WordNet

a rise in the temperature of the body; frequently a symptom of infection (同)febrility, febricity, pyrexia, feverishness
intense nervous anticipation; "in a fever of resentment"
highly excited; "a fevered imagination"

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〈U〉《しばしばa fever》(身体の異常な)『熱』,発熱 / 〈U〉『熱病』 / 〈U〉〈C〉(…に対する)『興奮』,熱狂《+『for』+『名』》
(比較変化なし)《名詞の前にのみ用いて》熱のある,熱病にかかった / 熱にうかされたような,興奮した / (憎しみなどが)異常に強い

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/04/06 21:25:14」(JST)

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Heerfordt's syndrome, also referred to as uveoparotid fever,^[1] Heerfordt–Mylius syndrome, Heerfordt–Waldenström syndrome, and Waldenström’s uveoparotitis,^[2] is a rare manifestation of sarcoidosis. The symptoms include inflammation of the eye (uveitis), swelling of the parotid gland, chronic fever, and in some cases, palsy of the facial nerves.^[1]

History

The condition was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named.^[3] It was originally attributed to mumps, but after further studies by Swedish doctor Jan G. Waldenström in 1937, it was classified as a distinct manifestation of sarcoidosis.^[2]^[4]

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 and Propionibacteria species have both been suggested as the environmental agent, though the evidence for this is inconclusive.^[1]

Prevalence

In the United States, sarcoidosis has a prevalence of approximately 10 cases per 100,000 whites and 36 cases per 100,000 blacks.^[5] Heerfordt's syndrome is present in 4.1–5.6% of those with sarcoidosis.^[6]

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, fever, and facial nerve palsy. In cases of parotitis, ultrasound-guided biopsy is used to exclude the possibility of lymphoma.^[7] There are many possible causes of facial nerve palsy, including Lyme disease, HIV, Melkersson–Rosenthal syndrome, schwannoma, and Bell's palsy. Heerfordt's syndrome exhibits spontaneous remission. Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs.^[1]

Notes

Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.

References

^ ^a ^b ^c ^d Evanchan, Jason; Barreiro, Timothy J.; Gemmel, David (May 2010). "Uveitis, salivary gland swelling, and facial nerve palsy in a febrile woman". Journal of the American Academy of Physician Assistants 23 (5): 46–50. doi:10.1097/01720610-201005000-00012. PMID 20480871.
^ ^a ^b synd/3546 at Who Named It?
^ Heerfordt C.F. (1909). "Über eine "Febris uveo-parotidea subchronica" an der Glandula parotis und der Uvea des Auges lokalisiert und häufug mit Paresen cerebrospinaler Nerven kompliziert". Albrecht von Grafes Archiv für Ophthalmologie 70: 254–273. doi:10.1007/bf02008817.
^ Waldenström, J. G. (1937). "Some observations on uveoparotitis and allied conditions with special reference to the symptoms from the nervous system". Acta Medica Scandinavica 91: 53–68. doi:10.1111/j.0954-6820.1937.tb16029.x.
^ Iannuzzi, Michael C.; Rybicki, Benjamin A.; Teirstein, Alvin S. (22 November 2007). "Sarcoidosis". New England Journal of Medicine 357 (21): 2153–65. doi:10.1056/NEJMra071714. PMID 18032765.
^ Fukuhara K, Fukuhara A, et al. (August 2013). "Radiculopathy in patients with Heerfordt's syndrome: two case presentations and review of the literature". Brain and Nerve 65 (8): 989–92. PMID 23917502.
^ Fischer, T.; et al. (January 2002). "Diagnosis of Heerfordt's syndrome by state-of-the-art ultrasound in combination with parotid biopsy: a case report.". European Radiology 12 (1): 134–7. doi:10.1007/s003300100879. PMID 11868089.

UpToDate Contents

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1. 周期性発熱症候群およびその他の自己炎症性疾患：概要 periodic fever syndromes and other autoinflammatory diseases an overview
2. 免疫能の正常な患者における発熱および発疹 fever and rash in the immunocompetent patient
3. 成人における不明熱の病因 etiologies of fever of unknown origin in adults
4. 成人における不明熱に対するアプローチ approach to the adult with fever of unknown origin
5. 術後発熱 postoperative fever

English Journal

Do you know this syndrome? Heerfordt-Waldenström syndrome.

Fraga RC1, Kakizaki P2, Valente NYS2, Portocarrero LKL2, Teixeira MFS2, Senise PF3.
Anais brasileiros de dermatologia.An Bras Dermatol.2017 Jul-Aug;92(4):571-572. doi: 10.1590/abd1806-4841.20175211.
PMID 28954117

Neurosarcoidosis: a clinical approach to diagnosis and management.

Ibitoye RT1,2, Wilkins A3,4, Scolding NJ3,4.
Journal of neurology.J Neurol.2017 May;264(5):1023-1028. doi: 10.1007/s00415-016-8336-4. Epub 2016 Nov 22.
PMID 27878437

Heerfordt's Syndrome Associated with a High Fever and Elevation of TNF-α.

Makimoto G1, Miyahara N, Yoshikawa M, Taniguchi A, Kanehiro A, Tanimoto M, Kiura K.
Acta medica Okayama.Acta Med Okayama.2016 Aug;70(4):273-7.
PMID 27549672

Japanese Journal

難聴と前庭機能低下を伴つたHEERFORDT症候群の1症例

耳鼻と臨床 33(1), 1-5, 1987
NAID 130004403219

Ｕｖｅｏ‐ｐａｒｏｔｉｄ　ｆｅｖｅｒならびに胸水貯留を伴ったｓａｒｃｏｉｄｏｓｉｓの１症例

日本内科学会雑誌 72(4), 446-451, 1983
NAID 130000891815

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