Understanding hypermobile Ehlers-Danlos syndrome, and hypermobility spectrum disorder
previously known as Ehlers-Danlos syndrome hypermobility type, and joint hypermobility syndrome
Contents page
Chapter 1 - Frequently asked questions
Introduction
Hypermobility
Heritable Disorders of Connective Tissue
Ehlers-Danlos syndrome
Hypermobile Ehlers-Danlos syndrome
Genetics and hypermobile Ehlers-Danlos syndrome
Joint hypermobility syndrome - where does it fit into the picture?
EDS classification history
EDS international classification 2017
Introducing ‘hypermobility spectrum disorder‘
Concerns arising from the 2017 classification
Why are people so differently affected?
Are hEDS or HSD progressive?
Chapter 2 - Symptoms & comorbidities
Symptoms, manifestations, syndromes and comorbidities
Understanding the layout of this chapter
Symptoms Part 1 - Widespread symptoms and comorbidities
Joint hypermobility
Recurrent musculoskeletal disorders
Hyperextended alignment of joints
Pain
Complex regional pain syndrome
Fatigue
Proprioceptive dysfunction
Muscle stiffness and tightness
Habitual cracking of joints
Frequent dislocations or subluxations
Osteoarthritis
Osteopenia
Osteoporosis
Skin involvement
Local anaesthetic
Anxiety
Cardiovascular autonomic dysfunction
Mast cell activation syndrome
Raynaud’s phenomenon and chilblains
Fibromyalgia
Symptoms Part 2 - Area specific symptoms and comorbidities
Headaches
Ocular features
The nose and throat
Asthma-like symptoms
Dental features
Chronic neck strain
The shoulders
The chest and ribs
The heart
The elbow
The wrist and hand
The spine
The bony pelvis
The hip
Gastrointestinal dysfunction
Contraception, pregnancy and childbirth
Urogynaecological symptoms
Male urinary tract dysfunction
Restless leg syndrome
Disorders of the knee
Disorders of the foot and ankle
Chapter 3 - Diagnosis and management of hEDS and HSD
IInitial diagnosis by clinical assessment
A note to clinicians
Quick questions designed to assist GPs
The 2017 International Diagnositic Criteria for Hypermobile Ehlers-Danlos Syndrome
The Beighton Score to diagnose generalised joint hypermobility
Reaching a diagnosis
The 2017 Diagnostic Framework for Hypermobility Spectrum Disorder
Diagnosing children and adolescents
The 2023 Diagnostic Criteria for Paediatric Joint Hypermobility
Paediatric Classification Categories
Summary of investigations and management of HSD and hEDS
What might a patient expect at a rheumatology clinical assessment?
An overview of services that may be involved in patient care
Management strategy and multi-disciplinary referral
Why patients must be assessed on an individual basis
Physiotherapy
Occupational therapy
Joint protection
Splinting, bracing, taping, compression ware, and orthotics
Relief of severe pain, and using painkillers effectively
Self-management
Cognitive behavioural therapy
Complementary and physical therapies
Making the most of your health care team
Dental management in hEDS and HSD
Surgical issues
Bibliography
Image Attribution
The 2017 classification criteria for 8 of the 13 Ehlers-Danlos syndrome subtypes
EDS Types Chart
