779. |
[17] | Hakim AJ, Grahame R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain. Int J Clin Pract. 2003; 57 (3): 163-166. |
[18] | De Wandele I, Rombaut L, Leybaert L, et al. Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome. Semin Arthritis Rheum. 2014; 44 (1): 93-100. |
[19] | Gazit Y, Nahir AM, Grahame R, Jacob G. Dysautonomia in the joint hypermobility syndrome. Am J Med. 2003; 115 (1): 33-40. |
[20] | Botrus G, Baker O, Borrego E, et al. Spectrum of Gastrointestinal Manifestations in Joint Hypermobility Syndromes. Am J Med Sci. 2018; 355 (6): 573-580. |
[21] | Castori M, Morlino S, Pascolini G, Blundo C, Grammatico P. Gastrointestinal and nutritional issues in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. Am J Med Genet C Semin Med Genet. 2015; 169C (1): 54-75. |
[22] | Baeza-Velasco C, Bourdon C, Montalescot L, et al. Low- and high-anxious hypermobile Ehlers-Danlos syndrome patients: comparison of psychosocial and health variables. Rheumatol Int. 2018; 38 (5): 871-878. |
[23] | Sanches SH, Osório Fde L, Udina M, Martín-Santos R, Crippa JA. Anxiety and joint hypermobility association: a systematic review. Braz J Psychiatry. 2012; 34 Suppl 1: S53-S60. |
[24] | Cederlöf M, Larsson H, Lichtenstein P, Almqvist C, Serlachius E, Ludvigsson JF. Nationwide population-based cohort study of psychiatric disorders in individuals with Ehlers-Danlos syndrome or hypermobility syndrome and their siblings. BMC Psychiatry. 2016; 16: 207. |
[25] | Bulbena A, Baeza-Velasco C, Bulbena-Cabré A, et al. Psychiatric and psychological aspects in the Ehlers-Danlos syndromes [published correction appears in Am J Med Genet A. 2017 Dec; 173 (12): 3241]. Am J Med Genet C Semin Med Genet. 2017; 175 (1): 237-245. |
[26] | Hakim A, De Wandele I, O'Callaghan C, Pocinki A, Rowe P. Chronic fatigue in Ehlers-Danlos syndrome-Hypermobile type. Am J Med Genet C Semin Med Genet. 2017; 175 (1): 175-180. |
[27] | Krahe AM, Adams RD, Nicholson LL. Features that exacerbate fatigue severity in joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type. Disabil Rehabil. 2018; 40 (17): 1989-1996. |
[28] | Henderson FC Sr, Austin C, Benzel E, et al. Neurological and spinal manifestations of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017; 175 (1): 195-211. |
[29] | Martin VT, Neilson D. Joint hypermobility and headache: the glue that binds the two together--part 2. Headache. 2014; 54 (8): 1403-1411. |
[30] | Bendik EM, Tinkle BT, Al-shuik E, et al. Joint hypermobility syndrome: A common clinical disorder associated with migraine in women. Cephalalgia. 2011; 31 (5): 603-613. |
[31] | Russek LN, Stott P, Simmonds J. Recognizing and Effectively Managing Hypermobility-Related Conditions. Phys Ther. 2019; 99 (9): 1189-1200. |
[32] | Palmer S, Terry R, Rimes KA, Clark C, Simmonds J, Horwood J. Physiotherapy management of joint hypermobility syndrome--a focus group study of patient and health professional perspectives. Physiotherapy. 2016; 102 (1): 93-102. |
[33] | Engelbert RH, Juul-Kristensen B, Pacey V, et al. The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome. Am J Med Genet C Semin Med Genet. 2017; 175 (1): 158-167. |
[34] | Kemp S, Roberts I, Gamble C, et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility. Rheumatology (Oxford). 2010; 49 (2): 315-325. |
[35] | Pacey V, Tofts L, Adams RD, Munns CF, Nicholson LL. Exercise in children with joint hypermobility syndrome and knee pain: a randomised controlled trial comparing exercise into hypermobile versus neutral knee extension. Pediatr Rheumatol Online J. 2013; 11 (1): 30. |
[36] | Chopra P, Tinkle B, Hamonet C, et al. Pain management in the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017; 175 (1): 212-219. |
APA Style
Jobson Lopes de Oliveira, Igor Albuquerque Nogueira, Camila Távora Nogueira, Saulo Venicius Parente Lopes, Gabriela Silva Teles, et al. (2023). Joint Hypermobility Syndrome: A Narrative Review. American Journal of Internal Medicine, 11(3), 35-40. https://doi.org/10.11648/j.ajim.20231103.12
ACS Style
Jobson Lopes de Oliveira; Igor Albuquerque Nogueira; Camila Távora Nogueira; Saulo Venicius Parente Lopes; Gabriela Silva Teles, et al. Joint Hypermobility Syndrome: A Narrative Review. Am. J. Intern. Med. 2023, 11(3), 35-40. doi: 10.11648/j.ajim.20231103.12
AMA Style
Jobson Lopes de Oliveira, Igor Albuquerque Nogueira, Camila Távora Nogueira, Saulo Venicius Parente Lopes, Gabriela Silva Teles, et al. Joint Hypermobility Syndrome: A Narrative Review. Am J Intern Med. 2023;11(3):35-40. doi: 10.11648/j.ajim.20231103.12
@article{10.11648/j.ajim.20231103.12, author = {Jobson Lopes de Oliveira and Igor Albuquerque Nogueira and Camila Távora Nogueira and Saulo Venicius Parente Lopes and Gabriela Silva Teles and Raul Sancho de Carvalho Rocha and Pedro Iughetti Morais and Ana Beatriz Cordeiro Carvalho and Pedro Henrique de Santana Pereira and David Johnson Pinheiro Alencar}, title = {Joint Hypermobility Syndrome: A Narrative Review}, journal = {American Journal of Internal Medicine}, volume = {11}, number = {3}, pages = {35-40}, doi = {10.11648/j.ajim.20231103.12}, url = {https://doi.org/10.11648/j.ajim.20231103.12}, eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20231103.12}, abstract = {Joint hypermobility syndrome (JHS) is a complex connective tissue disorder characterized by excessive joint mobility and chronic musculoskeletal pain. The condition extends beyond the joints and can affect various systems such as the skin, gastrointestinal tract, neurological system, and cardiovascular system, necessitating a multidisciplinary approach to optimize care. Notably, the prevalence of JHS is higher in young individuals, females, and those of Asian descent. Despite being a common condition, JHS is frequently underdiagnosed, resulting in persistent pain and disability. In order to diagnose JHS, it is necessary to rule out the presence of any consistent feature that indicates the existence of other connective tissue disorders that partially overlap with it, including Marfan and Ehlers-Danlos syndromes. A thorough clinical assessment, coupled with the application of the Brighton Criteria, can aid in making an accurate diagnosis. Management of JHS poses significant challenges and typically involves symptomatic treatment, including physiotherapy, rehabilitation, and pharmacological therapy. Additionally, a multidisciplinary approach, including collaboration with different specialists, is vital. Further research is needed to improve our understanding of JHS and develop interventions based on solid evidence. However, with comprehensive management strategies, individuals with JHS can achieve better pain control and improved function, enabling them to lead more fulfilling lives.}, year = {2023} }
TY - JOUR T1 - Joint Hypermobility Syndrome: A Narrative Review AU - Jobson Lopes de Oliveira AU - Igor Albuquerque Nogueira AU - Camila Távora Nogueira AU - Saulo Venicius Parente Lopes AU - Gabriela Silva Teles AU - Raul Sancho de Carvalho Rocha AU - Pedro Iughetti Morais AU - Ana Beatriz Cordeiro Carvalho AU - Pedro Henrique de Santana Pereira AU - David Johnson Pinheiro Alencar Y1 - 2023/05/25 PY - 2023 N1 - https://doi.org/10.11648/j.ajim.20231103.12 DO - 10.11648/j.ajim.20231103.12 T2 - American Journal of Internal Medicine JF - American Journal of Internal Medicine JO - American Journal of Internal Medicine SP - 35 EP - 40 PB - Science Publishing Group SN - 2330-4324 UR - https://doi.org/10.11648/j.ajim.20231103.12 AB - Joint hypermobility syndrome (JHS) is a complex connective tissue disorder characterized by excessive joint mobility and chronic musculoskeletal pain. The condition extends beyond the joints and can affect various systems such as the skin, gastrointestinal tract, neurological system, and cardiovascular system, necessitating a multidisciplinary approach to optimize care. Notably, the prevalence of JHS is higher in young individuals, females, and those of Asian descent. Despite being a common condition, JHS is frequently underdiagnosed, resulting in persistent pain and disability. In order to diagnose JHS, it is necessary to rule out the presence of any consistent feature that indicates the existence of other connective tissue disorders that partially overlap with it, including Marfan and Ehlers-Danlos syndromes. A thorough clinical assessment, coupled with the application of the Brighton Criteria, can aid in making an accurate diagnosis. Management of JHS poses significant challenges and typically involves symptomatic treatment, including physiotherapy, rehabilitation, and pharmacological therapy. Additionally, a multidisciplinary approach, including collaboration with different specialists, is vital. Further research is needed to improve our understanding of JHS and develop interventions based on solid evidence. However, with comprehensive management strategies, individuals with JHS can achieve better pain control and improved function, enabling them to lead more fulfilling lives. VL - 11 IS - 3 ER -