What can be done to support healing and pain reduction in a patient with hypermobile Ehlers-Danlos Syndrome (hEDS) experiencing persistent knee pain?

Management of Persistent Knee Pain in Hypermobile Ehlers-Danlos Syndrome

For hEDS patients with persistent knee pain, initiate a structured physical therapy program focused on quadriceps strengthening exercises (at least 2 days per week at 60-80% of one repetition maximum for 8-12 repetitions), combined with patient education and pain management strategies, as this multimodal approach addresses both joint instability and chronic pain mechanisms specific to this population. 1, 2, 3

Understanding the hEDS Pain Context

hEDS-associated knee pain is multifactorial, stemming from joint instability, recurrent subluxations, and central pain sensitization mechanisms that differ from typical osteoarthritis. 3, 4 The chronic pain in hEDS commonly presents as musculoskeletal pain, fibromyalgia-like symptoms, or neuropathic pain, requiring recognition that standard knee pain protocols may need modification. 4

First-Line Therapeutic Approach

Physical Therapy (Cornerstone of Treatment)

• Quadriceps strengthening exercises are essential and should be performed at least 2 days per week at moderate to vigorous intensity (60-80% of one repetition maximum) for 8-12 repetitions. 1, 5
• Supervised exercise sessions produce superior outcomes compared to unsupervised programs, with 12 or more directly supervised sessions showing better pain reduction (Effect Size 0.46 vs 0.28) and functional improvement (Effect Size 0.45 vs 0.23). 1, 5
• Dynamic strengthening exercises are preferred over isometric exercises based on available evidence. 1
• Exercise programs must be modified based on joint hypermobility severity and individual capabilities to prevent injury from overstretching. 1, 3

Critical Pitfall: Standard physical therapy protocols designed for osteoarthritis may worsen hEDS symptoms if they emphasize stretching over strengthening—the focus must be on joint stabilization through muscle strengthening, not increasing range of motion. 3

Patient Education and Self-Management

• Education about hEDS, joint protection strategies, and self-management has strong evidence for improving pain outcomes and should be initiated immediately. 2, 3
• Teach patients to recognize early signs of joint subluxation and implement protective strategies during daily activities. 3
• Emphasize that hypermobility is not an advantage but requires active muscle stabilization to prevent injury. 3

Pharmacological Pain Management

First-Line Medication

• Acetaminophen (paracetamol) at doses less than 4g/day is the safest oral analgesic for initial pain management. 6, 2
• Topical NSAIDs (such as diclofenac) or capsaicin provide localized pain relief with minimal systemic absorption and are safer alternatives to oral NSAIDs. 2

Second-Line Options

• Oral NSAIDs can be used for acute pain exacerbations but should be combined with nonpharmacologic therapy. 6
• For neuropathic pain components (common in hEDS), consider pregabalin or gabapentin, which have demonstrated efficacy in neuropathic conditions. 6

Interventional Approaches

• Intra-articular corticosteroid injections should be considered for acute pain exacerbations, especially when accompanied by joint effusion, providing targeted therapy with short-term benefit (typically 1-3 months). 6, 2
• Trigger point injections may address myofascial pain components that frequently accompany hEDS. 4
• Peripheral nerve blocks or peripheral nerve stimulation can be considered for refractory cases, though evidence specific to hEDS is limited to case reports. 4

Important Caveat: PRP injections are strongly recommended against by the American College of Rheumatology due to lack of standardization and should only be considered after exhausting all first-line treatments. 5

Adjunctive Interventions

• Appropriate footwear with shock-absorbing insoles can complement exercise interventions to reduce pain and improve function. 1
• Knee sleeves or elastic bandages may provide modest pain relief and proprioceptive feedback to improve joint stability. 2
• Weight reduction for overweight patients significantly improves knee pain and reduces disease progression. 1, 2, 7

Multidisciplinary Coordination

• Psychological support addressing anxiety disorders (common in hEDS) and chronic pain coping strategies should be integrated early, as psychological therapies combined with exercise reduce long-term pain and disability. 6, 3
• Occupational therapy consultation helps with activity modification and joint protection techniques for daily living. 3
• Primary care physicians should coordinate the multidisciplinary team that many hEDS patients require for comprehensive management. 3

Treatment Algorithm Sequence

1. Immediate initiation: Supervised quadriceps strengthening program (12+ sessions) + patient education + acetaminophen or topical NSAIDs as needed. 1, 2, 5
2. Week 4-6 assessment: If inadequate response, add psychological support for pain coping + consider oral NSAIDs for breakthrough pain. 6, 3
3. Month 3 assessment: If persistent pain despite adherence, consider intra-articular corticosteroid injection + continue strengthening exercises. 2
4. Ongoing management: Transition to home-based exercise maintenance program once proper technique established, with periodic supervised sessions for progression. 1, 5

Monitoring and Long-Term Management

• Regular assessment of pain levels, functional capacity, and exercise adherence is essential, as benefits from strengthening exercises persist for 6-18 months with continued participation. 5
• Adjust treatment intensity based on response, recognizing that hEDS patients may have fluctuating symptoms requiring flexible management strategies. 3, 4

Critical Success Factor: The combination of strengthening exercises with pain management produces superior outcomes compared to either approach alone, with multimodal therapies reducing long-term pain and disability more effectively than single interventions. 6, 8