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Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder.
Yael Gazit1, Giris Jacob1,2, Rodney Grahame3
1Internal Medicine F and the Institute of Rheumatology, Tel Aviv Sourasky Medical Center, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Rambam Maimonides Medical Journal
|November 9, 2016
Summary
Ehlers-Danlos syndrome-hypermobility type (EDS-HT), the most common EDS subtype, causes joint instability and pain. This review details its widespread effects beyond joints, emphasizing the need for better recognition and management to prevent disability.
Area of Science:
- Rheumatology and Genetics
- Connective Tissue Disorders
Background:
- Ehlers-Danlos syndrome-hypermobility type (EDS-HT) is the most prevalent and typically least severe EDS subtype, often equated with joint hypermobility syndrome.
- It primarily presents with musculoskeletal issues like joint pain, instability (leading to dislocations/subluxations), and soft tissue injuries.
Approach:
- This review synthesizes current knowledge on EDS-HT.
- It focuses on the diverse range of extra-articular manifestations across multiple organ systems.
Key Points:
- EDS-HT involves significant joint instability and chronic pain, contributing to high disability potential.
- Extra-articular manifestations are extensive, affecting cardiovascular, neurological, gastrointestinal, and other systems, underscoring its multisystemic nature.
- Under-recognition and inadequate management of EDS-HT are prevalent, leading to potentially avoidable patient neglect and severe disability.
Conclusions:
- EDS-HT is a complex multisystemic disorder requiring comprehensive diagnostic and management strategies.
- Early and accurate diagnosis is crucial to mitigate the significant disability associated with this condition.
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