How to manage fibromyalgia symptoms in patients with hypermobile Ehlers-Danlos Syndrome (hEDS)?

Managing Fibromyalgia Symptoms in Hypermobile Ehlers-Danlos Syndrome (hEDS)

Fibromyalgia is a distinct condition characterized by widespread pain and other symptoms that frequently co-occurs with hypermobile Ehlers-Danlos Syndrome (hEDS), requiring specific management strategies tailored to address both conditions simultaneously.

Understanding the Relationship Between hEDS and Fibromyalgia

• There is significant overlap between hEDS/HSD and fibromyalgia, with studies showing 68-88.9% of patients with hEDS/HSD also meeting criteria for fibromyalgia 1
• Patients diagnosed with both hEDS/HSD and fibromyalgia typically experience more severe symptoms than those with either condition alone 2
• While related, fibromyalgia and hEDS are distinct conditions with different underlying pathophysiologies, though they share many clinical manifestations 3

Non-Pharmacological Management Approaches

Exercise Therapy

• Aerobic and strengthening exercise should be the first-line treatment for fibromyalgia symptoms in hEDS patients, with strong evidence supporting its effectiveness 4
• Low-resistance exercise is particularly recommended for hEDS patients to improve joint stability by increasing muscle tone while minimizing injury risk 5
• Physical therapy with myofascial release techniques should be incorporated to facilitate participation in exercise programs for hEDS patients with fibromyalgia symptoms 5

Cognitive and Behavioral Approaches

• Cognitive behavioral therapy (CBT) is recommended for managing fibromyalgia symptoms in hEDS patients 4
• Multicomponent therapies combining exercise, education, and psychological approaches show benefit for fibromyalgia symptoms 4

Other Non-Pharmacological Options

• Acupuncture and hydrotherapy have weak evidence supporting their use for fibromyalgia symptoms 4
• Meditative movement therapies (yoga, tai chi, qigong) and mindfulness-based stress reduction may provide benefit 4
• For patients with comorbid POTS (common in hEDS), increased fluid and salt intake, exercise training, and compression garments are recommended 4, 5

Pharmacological Management

First-Line Medications

• Low-dose amitriptyline is recommended as a first-line pharmacological treatment for fibromyalgia symptoms in hEDS 4
• Duloxetine or milnacipran are also recommended first-line options with good evidence for fibromyalgia pain management 4, 6
• Pregabalin is recommended for fibromyalgia pain with evidence showing effectiveness at doses of 300-450 mg/day 4, 7

Second-Line Medications

• Cyclobenzaprine has weak evidence supporting its use for fibromyalgia symptoms 4
• Tramadol may be considered with caution for short-term use in specific cases 4

Medications to Avoid

• Opioids should be avoided for chronic pain management in hEDS patients with fibromyalgia symptoms 4, 5
• For patients already prescribed opioids, a careful multidisciplinary approach to facilitate opioid cessation is recommended 4

Managing Comorbid Conditions

Autonomic Dysfunction (POTS)

• For patients with comorbid POTS, consider pharmacological treatments for volume expansion, heart rate control, and vasoconstriction when conservative measures fail 4, 5
• Earlier testing of gastric motor functions should be considered in hEDS patients with POTS who report chronic upper GI symptoms 4

Mast Cell Activation Syndrome (MCAS)

• When MCAS is suspected in hEDS patients with fibromyalgia symptoms, consider treatment with histamine receptor antagonists and/or mast cell stabilizers 4, 5
• Advise patients to avoid triggers such as certain foods, alcohol, strong smells, temperature changes, and specific medications 4, 5

Multidisciplinary Approach

• A multidisciplinary approach involving rheumatology, pain management, physical therapy, psychology, and other specialties as needed is essential 4, 8
• Treatment should focus on the most prominent symptoms while addressing both conditions simultaneously 4
• Special diets (including gastroparesis diet and various elimination diets) can be considered with appropriate nutritional counseling to avoid restrictive eating 4

Common Pitfalls to Avoid

• Failing to recognize the high prevalence of fibromyalgia in hEDS patients (up to 81% in some studies) 3
• Treating only one condition while ignoring the other, leading to suboptimal outcomes 1
• Overreliance on opioids for pain management, which can worsen symptoms long-term 4, 5
• Implementing overly restrictive diets without proper nutritional guidance 4
• Neglecting to screen for and manage comorbid conditions like POTS and MCAS that can exacerbate symptoms 4, 9