Working Diagnosis:
Arterial Thoracic Outlet Syndrome (aTOS) due to Joint Hypermobility

Treatment:
Dupilumab holiday was attempted for 1 month. The brachial plexus rehab with Physical Therapy was done to optimize thoracic extension and scapulothoracic movement. A 1st rib resection was discussed with vascular surgery and the patient, who opted for conservative management through shared decision-making. An injection consisting of bupivacaine and a steroid was injected into the distal clavicle and surrounding structures by a chronic pain specialist.

Outcome:
The patient's symptoms recurred after 1 month off of Dupilumab. The dermato-rheumatologist recommended that the patient restart the medication.
His return to activity remains restricted from repetitive overhead activities and limited by shoulder pain, which significantly improved but not resolved after a bupivacaine and steroid injection.

Author's Comments:
The patients case was approached using a multidisciplinary team (Sports Medicine, Neurology, Vascular Surgery, Dermatology, Rheumatology) and an extensive workup, identifying aTOS without structural compression or structural abnormality. This suggested an underlying medical etiology that we tried to identify.
We considered Dupilumab, an efficacious medication with expanding applications, as a possible etiology. While generally well-tolerated, it is increasingly recognized for causing spondyloarthritis and joint pain. Typically bilateral and peripheral, joint pain begins within 4 months after initiation of dupilumab and resolves with discontinuation. These inconsistencies with our patients clinical course made Dupilumab-associated arthritis unlikely. One less understood side effect of Dupilumab is autoimmune vasculitis marked by eosinophilia and small vessel damage; our patient also lacked these characteristic findings.
We also considered joint hypermobility as a possible contributor. One study of 50 patients with hypermobility showed about half to have symptoms of TOS. Interestingly, joint hypermobility has also been shown to be strongly associated with various rheumatologic and inflammatory conditions, again adding to the possible interactions with our patients autoimmune disease and treatment.

Editor's Comments:
Symptoms of thoracic outlet syndrome (TOS) affects around 2% of the North American population, and affects women more than men. Around 95% of known TOS cases are Neurogenic TOS (nTOS) is the most common type, followed by venous TOS (4%), and then arterial TOS (1%). This is an interesting case of a very rare presentation of thoracic outlet syndrome.

References:
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