Heerfordt's syndrome, also referred to as uveoparotid fever, Heerfordt-Mylius syndrome, Heerfordt-Waldenström syndrome, and Waldenström’s uveoparotitis, is a rare manifestation of sarcoidosis

Sarcoidosis

Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...

. The symptoms include inflammation of the eye (uveitis

Uveitis

Uveitis specifically refers to inflammation of the middle layer of the eye, termed the "uvea" but in common usage may refer to any inflammatory process involving the interior of the eye....

), swelling of the parotid gland

Parotid gland

The paired parotid glands are the largest of the salivary glands. They are each found wrapped around the mandibular ramus, and secrete saliva through Stensen's ducts into the oral cavity, to facilitate mastication and swallowing and to begin the digestion of starches.-Location:The parotid glands...

, chronic fever

Fever

Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...

, and in some cases, palsy

Palsy

In medicine, palsy is the paralysis of a body part, often accompanied by loss of sensation and by uncontrolled body movements, such as shaking. Medical conditions involving palsy include cerebral palsy , brachial palsy , and Bell's palsy ....

 of the facial nerve

Facial nerve

The facial nerve is the seventh of twelve paired cranial nerves. It emerges from the brainstem between the pons and the medulla, and controls the muscles of facial expression, and functions in the conveyance of taste sensations from the anterior two-thirds of the tongue and oral cavity...

s.

History

The condition was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named. It was originally attributed to mumps

Mumps

Mumps is a viral disease of the human species, caused by the mumps virus. Before the development of vaccination and the introduction of a vaccine, it was a common childhood disease worldwide...

, but after further studies by Swedish doctor Jan G. Waldenström

Jan G. Waldenström

Jan Gösta Waldenström was a Swedish doctor of internal medicine, who first described the disease which bears his name, Waldenström's macroglobulinemia....

 in 1937, it was classified as a distinct manifestation of sarcoidosis

Sarcoidosis

Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...

.

Causes

The exact cause of Heerfordt's syndrome has not yet been definitively determined. Of those patients who have been diagnosed with Heerfordt's 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

Mycobacterium

Mycobacterium is a genus of Actinobacteria, given its own family, the Mycobacteriaceae. The genus includes pathogens known to cause serious diseases in mammals, including tuberculosis and leprosy...

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

Prevalence

In the United States, sarcoidosis has a prevalence

Prevalence

In epidemiology, the prevalence of a health-related state in a statistical population is defined as the total number of cases of the risk factor in the population at a given time, or the total number of cases in the population, divided by the number of individuals in the population...

 of approximately 10 cases per 100,000 whites

White American

White Americans are people of the United States who are considered or consider themselves White. The United States Census Bureau defines White people as those "having origins in any of the original peoples of Europe, the Middle East, or North Africa...

 and 36 cases per 100,000 blacks

African American

African Americans are citizens or residents of the United States who have at least partial ancestry from any of the native populations of Sub-Saharan Africa and are the direct descendants of enslaved Africans within the boundaries of the present United States...

. Heerfordt's syndrome is present in approximately 0.3% of those with sarcoidosis.

Diagnosis and treatment

In patients that have already been diagnosed with sarcoidosis, Heerfordt's syndrome can be inferred from the major symptoms of the syndrome, which include parotitis

Parotitis

Parotitis is an inflammation of one or both parotid glands, the major salivary glands located on either side of the face, in humans. The parotid gland is the salivary gland most commonly affected by inflammation.-Infectious parotitis:...

, fever, and facial nerve palsy. In cases of parotitis, ultrasound

Medical ultrasonography

Diagnostic sonography is an ultrasound-based diagnostic imaging technique used for visualizing subcutaneous body structures including tendons, muscles, joints, vessels and internal organs for possible pathology or lesions...

-guided biopsy

Biopsy

A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...

 is used to exclude the possibility of lymphoma

Lymphoma

Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...

. There are many possible causes of facial nerve palsy, including Lyme disease

Lyme disease

Lyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...

, HIV

HIV

Human immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...

, Melkersson–Rosenthal syndrome, schwannoma

Schwannoma

A schwannoma is a benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves....

, and Bell's palsy

Bell's palsy

Bell's palsy is a form of facial paralysis resulting from a dysfunction of the cranial nerve VII that results in the inability to control facial muscles on the affected side. Several conditions can cause facial paralysis, e.g., brain tumor, stroke, and Lyme disease. However, if no specific cause...

. Heerfordt's syndrome exhibits spontaneous remission

Spontaneous remission

Spontaneous healing, also called spontaneous remission or spontaneous regression, means an unexpected improvement or cure from a disease which usually is taking a different course. Both terms are mainly used for unexpected transient or final improvements in cancer. Spontaneous remissions concern...

. Treatments for sarcoidosis include corticosteroid

Corticosteroid

Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...

s and immunosuppressive drug

Immunosuppressive drug

Immunosuppressive drugs or immunosuppressive agents are drugs that inhibit or prevent activity of the immune system. They are used in immunosuppressive therapy to:...

s.