Heerfordt's syndrome

Heerfordt's syndrome, also known as Heerfordt-Waldenström syndrome or uveoparotid fever, is a rare disease characterized by the combination of parotitis (inflammation of the parotid gland), anterior uveitis (inflammation of the uvea in the eye), facial nerve palsy, and sometimes fever. It is considered a subtype of sarcoidosis, a systemic condition that affects various organs in the body, primarily the lungs and lymphatic system.

Symptoms and Diagnosis

The hallmark symptoms of Heerfordt's syndrome include:

• Parotitis: Swelling of one or both parotid glands, which are major salivary glands located on either side of the face.
• Anterior Uveitis: Inflammation of the uvea, the middle layer of the eye, leading to pain, redness, and vision problems.
• Facial Nerve Palsy: Weakness or paralysis of the facial muscles, often on one side, which can cause drooping of the eyelid and corner of the mouth, and difficulty closing the eye.
• Fever: Some patients may also experience a fever.

Diagnosis of Heerfordt's syndrome involves a combination of clinical evaluation, imaging studies such as MRI or CT scan to assess the parotid glands, and blood tests to check for markers of inflammation and sarcoidosis. A definitive diagnosis may require a biopsy of the affected gland or tissue.

Treatment

Treatment of Heerfordt's syndrome focuses on managing symptoms and reducing inflammation. Options include:

• Corticosteroids: These are the mainstay of treatment, used to reduce inflammation and swelling.
• Immunosuppressive Medications: In cases where corticosteroids are not effective, medications that suppress the immune system may be used.
• Topical Treatments: For eye inflammation, corticosteroid eye drops may be prescribed.

Prognosis

The prognosis for individuals with Heerfordt's syndrome is generally good, especially with prompt and appropriate treatment. Most symptoms can be effectively managed, and the condition often resolves over time. However, since Heerfordt's syndrome is a manifestation of sarcoidosis, monitoring for other potential complications related to sarcoidosis is important.

Epidemiology

Heerfordt's syndrome is a rare condition, and its exact prevalence is unknown. It can occur at any age but is most commonly diagnosed in adults. There is no known gender or racial predilection.

History

The syndrome is named after the Danish physician Christian Frederick Heerfordt, who first described the condition in 1909. Swedish physician Jan Gösta Waldenström later contributed to the understanding of the syndrome, leading to its alternative name, Heerfordt-Waldenström syndrome.