Horner syndrome as a manifestation of an exacerbation of Takayasu’s arteritis

A 14 year old female was diagnosed at the age of 11 years with Takayasu’s arteritis after developing signs and symptoms of acute congestive heart failure while receiving fluid resuscitation for dehydration. She had a history of mild fatigue, decreased endurance, and symptoms of claudication. She was found to have complete occlusion of the superior mesenteric artery and involvement of the celiac, splenic, hepatic, right subclavian, and right renal arteries along with cardiomyopathy. She was treated with prednisone, methotrexate and infliximab with improvement in her symptoms, and stabilization of her cardiomyopathy without signs of progressive vasculitis on serial magnetic resonance imaging studies (MRI/MRA). Shewas in stable condition two years after her diagnosis, treated with infliximab every 6 weeks and methotrexate, when she developed left shoulder pain and left conjunctival injection. On ophthalmologic exam, she was noted to have episcleritis of the left eye as well as ptosis of her left upper eyelid and miosis of her left pupil without anhidrosis (Horner syndrome). An MRI/MRA of her brain and neck revealed stable narrowing of her right subclavian but no masses, no new aneurysms or stenoses, and no evidence of new overt inflammation. Her ESR was 16 mm/h and CRP 0.6 mg/dL (prior baseline 15-17 mm/h and 0.3-0.6 mg/dL, respectively). Given the lack of anhidrosis, her lesion was localized to the superior cervical ganglion. In the absence of other causes, and with the concurrent development of episcleritis, the Horner syndrome was presumed secondary to an exacerbation of vasculitis. Prednisone was started at 40mg orally once daily, followed by a slow taper, and infliximab and methotrexate were continued. Within 1 week the episcleritis resolved, and within 3 weeks, the ptosis and miosis had nearly completely resolved.