Can Ehlers-Danlos Syndrome (EDS) cause bilateral wrist synovitis?

Bilateral Wrist Synovitis in Ehlers-Danlos Syndrome

Yes, bilateral wrist synovitis can occur in Ehlers-Danlos Syndrome (EDS), particularly in the hypermobility type, due to joint instability and recurrent subluxations that lead to inflammatory changes in the synovial lining of the wrist joints.

Pathophysiology and Presentation

Wrist involvement in EDS occurs through several mechanisms:

• Joint instability: The fundamental collagen defects in EDS lead to ligamentous laxity, causing joint hypermobility and instability, particularly in the wrists 1
• Recurrent subluxations: Patients with EDS-hypermobility type frequently experience subluxations of the midcarpal joints, which can trigger inflammatory responses 2
• Synovial inflammation: Repeated microtrauma from unstable joints can lead to synovitis (inflammation of the synovial membrane) in both wrists

The presentation typically includes:

• Bilateral wrist pain, often worse with activity
• Visible swelling around the wrist joints
• Limited range of motion due to pain and effusion
• Sensation of instability or "giving way" of the wrists

Diagnostic Considerations

When evaluating bilateral wrist synovitis in a patient with EDS:

1. Ultrasound examination: Particularly valuable for detecting synovitis, effusion, and tenosynovitis in the wrist 3

   • Volar transverse and longitudinal scans
   • Dorsal transverse and longitudinal scans (radial, median, ulnar)

2. Differential diagnosis: Important to distinguish from other causes of bilateral wrist synovitis:

   • Rheumatoid arthritis (check for RF and anti-CCP antibodies)
   • Immune checkpoint inhibitor-related arthritis 3
   • Adult-onset Still's disease (which presents with characteristic fever pattern and rash) 3
   • Crystal arthropathies (gout, pseudogout)

3. Laboratory testing: While not diagnostic for EDS-related synovitis, can help rule out other conditions:

   • Inflammatory markers (ESR, CRP) may be elevated but less prominently than in autoimmune arthritis
   • Autoantibodies (RF, anti-CCP) typically negative

Management Approach

Treatment of bilateral wrist synovitis in EDS should focus on:

1. Joint stabilization:

   • Wrist splinting or bracing to reduce subluxations
   • Physical therapy focusing on proprioceptive training and muscle strengthening around the wrist 4

2. Anti-inflammatory measures:

   • NSAIDs for pain and inflammation control
   • Local corticosteroid injections for acute flares (use with caution due to potential tissue fragility)

3. Surgical options (for refractory cases):

   • Tenodesis procedures using extensor carpi radialis brevis strips can stabilize the midcarpal joint 2
   • Careful patient selection is crucial as tissue fragility can complicate surgical outcomes

Important Considerations and Pitfalls

• Avoid aggressive manipulation: Patients with EDS have fragile tissues and excessive manipulation can worsen injury
• Recognize associated conditions: Trapeziometacarpal joint abnormalities are common in EDS (66% have subluxation, 29% have dislocation) 5
• Long-term monitoring: Progressive joint degeneration can occur, with 16% showing radiographic evidence of arthritis 5
• Functional impact: EDS patients often have significant hand strength deficits (71% for tip pinch, 55% for palmar pinch, 46% for key pinch, and 70% for grip) 5

Conclusion

Bilateral wrist synovitis is a recognized manifestation of EDS, particularly in the hypermobility type. The primary mechanism involves joint instability leading to recurrent subluxations and inflammatory changes. Management should focus on joint stabilization, anti-inflammatory measures, and in severe cases, surgical intervention to improve stability and function.