Heerfordt syndrome

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Heerfordt syndrome
Other namesUveoparotid fever, [1] Heerfordt–Mylius syndrome,
Specialty Angiology   OOjs UI icon edit-ltr-progressive.svg

Heerfordt syndrome 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] [2]

Contents

Causes

The exact cause of Heerfordt syndrome has not yet been definitively determined. [3] Of those patients who have been diagnosed with Heerfordt syndrome, 15% have a close relative who also has the syndrome. [4] 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]

Diagnosis

In patients that have already been diagnosed with sarcoidosis, Heerfordt syndrome can be inferred from the major symptoms of the syndrome, which include parotitis, fever, facial nerve palsy and anterior uveitis. In cases of parotitis, ultrasound-guided biopsy is used to exclude the possibility of lymphoma. [5] There are many possible causes of facial nerve palsy, including Lyme disease, HIV, Melkersson–Rosenthal syndrome, schwannoma, and Bell's palsy. Heerfordt syndrome exhibits spontaneous remission. [6]

Treatment

Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs. [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. [7] [8] [9] Heerfordt syndrome is present in 4.1 to 5.6% of those with sarcoidosis. [10]

History

The condition was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named. [11] 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. [12] [13]

See also

Notes

References

  1. 1 2 3 4 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.
  2. ↑ Dua, Anisha; Manadan, Augustine (2013-08-01). "Heerfordt's Syndrome, or Uveoparotid Fever". New England Journal of Medicine. 369 (5): 458. doi:10.1056/NEJMicm1303454. ISSN   0028-4793. PMID   23902485.
  3. ↑ Fujiwara, Keishi; Furuta, Yasushi; Fukuda, Satoshi (2016). "Two Cases of Heerfordt's Syndrome: A Rare Manifestation of Sarcoidosis". Case Reports in Otolaryngology. 2016 3642735. doi: 10.1155/2016/3642735 . ISSN   2090-6765. PMC   4739221 . PMID   26885424.
  4. ↑ Makimoto, Go; Kawakado, Keita; Nakanishi, Masamoto; Tamura, Tomoki; Noda, Minori; Makimoto, Satoko; Sato, Yumiko; Kuyama, Shoichi (2021-06-01). "Heerfordt's Syndrome Associated with Trigeminal Nerve Palsy and Reversed Halo Sign". Internal Medicine (Tokyo, Japan). 60 (11): 1747–1752. doi:10.2169/internalmedicine.6176-20. ISSN   1349-7235. PMC   8222113 . PMID   33390492.
  5. ↑ 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. S2CID   1088521.
  6. ↑ Itoh, T.; Tanaka, R.; Matsushita, H. (1998-05-01). "[Heerfordt's syndrome remitting without corticosteroid therapy]". Nihon Kokyuki Gakkai Zasshi = the Journal of the Japanese Respiratory Society. 36 (5): 494–497. ISSN   1343-3490. PMID   9742871.
  7. ↑ 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.
  8. ↑ Hena, Kerry Maryse (2020). "Sarcoidosis Epidemiology: Race Matters". Frontiers in Immunology. 11 537382. doi: 10.3389/fimmu.2020.537382 . ISSN   1664-3224. PMC   7522309 . PMID   33042137.
  9. ↑ Newman, Lee S.; Rose, Cecile S.; Maier, Lisa A. (1997-04-24). "Sarcoidosis". New England Journal of Medicine. 336 (17): 1224–1234. doi:10.1056/NEJM199704243361706. ISSN   0028-4793. PMID   9110911.
  10. ↑ 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.
  11. ↑ 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 Graefes Archiv für Ophthalmologie. 70 (2): 254–273. doi:10.1007/bf02008817. S2CID   10880812.
  12. ↑ synd/3546 at Whonamedit?
  13. ↑ 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 (1–2): 53–68. doi:10.1111/j.0954-6820.1937.tb16029.x.
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