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Heerfordt syndrome

AI overview

Heerfordt syndrome is a rare manifestation of sarcoidosis.

Key points
  • The exact cause has not been definitively determined.
    source quote
    The exact cause of Heerfordt syndrome has not yet been definitively determined.
  • 15% of diagnosed patients have a close relative with the syndrome.
    source quote
    Of those patients who have been diagnosed with Heerfordt syndrome, 15% have a close relative who also has the syndrome.
  • It may result from a combination of environmental agent and hereditary predisposition.
    source quote
    the syndrome results from a combination of an environmental agent and a hereditary predisposition
  • Mycobacterium and Propionibacteria have been suggested as possible environmental agents.
    source quote
    Mycobacterium and Propionibacteria species have both been suggested as the environmental agent, though the evidence for this is inconclusive.
  • It is present in 4.1 to 5.6% of those with sarcoidosis.
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    Heerfordt syndrome is present in 4.1 to 5.6% of those with sarcoidosis.
  • It was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt.
    source quote
    The condition was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named.
  • Originally attributed to mumps, it was classified as a distinct manifestation of sarcoidosis in 1937.
    source quote
    after further studies by Swedish doctor Jan G. Waldenström in 1937, it was classified as a distinct manifestation of sarcoidosis
Symptoms
  • Inflammation of the eye (uveitis)
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    inflammation of the eye ( uveitis )
  • Swelling of the parotid gland
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    swelling of the parotid gland
  • Chronic fever
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    chronic fever
  • Palsy of the facial nerves in some cases
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    in some cases, palsy of the facial nerves
Diagnosis
  • In patients already diagnosed with sarcoidosis, it can be inferred from major symptoms including parotitis, fever, facial nerve palsy and anterior uveitis.
    source quote
    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.
Prognosis

Heerfordt syndrome exhibits spontaneous remission.

AI-synthesized from the Wikipedia article “Heerfordt syndrome”. Not medical advice. Verify source →
Heerfordt syndrome
Other namesUveoparotid fever,[1] Heerfordt–Mylius syndrome,
SpecialtyAngiology 

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]

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]

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]

Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs.[1]

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]

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]

  • Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.

  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.