Ehlers–Danlos Syndrome—Hypermobility Type: A Much Neglected Multisystemic Disorder
Open Access
- 31 October 2016
- journal article
- review article
- Published by Rambam Health Corporation in Rambam Maimonides Medical Journal
- Vol. 7 (4), e0034
- https://doi.org/10.5041/rmmj.10261
Abstract
Ehlers–Danlos syndrome (EDS)—hypermobility type (HT) is considered to be the most common subtype of EDS and the least severe one; EDS-HT is considered to be identical to the joint hypermobility syndrome and manifests with musculoskeletal complaints, joint instability, and soft tissue overuse injury. Musculoskeletal complaints manifest with joint pain of non-inflammatory origin and/or spinal pain. Joint instability leads to dislocation or subluxation and involves peripheral joints as well as central joints, including the temporomandibular joints, sacroiliac joints, and hip joints. Soft tissue overuse injury may lead to tendonitis and bursitis without joint inflammation in most cases. Ehlers–Danlos syndrome-HT carries a high potential for disability due to recurrent dislocations and subluxations and chronic pain. Throughout the years, extra-articular manifestations have been described, including cardiovascular, autonomic nervous system, gastrointestinal, hematologic, ocular, gynecologic, neurologic, and psychiatric manifestations, emphasizing the multisystemic nature of EDS-HT. Unfortunately, EDS-HT is under-recognized and inadequately managed, leading to neglect of these patients, which may lead to severe disability that almost certainly could have been avoided. In this review article we will describe the known manifestations of the extra-articular systems.Keywords
This publication has 74 references indexed in Scilit:
- Is there any link between joint hypermobility and mitral valve prolapse in patients with fibromyalgia syndrome?Clinical Rheumatology, 2015
- Balance, gait, falls, and fear of falling in women with the hypermobility type of Ehlers‐Danlos syndromeArthritis Care & Research, 2011
- Pregnancy and Delivery in Ehlers-Danlos Syndrome (Hypermobility Type): Review of the LiteratureObstetrics and Gynecology International, 2011
- Desmopressin responsiveness in children with Ehlers‐Danlos syndrome associated bleeding symptomsBritish Journal of Haematology, 2008
- Bleeding and bruising in patients with Ehlers–Danlos syndrome and other collagen vascular disordersBritish Journal of Haematology, 2004
- Ehlers–Danlos Syndrome (EDS) focusing on oral symptoms: a questionnaire studyOrthodontics & Craniofacial Research, 2004
- Time to take hypermobility seriously (in adults and children)Rheumatology, 2001
- Genitourinary prolapse and joint hypermobility in womenObstetrics & Gynecology, 1995
- Platelet and coagulation studies in Ehlers-Danlos syndromeBritish Journal of Dermatology, 1991
- Insufficient effect of local analgesics in Ehlers Danlos type III patients (connective tissue disorder)Acta Anaesthesiologica Scandinavica, 1990