Hypermobile Ehlers-Danlos Syndrome Clinical Manifestations

Core Musculoskeletal Manifestations

The American College of Rheumatology suggests that joint hypermobility is confirmed by Beighton score ≥5/9 points for adults under 50 years, ≥4/9 for those over 50, and ≥6/9 for prepubertal children 2, 3
Recurrent joint dislocations or subluxations affect multiple joints, particularly shoulders, patellae, and digits, with a significant impact on patient mobility and quality of life 3
Easy bruising without significant trauma is a common feature of hEDS, affecting patients' daily activities and overall well-being 3

The International Foundation for Gastrointestinal Disorders recommends that nausea and abdominal pain be recognized as predictive symptoms of abnormal GI motility in hEDS patients 1
Severe constipation, bloating, diarrhea, and vomiting are common gastrointestinal symptoms in hEDS patients, affecting up to 98% of the population 1
Early satiety suggesting delayed gastric emptying is a significant concern in hEDS patients, impacting nutritional intake and overall health 1
Gastroesophageal reflux and gastritis are prevalent in hEDS patients, requiring appropriate management and treatment 5

The American Heart Association notes that aortic root dilation occurs in 25-33% of hypermobile EDS cases, highlighting the need for regular cardiovascular monitoring 4, 3
Postural Orthostatic Tachycardia Syndrome (POTS) is characterized by a heart rate increase ≥30 beats/min within 10 minutes of standing in adults, and ≥40 beats/min in adolescents 12-19 years, affecting patients' daily activities and quality of life 1, 2
Palpitations and tremulousness are common cardiovascular symptoms in hEDS patients, requiring prompt medical attention 2

The Autonomic Nervous System Society recommends that orthostatic intolerance with lightheadedness and generalized weakness be recognized as a significant symptom of autonomic dysfunction in hEDS patients 1, 2
Blurred vision upon standing is a common symptom of autonomic dysfunction in hEDS patients, affecting daily activities and overall well-being 2
Cognitive dysfunction (brain fog) is a significant concern in hEDS patients, impacting daily functioning and quality of life 1
Bladder dysfunction is a common feature of autonomic dysfunction in hEDS patients, requiring appropriate management and treatment 1

The Mastocytosis Society notes that flushing and urticaria are common symptoms of Mast Cell Activation Syndrome (MCAS) in hEDS patients, requiring prompt medical attention 5
Wheezing and respiratory symptoms are prevalent in hEDS patients with MCAS, affecting patients' daily activities and overall well-being 4
Multisystem symptoms triggered by foods, alcohol, strong smells, temperature changes, and specific medications are a significant concern in hEDS patients with MCAS, requiring careful management and treatment 5
Up to 40% of patients report a viral infection as the precipitating event for hEDS symptoms, highlighting the need for prompt medical attention and treatment 1