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Heerfordt syndrome
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Heerfordt syndrome

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Contributors: Sanuja Bose BS, BA, Abhijeet Waghray MD
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Synopsis

Heerfordt syndrome (also known as uveoparotid fever and Heerfordt-Waldenström syndrome) is a rare presentation of sarcoidosis comprising parotid gland swelling, uveitis, fever, and, in a minority of cases, facial palsy. Heerfordt syndrome may present with a combination of some or all of these symptoms. "Complete" Heerfordt syndrome, which would involve all symptoms, is only 0.3% of sarcoidosis cases.

The precise cause has not been identified. A minority of patients have a close family member with the syndrome. Most patients present as young or middle-aged adults, although the disease may present at any age. There is some evidence to show Heerfordt syndrome may result from a combination of environmental factors and possibly a hereditary component, but the evidence is insufficient.

Prognosis is dependent on managing the patient's underlying systemic symptoms. The disease course is variable as spontaneous remission may occur in over half of patients, while other patients may require more continuous management with corticosteroids.

Codes

ICD10CM:
D86.89 – Sarcoidosis of other sites

SNOMEDCT:
4416007 –  Heerfordt's syndrome

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Last Reviewed: 06/06/2019
Last Updated: 06/21/2019
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Heerfordt syndrome
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Heerfordt syndrome : Fever, Facial palsy, Hilar lymphadenopathy, Parotid enlargement, Lacrimal gland enlarged, Dry mouth
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