What are the current management strategies for patients with Ehlers-Danlos Syndromes experiencing joint pain?

Management of joint pain in patients with Ehlers-Danlos Syndromes (EDS) primarily involves a multidisciplinary approach focusing on symptom relief, functional improvement, and prevention of complications. Initial strategies include referral to physiotherapy, occupational therapy, and podiatry to provide tailored exercise programmes that emphasize muscle strengthening, joint stability, and aerobic fitness, alongside advice on pacing activities to avoid overuse injuries and joint subluxations ¹. Use of protective joint supports such as braces, splints, or gloves may help reduce joint load and instability, thereby alleviating pain ¹.

Pharmacological management should be cautious; paracetamol may be used for short-term pain relief, but its efficacy is limited, and oral NSAIDs can be considered if tolerated, with awareness of their gastrointestinal, renal, and cardiovascular risks ³,⁴. Topical NSAIDs are preferred in some cases due to a better safety profile ³. Codeine may be considered infrequently for short-term relief if other analgesics are ineffective or contraindicated ³. Intra-articular corticosteroid injections might be used selectively to support therapeutic exercise, although their benefit is typically short-term ².

Education and behavioural approaches are integral, including pain coping skills training, goal setting, and advice on activity modification to manage chronic pain and prevent flare-ups ⁴,⁶. Assistive devices and home/work adaptations should be considered to improve function and reduce joint stress ³,⁴.

Referral to specialist services, such as rheumatology or pain management clinics, is recommended when there is severe hypermobility impacting daily activities, diagnostic uncertainty, or presence of red flag features such as vascular complications or significant functional impairment ¹. Chronic pain management principles apply, including multidisciplinary pain management programmes that combine physical and psychological components ⁶.

Recent literature highlights the complexity of pain in EDS, often involving neuropathic and central sensitization mechanisms, suggesting that management may require a broader approach including neurological assessment and tailored pain management strategies beyond conventional analgesics (Chopra et al., 2017). Neurological and spinal manifestations may contribute to pain and disability, warranting comprehensive evaluation (Henderson FC Sr et al., 2017).