Uveoparotid fever, also known as Heerfordt-Waldenstrom syndrome, is a rare manifestation of sarcoidosis. The clinical presentation encompasses enlargement of the parotid gland (often bilateral), anterior uveitis, fever, and palsy of either the facial or trigeminal nerve. This syndrome is very rare in clinical practice, but through a detailed clinical investigation, the diagnosis is made solely on the presence of characteristic symptoms. The exact pathogenetic pattern connecting sarcoidosis and Heerfordt-Waldenstrom syndrome still remains to be fully understood.
Presentation
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.
Entire Body System
- 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]
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]
Subacute Fever 100+ 0 0.0 Camp Fever 100+ 48.1 0.01 Bandana Fever 100+ 2.78 0.54 Lyme Fever 10+ 0.04 0.06 Tree Fever 10+ 0.93 0.08 Fever Fue 10+ 0 0.08 Water Fever 10+ 0 0.03 Drinks For Fever 10+ 0 0.03 Fever Drink 10+ 1.63 0.02 The Particle Fever 10 [minikeyword.com]
Jew Fever attraction to women with jewish features; the jewish answer to jungle fever and yellow fever... READ MORE fever nightmare A fever nightmare, much like a fever dream, is induced by a fever. [azdictionary.co]
[…] uveoparotid fever The new mediacal dictionary. 2014. [medical_en.enacademic.com]
- Infertility
[…] massage, sialogogic agents, duct probing, antibiotics, mouthwashes, good oral hygiene, and adequate hydration History of Radiation treatment: sialadenitis History of Measles, mumps, rubella vaccine: Mumps associated with sensorineural hearing loss, infertility [medicine.uiowa.edu]
Jaw & Teeth
- 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]
Sialosis: 35 cases of persistent parotid swelling from two countries. [medicine.uiowa.edu]
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]
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]
Skin
- Skin Rash
Other clinical features may include hilar adenopathy, generalized lymphadenopathy, visceral lesion and skin rash. A rare disease which is probably a manifestation of Boeck’s disease. Aetiology is unknown. [whonamedit.com]
Cutaneous forms of the disease may be in many forms ranging from skin rash to scarring caused by severe fibrosis. The heart is affected in approximately 20-30% of all patients but only 5% of these actually experience heart issues. [ic.steadyhealth.com]
- Dermatitis
The special cases are serious hayfever, serious asthma, chronic allergic rhinitis, allergire dermatitis, contact dermatitis, drug interactions andserum disease. [namhanpharma.vn]
[…] of the oral mucosa to the local application of a drug, such as aspirin burns d. oral ulcerations induced as a result of pharmacologic effects of drugs on systemic administration A Drug allergy (drug idiosyncrasy, drug sensitivity, and stomatitis or dermatitis [mdsclasses.com]
Eyes
- 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]
Neurologic
- Confusion
The difference between a fever dream and nightmare is while a fever dream is intense and confusing, a fever nightmare is extremely frightening... READ MORE Fever Junk The stretching and sagging of a male's scrotum while experiencing a fever.... [azdictionary.co]
Diagnostic Considerations Approximately 10% of all infected patients develop a mild case of aseptic meningitis, which initially due to clinical presentation can be confused with bacterial meningitis. [reference.medscape.com]
It is also possible to misdiagnose sarcoidosis and confuse it with tuberculosis, another granulomatous disease with similar symptoms. Finally, there are some changes when it comes to races and sarcoidosis. [ic.steadyhealth.com]
Differential diagnosis The syndrome is a fairly distinct clinical entity and once recognised is not likely to be confused with illnesses other than sarcoidosis, being virtually pathognomonic for the disease. [patient.info]
Workup
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].
Other Pathologies
- Granulomatous Tissue
Microscopy with hematoxylin and eosin stain revealed tissue composed of salivary gland acini replaced by non-caseating granulomatous tissue. [journalgateway.com]
Treatment
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]
Treatment and prognosis The constellation of symptoms along with blood samples and biopsy usually allows the diagnosis. [radiopaedia.org]
Prognosis
Treatment and prognosis The constellation of symptoms along with blood samples and biopsy usually allows the diagnosis. [radiopaedia.org]
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]
The degree of involvement of sarcoidosis at other sites will influence the overall prognosis. [patient.info]
Prognosis is not so positive if granulomas affect the heart. Unfortunately, people with sarcoidosis are prone to certain cancers such as lung cancer or lymphomas. [ic.steadyhealth.com]
Etiology
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]
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]
Epidemiology
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. [content.nejm.org]
Journal Journal ID (nlm-ta): Pan Afr Med J Journal ID (iso-abbrev): Pan Afr Med J Journal ID (publisher-id): PAMJ Title: The Pan African Medical Journal Publisher: The African Field Epidemiology Network ISSN (Electronic): 1937-8688 Publication date (Electronic [scienceopen.com]
Epidemiology Heerfordt's syndrome is relatively rare. [patient.info]
Pathophysiology
Pathophysiology The exact etiology of sarcoidosis is unknown, making it largely a diagnosis of exclusion. [eyewiki.aao.org]
Prevention
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 Quiz questions References [radiopaedia.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]
Oral hygiene plays a pivotal role for these patients, as the dental surgeon recommends regular prevention treatment for restorative lesions and oral infections. [icliniq.com]
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]
References
- Fujiwara K, Furuta Y, Fukuda S. Two Cases of Heerfordt’s Syndrome: A Rare Manifestation of Sarcoidosis. Case Rep Otolaryngol. 2016;2016:3642735.
- Denny MC, Fotino AD. The Heerfordt-Waldenström syndrome as an initial presentation of sarcoidosis. Proc (Bayl Univ Med Cent). 2013;26(4):390-392.
- Koyama T, Ueda H, Togashi K, et al. Radiologic manifestations of sarcoidosis in various organs. Radiographics. 2004;24(1):87-104.
- Dua A, Manadan A. Images in clinical medicine. Heerfordt's syndrome, or uveoparotid fever. N Engl J Med. 2013;369(5):458.
- 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.
- 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.