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]
- Swelling
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]
There was moderate swelling of the preauricular glands hut. no swelling of the lacrimal glands. [docslide.com.br]
She was started on 60 mg of prednisone daily, and at follow-up 19 days later, the swelling and facial nerve palsy had resolved (Panel D). [nejm.org]
The case presented here describes the diagnostic work-up and differentiation of swelling of the parotid gland by employing state-of-the-art ultrasound techniques in a 33-year-old patient with Heerfordt's syndrome. [ncbi.nlm.nih.gov]
A 35-year-old man, an ex-smoker and a varnisher, presented a few days history of left thoracic pain, low-grade fever, asthenia, swelling of both parotid glands and dry eyes. [nzma.org.nz]
- Pain
Some of the released mediators such as increase the sensitivity to pain [wikivisually.com]
We present a 38-year-old female patient who had a solid and painful swelling behind each ear 20 days after the complaints of redness of both eyes, fatigue, night sweat, and weight loss. [tjn.org.tr]
Anterior uveitis classically presents with infected conjunctiva, blurred vision, or eye pain ( 13 ). This patient's blurred vision and eye pain were thought to be related to uveitis. [ncbi.nlm.nih.gov]
- Malaise
He had a 3-month history of fever, malaise, night sweats, and a 7-kg weight loss. Visual acuity was 20/25 OU at distance. [jamanetwork.com]
Prodromal symptoms, lasting for several weeks or months, include general malaise, drowsiness, FULL TEXT [annals.org]
Mild malaise and cough are chief features. Involvement of a specific organ is evidenced by dysfunction of that organ. Cutaneous lesion may be the only distinct manifestation in 25-35% cases. 5. Eye lesions in sarcoidosis 6. [de.slideshare.net]
[…] sarcoid Elevated conversion of vitD to its active form in epithelioid macrophages Restrictive lung disease Erythema nodosum Bell's palsy Hypercalcemia Uveoparotitis Polymyalgia rheumatica symptoms Pain and stiffness in shoulder and hips, often with fever, malaise [cram.com]
The patient will complain of pain and such symptoms as fever and malaise. Fortunately, the swelling is self-limiting and typically resolves with anti-staphylococcal antibiotic therapy and increased fluid intake. [clinicaladvisor.com]
- Chills
toxoplasmosis (acquired) hepatitis ( B58.1 ) tuberculosis with liver disease ( A18.83 ) Diagnosis Index entries containing back-references to D86.89 : Deposit bone in Boeck's sarcoid D86.89 Fever (inanition) (of unknown origin) (persistent) (with chills [icd10data.com]
Keyword Volume CPC($) Competition Congo Fever 1,000+ 0.39 0.01 Chills No Fever 1,000+ 0.12 0.07 Latin Fever 1,000+ 0.75 0.0 Fevered Letter 1,000+ 0 0.0 Hectic Fever 1,000+ 0.11 0.01 Fever Sweating 1,000+ 20.84 0.05 Continuous Fever 1,000+ 0.04 0.02 Chemo [minikeyword.com]
It usually occurs in children, and there is preauricular pain (pain felt in front of the ear), swelling of the parotid, fever, chills, and headaches. Bacterial sialadentitis is usually caused by ascending organisms from the oral cavity. [monsterologist.blogspot.com]
Gastrointestinal
- Vomiting
Clinical features A chronic, low grade fever is often present Complains of lassitude, malaise and vague gastrointestinal disturbances or even nausea, and vomiting. Xerostomia. A patchy erythema of skin in early stages of the disease. Enlargement [de.slideshare.net]
[…] knuckle calluses, dental enamel erosion, salivary gland enlargement -metabolic alkalosis (from vomiting), hypokalemia (from laxative/diuretic use), & elevated salivary amylase [studyblue.com]
- Nausea
Clinical features A chronic, low grade fever is often present Complains of lassitude, malaise and vague gastrointestinal disturbances or even nausea, and vomiting. Xerostomia. A patchy erythema of skin in early stages of the disease. Enlargement [de.slideshare.net]
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
- Sweating
He had a 3-month history of fever, malaise, night sweats, and a 7-kg weight loss. Visual acuity was 20/25 OU at distance. [jamanetwork.com]
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. [symptoma.com]
We present a 38-year-old female patient who had a solid and painful swelling behind each ear 20 days after the complaints of redness of both eyes, fatigue, night sweat, and weight loss. [tjn.org.tr]
If you have symptoms, they may include Cough Shortness of breath Weight loss Night sweats Fatigue Tests to diagnose sarcoidosis include chest x-rays, lung function tests, and a biopsy. Not everyone who has the disease needs treatment. [icdlist.com]
Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional 30-pound weight loss. [ncbi.nlm.nih.gov]
- Macula
Maculopathy Maculopathy is a word used to refer to any abnormality of the macula of the eye, an example is bull's eye maculopathy. [m.ivyroses.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
- Headache
It usually occurs in children, and there is preauricular pain (pain felt in front of the ear), swelling of the parotid, fever, chills, and headaches. Bacterial sialadentitis is usually caused by ascending organisms from the oral cavity. [monsterologist.blogspot.com]
Fever, headache, painful testicular swelling (orchitis) and a rash may occur as well. The symptoms are generally not severe in children. [clinicaladvisor.com]
Other symptoms such as headache and weight loss may occur. Eye symptoms Acute uveitis presents with: Eye discomfort Photophobia Blurred vision Seeing 'floaters' Red eye The lacrimal glands may be involved, causing puffy eyelids. [patient.info]
*4 month later she was again taken ill with tiredness, headache, rise of temperature to 39â, and pains in the knee-joints. [docslide.com.br]
– Recurrent apthous major – Recurrent herpetiform ulceration – Behcet’s syndrome 533. apthous ulcers begins their clinical course usually by a. a prodromal phase causing fever, headache and lymphadenopathy b. single or multiple superficial erosions [mdsclasses.com]
- Neuralgia
She had a history of postherpetic neuralgia and purpura pigmentosa chronica. As uveitis and BHL had been previously diagnosed, she underwent a detailed examination for sarcoidosis in the Department of Respiratory Medicine in our hospital. [hindawi.com]
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.