So far, reports show that uveoparotid fever affects only about 0.3% of all patients suffering from sarcoidosis [1]. Furthermore, very few patients with uveoparotid fever have been described in the literature, but the clinical picture is well-established [2] [3] [4] [5]. Designated as an atypical clinical manifestation of sarcoidosis, uveoparotid fever presents with the following signs: parotid gland enlargement, anterior uveitis, fever, and cranial nerve palsy [1] [2] [3] [4] [5] [6]. Involvement of the parotid gland is rare and often develops in a bilateral fashion, which is also the case for uveitis, manifesting as blurred vision [2] [3]. Some authors also describe various forms of uveitis, such as panuveitis, granulomatous uveitis, myodesopsia, all frequently accompanied by eyelid swelling [5]. Fever is virtually always present and is one of the main symptoms seen in typical sarcoidosis (along with respiratory symptoms, joint pain, and weight loss) [1] [2] [3] [6]. Cranial nerve palsy predominantly targets the facial nerve either unilaterally or bilaterally (a facial droop is usually observed) but cases with a trigeminal nerve palsy have also been reported [1] [2] [3] [5] [6]. In addition, earlier reports noted weight loss and night sweats as features for uveoparotid fever, but these manifestations haven't been recognized in a significant number of individuals in more recent publications [5] [6]. Uveoparotid fever, generally diagnosed in patients between the second and fourth decade of life, is generally viewed as a self-limiting condition [1], but an early diagnosis undoubtedly facilitates better therapeutic response.

• Fever

  Designated as an atypical clinical manifestation of sarcoidosis, uveoparotid fever presents with the following signs: parotid gland enlargement, anterior uveitis, fever, and cranial nerve palsy. [symptoma.com]

  […] uveoparotid fever The new mediacal dictionary. 2014. [medical_en.enacademic.com]

  […] uveoparotid fever uveo·pa·rot·id fever .yü-vē-ō-pə-'rät-əd- n chronic inflammation of the parotid gland and uvea marked by low-grade fever, lassitude, and bilateral iridocyclitis and sometimes associated with sarcoidosis called also Heerfordt's syndrome [medicine.academic.ru]

  Synonym(s): uveoparotid fever Heerfordt syndrome u·ve·o·pa·rot·id fe·ver ( yū'vē-ō-păr-ot'id fē'vĕr ) Chronic enlargement of the parotid glands and inflammation of the uveal tract accompanied by a long-continued fever of low degree; a form of sarcoidosis [medical-dictionary.thefreedictionary.com]

  None source_ontology Experimental Factor Ontology submitted_by no view permissions synonyms Heerfordt's syndrome (disorder) Uveoparotid Fever Uveoparotid fever term_id EFO:1001232 term_name uveoparotid fever term_url http://www.ebi.ac.uk/efo/EFO_1001232 [testportal.4dnucleome.org]

• Plethora

  Several physicians were involved in its discovery and characterisation.[2]Hence, it has a plethora of eponymous names. It forms a small proportion (<5%) of sarcoidosis cases, with variation in its incidence across different ethnic groups. [patient.info]

• Pharyngeal Edema

  Esophagogastroduodenoscopy showed pharyngeal edema but no evidence of obstruction. [ncbi.nlm.nih.gov]

• Parotid Swelling

  Parotid swelling May be unilateral or bilateral - bilateral in 73% of cases of parotid sarcoidosis.[3] Diffuse, painless swelling of the parotid ± other salivary glands/lacrimal glands. May cause a dry mouth. [patient.info]

  Sarcoidosis can cause swelling of the parotid gland and sometimes facial-nerve palsy. Heerfordt’s uveoparotitis is a manifestation of sarcoidosis that occurs when the lacrimal gland, uveal tract and parotid gland are involved. [clinicaladvisor.com]

  Sialosis: 35 cases of persistent parotid swelling from two countries. [medicine.uiowa.edu]

  A 53-year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland. [hindawi.com]

  When the temperature had been normal for a couple of days, bilateral swelling of the parotid occurred; the swelling was shar1)ly outlined, without peri-edema, of firm consistence, and quite indo- lent. [docslide.com.br]

• Normal Hearing

  A pure tone audiogram showed normal hearing and stapedial reflex on the right side was normal. The patient did not complain of impaired taste. [hindawi.com]

• Lagophthalmos

  Prednisone dosages of 20 to 40 mg/day (the dosage may be up to 80 mg/ day in some cases) are prescribed over six to 12 months.6 Artificial tears are useful when facial paralysis with lagophthalmos is present. [docksci.com]

  Prednisone dosages of 20 to 40 mg/day (the dosage may be up to 80 mg/day in some cases) are prescribed over six to 12 months.6 Artificial tears are useful when facial paralysis with lagophthalmos is present. [scielo.br]

• Lacrimal Gland Enlargement

  The fever may be accompanied by dry eyes, diminished tear production (lacrimation), and inflammation of various parts of the eyes (uveitis). Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs. [rarediseases.org]

• Unilateral Facial Weakness

  The principal vascular causes of unilateral facial weakness are ischemic stroke and intraparenchymal hemorrhage involving the cranial nerve VII nucleus. [ncbi.nlm.nih.gov]

• Hyperalgesia

  There may be associated hyperalgesia, papilloedema, meningism and pleocytosis on the cerebrospinal fluid due to sarcoidosis. Other clinical features may include hilar adenopathy, generalized lymphadenopathy, visceral lesion and skin rash. [whonamedit.com]

• Radiculopathy

  Radiculopathy is also a rare manifestation in patients with neurosarcoidosis. A literature review revealed that only 51 cases of radiculopathy associated with sarcoidosis have been documented. [ncbi.nlm.nih.gov]

Because of the rarity of uveoparotid fever, the diagnosis may be difficult to make, especially if the primary symptoms of sarcoidosis are absent. For this reason, the physician must conduct a thorough clinical assessment, starting with detailed patient history, that will cover the onset of symptoms, duration, as well as progression. During the physical examination, neurological testing and a thorough ear, nose, and throat (ENT) exam can identify the main signs of uveoparotid fever - parotid gland enlargement, cranial nerve palsy, and uveitis. Thus, in the absence of specific tests, the diagnosis is predominantly made on clinical grounds [2] [4] [5] [6]. To confirm sarcoidosis as the underlying cause of symptoms, several tests need to be employed. High values of angiotensin-converting enzyme (ACE) is a highly useful finding during laboratory workup and it is assumed that granulomatous inflammation induces the production of this enzyme [2] [6]. If sufficient evidence is obtained, a lymph node biopsy should be conducted and a histological examination showing non-caseating granulomatous inflammation yields a definite diagnosis of sarcoidosis and uveoparotid fever [2] [5] [6].

• Granulomatous Tissue

  Microscopy with hematoxylin and eosin stain revealed tissue composed of salivary gland acini replaced by non-caseating granulomatous tissue. [journalgateway.com]

Oral corticosteroids represent the first line treatment option. [docksci.com]

The standard treatment for Heerfordt’s syndrome has not yet been established due to the rarity of this syndrome. [hindawi.com]

The format includes the CPT codes for billing purposes, short description of the condition, etiology/incidence, course/prognosis, laboratory findings, differential diagnosis; prophylaxis, treatment (local and systemic, surgical or other), miscellaneous [books.google.com]

Treatment and prognosis The constellation of symptoms along with blood samples and biopsy usually allows the diagnosis. [radiopaedia.org]

The degree of involvement of sarcoidosis at other sites will influence the overall prognosis. [patient.info]

The format includes the CPT codes for billing purposes, short description of the condition, etiology/incidence, course/prognosis, laboratory findings, differential diagnosis; prophylaxis, treatment (local and systemic, surgical or other), miscellaneous [books.google.com]

Electrophysiological tests, including electroneurography (ENoG) and the nerve excitability test (NET), showed no severe damage to the facial nerve, which indicated a good prognosis for the palsy. [hindawi.com]

Since such structural changes are not a specific sonographic criterion for sarcoidosis, ultrasound-guided biopsy was performed for further clarification of the etiology of parotitis and for excluding lymphoma. [ncbi.nlm.nih.gov]

From Etiology may be initial presentation of sarcoidosis [2] Pathology parotid gland biopsy (image) no necessary scattered granulomas with focal central necrosis stains for acid-fast bacilli & fungi negative histopathology image ( parotid ) [3] compression [anvita.info]

The format includes the CPT codes for billing purposes, short description of the condition, etiology/incidence, course/prognosis, laboratory findings, differential diagnosis; prophylaxis, treatment (local and systemic, surgical or other), miscellaneous [books.google.com]

Define seronegative spondyloarthropathies (genetic associations and epidemiology) Name the 3 diseases in this category Arthritis without RF Strong association with HLA B27 (codes for MHC I) Occurs more in males Anklyosing spondylitis, Reiter's syndrome [cram.com]

and the Department of Biostatistics and Research Epidemiology, Henry Ford Health System, Detroit (B.A.R.). Address reprint requests to Dr. [doi.org]

Epidemiology Heerfordt's syndrome is relatively rare. [patient.info]

Global epidemiology of sarcoidosis. What story do prevalence and incidence tell us? Clin Chest Med. 1997;18:681-94. 4. Reich JM, Johnson R. Incidence of clinically identified sarcoidosis in a northwest United States population. [journalgateway.com]

Pathophysiology The exact etiology of sarcoidosis is unknown, making it largely a diagnosis of exclusion. [eyewiki.aao.org]

One American study reported that of all deaths reported in the period 1988-2007, 0.05% listed sarcoidosis as the primary cause on the death certificate.[9] Prevention The cause of sarcoidosis is not really understood so primary preventative strategies [patient.info]

Association with sarcoidosis was observed by the Swedish internist Jan Gösta Waldenström (1906-1996) in 1937 6. misdiagnosing facial paralysis as simple Bell palsy prevents possible treatment of the underlying systemic disorder 2-4 [radiopaedia.org]

Isolation from other nonimmune individuals will prevent further spread. Although serious, sterility from orchitis is a rare complication of mumps. Noninfectious inflammatory disorders can also cause enlargement of the salivary glands. [clinicaladvisor.com]

When complete eye closure is not possible, the patient should use artificial tears while awake, use lubricant eye ointment at night, and cover the affected eye during sleep to prevent exposure keratopathy. [ncbi.nlm.nih.gov]

1. Fujiwara K, Furuta Y, Fukuda S. Two Cases of Heerfordt’s Syndrome: A Rare Manifestation of Sarcoidosis. Case Rep Otolaryngol. 2016;2016:3642735.
2. Denny MC, Fotino AD. The Heerfordt-Waldenström syndrome as an initial presentation of sarcoidosis. Proc (Bayl Univ Med Cent). 2013;26(4):390-392.
3. Koyama T, Ueda H, Togashi K, et al. Radiologic manifestations of sarcoidosis in various organs. Radiographics. 2004;24(1):87-104.
4. Dua A, Manadan A. Images in clinical medicine. Heerfordt's syndrome, or uveoparotid fever. N Engl J Med. 2013;369(5):458.
5. McGee J, Minagar A. Heerfordt’s Syndrome: An interesting and under-recognised manifestation of sarcoidosis. Sultan Qaboos Univ Med J. 2015;15(1):e7-e8.
6. Chappity P, Kumar R, Sahoo AK. Heerfordt’s Syndrome Presenting with Recurrent Facial Nerve Palsy: Case report and 10-year literature review. Sultan Qaboos Univ Med J. 2015;15(1):e124-e128.