1304 - Vagal nerve stimulation and buprenorphine for a patient with chronic pain secondary to Benign Joint Hypermobility Syndrome

Benign Joint Hypermobility Syndrome (BJHS) is a connective tissue disorder leading to hypermobility and musculoskeletal pain with no systemic rheumatological findings. BJHS has a genetic component and is more common in children, women, and people of African and Asian descent. Mutations in fibrillin have been implicated. Diagnosis is challenging but the Brighton index and a questionnaire by Hakim and Grahame, as well as laboratory tests, to rule out other joint disorders, such as Marfan's, Ehlers-Danlos, Osteogenesis Imperfecta and Juvenile Rheumatoid Arthritis.  

 Self-limited pain in multiple joints is attributed to joint laxity, which increases the risk of joint degeneration, as impaired proprioception, which leads to joint trauma. Extraarticular manifestations of BJHS include gastrointestinal symptoms, autonomic dysfunction, anxiety, depression, skin fragility and laxity, and easy bruising. While there is a relative lack clinical trials regarding treatment, management includes work and lifestyle modification, joint protection, strength training and nonsteroid anti-inflammatory medication.


Case Description: Ms. X is a 49-year-old female nurse with a history of right sided brachial plexus stretch injury and diffuse myalgias with hypermobility in multiple joints. On exam she had diffuse macular erythema, dysesthesias in bilateral upper extremities, as well as thoracic, lumbar, pelvic and left hip pain that radiated into her foot.  She had tried radiofrequency alations, physical therapy, and steroid injections. 

Discussions: Medication included oxycodone ER 30 mg BID and 5 mg for breakthrough pain, as well as diclofenac and Lyrica 100 mg, both contributing to her fatigue and dysphoric mood.  After discussion with the patient, she agreed to induction and down titration of MED on buprenorphine.  We augmented with vagal nerve simulation to the tragus to address autonomic features of her BJHS. At 6-week follow-up, she is stable on buprenorphine 450 mcg q12-24 hours and reports global improvement in pain and daily function.  

Conclusions: Diagnosis and treatment of BJHS can be complex