PRESENTATION: A healthy 18-year-old male with a history of autism presents with periorbital swelling and unintentional weight loss during the preceding 6 months. He and his family report swelling around his eyes, which has not responded to topical and systemic therapies for ocular allergy, including topical ketotifen and olopata-dine as well as loratadine and cetirizine. He denies a history of seasonal allergies, current conjunctival irritation, eye pain, or changes in vision. Since the onset of eye swelling 6 months ago, his father also reports that the patient has unintentionally lost 30 1b. He denies abdominal pain and nausea, his appetite is unchanged, and he has not had any change in his stools. Review of systems is otherwise negative. He has normal vital signs. On examination his bilateral eyelids and area overlying the bilateral lacrimal glands are swollen and mildly ery-thematous but nontender to palpation. He has no evidence of conjunctivitis; his pupils are equal, round, and reactive, and his extraocular movements are intact. He has no lymphadenopathy. He has an otherwise normal examination. Review of previous records reveals that he has had a normal complete metabolic panel, negative antinuclear antibody, elevated erythrocyte sedimentation rate (ESR) of 52 mm/hr (reference range, 0-13 mm/hr), and normal complement (C3/C4) levels since onset of symptoms. He had seen an ophthalmologist, who diagnosed ocular allergy and started therapy for his ocular symptoms with topical antihistamines and corticosteroids. He had no evidence of uveitis on eye examination. He was later prescribed a 6-week taper of empirical systemic corticosteroids with a starting dose of 60 mg daily with instructions to taper the daily dose by 10 mg each week. Once systemic corticosteroids were initiated, his eye swelling resolved and his weight stabilized. In addition, his ESR decreased to 36 mm/hr while taking corticosteroids. After he completed the corticosteroid taper, he had return of the eye swelling, prompting a computed tomographic (CT) scan of his orbits, which showed bilateral prominence of the lacrimal glands (Fig 1).
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