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.
So far, reports show that uveoparotid fever affects only about 0.3% of all patients suffering from sarcoidosis . Furthermore, very few patients with uveoparotid fever have been described in the literature, but the clinical picture is well-established    . 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      . 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  . Some authors also describe various forms of uveitis, such as panuveitis, granulomatous uveitis, myodesopsia, all frequently accompanied by eyelid swelling . 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)    . 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     . 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  . Uveoparotid fever, generally diagnosed in patients between the second and fourth decade of life, is generally viewed as a self-limiting condition , but an early diagnosis undoubtedly facilitates better therapeutic response.
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]
Figure 2: Chest X-ray showing the presence of bilateral hilar and mediastinal lymphadenopathy. Figure 3: Axial image of a contrast CT scan showing the presence of bilateral paratracheal, subcarinal and hylar lymphadenopathy. [nzma.org.nz]
Entire Body System
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]
From Wikidata Jump to navigation Jump to search Human disease Heerfordt's syndrome (disorder) Uveoparotid fever Heerfordt's syndrome edit Language Label Description Also known as English uveoparotid fever Human disease Heerfordt's syndrome (disorder) [wikidata.org]
[…] 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]
Several physicians were involved in its discovery and characterisation.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]
As an explanation of the negative tuberculin tests thc American pathologist Branch has set forth the theory that cheesy degeneration and caseation are necessary for the production of tuberculous allergy, since the substances re- quired to produce tuberculin [docslide.com.br]
Jaw & Teeth
Parotid swelling May be unilateral or bilateral - bilateral in 73% of cases of parotid sarcoidosis. Diffuse, painless swelling of the parotid other salivary glands/lacrimal glands. May cause a dry mouth. [patient.info]
Heerfordt described three patients with uveitis, parotid swelling, cranial nerve palsy, and fever in 1909  and Waldenström classified it as a distinct manifestation of sarcoidosis in 1937 . [hindawi.com]
The facial paresis disappeared in the course of a few days, the parotid swelling within a fortnight, whereas the irido- cyclitis persisted, running a remittent course. [docslide.com.br]
Acute suppurative parotitis presents with painful parotid swelling, fever, redness and purulent discharge from Stensen’s duct. Parotitis is attributable to a retrograde infection of the gland, usually bacterial in nature. [clinicaladvisor.com]
Sialosis: 35 cases of persistent parotid swelling from two countries. [medicine.uiowa.edu]
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]
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]
Figure 1: Papule on the forehead, with a translucent appearance and red-brownish color Figure 2: Conjunctival hyperemia secondary to uveitis Figure 3: Sarcoidal granulomas on the dermis (left photo, hematoxylin and eosin, X100). [scielo.br]
A biopsy of the facial lesion was performed, and the histopa- Figure 2: Conjunctival hyperemia secondary to uveitis Figure 1: Papule on the forehead, with a translucent appearance and red-brownish color Figure 3: Sarcoidal granulomas on the dermis (left [docksci.com]
Lacrimal Gland Enlargement
Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs. The exact cause of Mikulicz syndrome is not known. Some scientists believe that Mikulicz syndrome should be considered a form of Sjögren syndrome. [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]
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 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    . 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  . 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   .
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]
As with other manifestations of sarcoidosis, corticosteroids are the mainstay of treatment. [ncbi.nlm.nih.gov]
The degree of involvement of sarcoidosis at other sites will influence the overall prognosis. [patient.info]
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]
The likelihood of permanent paralysis increases in patients who are not treated with corticosteroids.4 The prognosis of sarcoidosis varies according to disease duration, severity and the organs affected. [scielo.br]
Treatment and prognosis The constellation of symptoms along with blood samples and biopsy usually allows the diagnosis. [radiopaedia.org]
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  Pathology parotid gland biopsy (image) no necessary scattered granulomas with focal central necrosis stains for acid-fast bacilli & fungi negative histopathology image ( parotid )  compression [anvita.info]
and the Department of Biostatistics and Research Epidemiology, Henry Ford Health System, Detroit (B.A.R.). Address reprint requests to Dr. [doi.org]
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]
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. 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]
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]
- 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.