Heerfordt Syndrome

Heerfordt syndrome is a rare manifestation of sarcoidosis. Sarcoidosis (also known as Besnier-Boeck-Schaumann disease) is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata.

Causes of Heerfordt Syndrome #

• The exact cause of Heerfordt syndrome has not yet been definitively determined.

• Of those patients who have been diagnosed with Heerfordt syndrome, 15% have a close relative who also has the syndrome.

• One possible explanation is that the syndrome results from a combination of an environmental agent and a hereditary predisposition.

• Mycobacterium and Propionibacteria species have both been suggested as the environmental agent, though the evidence for this is inconclusive.

Symptoms of Heerfordt Syndrome #

The symptoms include inflammation of the eye (uveitis), swelling of the parotid gland, chronic fever, and in some cases, palsy of the facial nerves.

Diagnosis of Heerfordt Syndrome #

• In patients that have already been diagnosed with sarcoidosis, Heerfordt syndrome can be inferred from the major symptoms of the syndrome, which include parotitis, fever, facial nerve palsy and anterior uveitis.

• In cases of parotitis, ultrasound-guided biopsy is used to exclude the possibility of lymphoma.

• There are many possible causes of facial nerve palsy, including Lyme disease, HIV, Melkersson–Rosenthal syndrome, schwannoma, and Bell’s palsy.

• Heerfordt syndrome exhibits spontaneous remission.

Treatment of Heerfordt Syndrome #

Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs.