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A6492 - Heerfordts Syndrome and Hypercalcemia as the Presenting Features of Sarcoidosis
Author Block: D. Lane1, I. Wong2, L. Gross3; 1New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, United States, 2Medicine, New York Methodist Hospital, Brooklyn, NY, United States, 3Pulmonary, Critical Care and Sleep Medicine, Brooklyn, NY, United States.
Introduction
Heerfordt’s syndrome, also referred to as uveoparotid fever, is a rare presentation of sarcoidosis that constitutes approximately 0.3% of all cases. The syndrome is characterized by parotid gland enlargement, anterior uveitis, facial nerve palsy, and fever. When three of the four symptoms are present, it is considered an incomplete form of Heerfordt’s syndrome. Here we are presenting a case of incomplete Heerfordt’s syndrome.
Case presentation
A 46 year old female presented to us after a syncopal episode. She felt dizzy in the shower and then lost consciousness for approximately one minute. There was no trauma, and no evidence of seizure-like activity. On additional questioning, She also complained of bilateral eye discomfort, itching, and redness that would occur on and off for the last two to three weeks. Her initial vital signs were significant for a fever of 100.8 F. On examination she had bilateral parotid swelling, cervical adenopathy and normal appearing conjunctiva. Her neurological exam was normal with no facial nerve palsy noted. Initial labs were notable for a calcium level of 15 mg/dL. Given concerns for an infectious process, a CT of the neck and chest was performed with contrast, revealing nonspecific parotid enlargement and significant mediastinal lymphadenopathy with multiple lower lobe nodules. A bronchoscopy with endobronchial ultrasound was then performed to confirm the suspected diagnosis of sarcoidosis. On bronchoscopy she was noted to have extensive endobronchial studding and EBUS-TBNA of station 7 was performed. Pathology showed non-necrotizing epithelioid granulomata, negative acid-fast bacilli and fungal stains, and so a final diagnosis of sarcoidosis was made. She was started on systemic steroids and within one week of therapy demonstrated excellent response.
Discussion
Sarcoidosis is a chronic granulomatous disease of unknown etiology that can present in many ways. We described a case in which our patient presented as incomplete Heerfordt’s syndrome with the absence of cranial nerve palsy. Only 5% of sarcoidosis cases have neurologic involvement, with the facial nerve being the most commonly affected of the cranial nerves. Parotid gland swelling is observed in 6% of cases, is bilateral in the majority of patients, and can cause a facial nerve palsy due to compression. Ocular involvement occurs in up to 25% of sarcoidosis patients, with anterior uveitis being the most common finding. Heerfordt's syndrome, while not pathognomonic for sarcoidosis, encompases unique extrapulmonary manifestations of the disease that should direct the clinician toward a diagnosis.