Heerfordt's Syndrome, or Uveoparotid Fever

Dua, Anisha B.; Manadan, Augustine

doi:10.1056/nejmicm1303454

Cited by 27 publications

(15 citation statements)

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“…The VII nerve paresis is usually evident unilaterally and less frequently affects both sides of the face (26,35,39). Rarely, the involvement of the VII nerve is associated with other specific symptoms such as Heerfordt's syndrome, a clinical variety characterized by facial nerve palsy, fever, swelling of the parotid glands, and uveitis (40). Hearing loss and vestibular dysfunction manifesting with dizziness are usually due to VIII nerve impairment (6).…”

Section: Central Nervous System (Cns) Sarcoidosismentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. Clinical and imaging findings are often non-specific since they can be found in a large number of neurological disorders. Histopathology can also be confounding if not performed by an expert pathologist and not placed in an appropriate clinical context. In this review, we discuss clinical features, laboratory findings, imaging, and histology of neurosarcoidosis, and we report current evidence regarding drug therapy. We conclude that a correct diagnostic approach should include a multidisciplinary evaluation involving clinicians, radiologists, and pathologists and that future studies should evaluate the genetic signature of neurosarcoidosis as they could be helpful in the assessment of this uncommon disease. With head-to-head comparisons of medical treatment for neurosarcoidosis still lacking due to the rarity of the disease and an increasing number of immunomodulating therapies at hand, novel therapeutic approaches are to be expected within the next few years. Key messages Neurosarcoidosis is a rare disorder that affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies, revealing that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. A multidisciplinary evaluation is useful to achieve a correct diagnosis because clinical and imaging findings are often non-specific. Corticosteroids are the first-line treatment for neurosarcoidosis, followed by steroid-sparing immune-modulating agents if prednisone therapy is insufficient. Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded.The following queries have arisen during the editing of your manuscript and are marked in the margins of the proofs. Unless advised otherwise, submit all corrections using the CATS online correction form. Once you have added all your corrections, please ensure you press the ''Submit All Corrections'' button.Please review the table of contributors below and confirm that the first and last names are structured correctly and that the authors are listed in the correct order of contribution. The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percenta...

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Section: Central Nervous System (Cns) Sarcoidosismentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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“…4 Additionally, incomplete presentations (two out of three symptoms) also occur. 6 Other reports mention parotitis (in lieu of parotid gland enlargement) as a diagnostic criterion. 7 We clinically diagnosed our patient with incomplete uveoparotid fever.…”

Section: Discussionmentioning

confidence: 99%

Uveoparotid fever as a presentation of sarcoidosis

Ramachandran

Haidari

Ahn

et al. 2019

Baylor University Medical Center Proceedings

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Uveoparotid fever, also known as Heerfordt-Waldenstr € om syndrome, is an uncommon acute presentation of systemic sarcoidosis. Patients may have features of complete/classic or incomplete disease. Early diagnosis and multidisciplinary care should be initiated to prevent sequelae. Herein, the authors report a rare case of retrospectively diagnosed incomplete uveoparotid fever in a patient with anterior uveitis, parotid gland enlargement, and fever who presented to our dermatology clinic with cutaneous sarcoidosis.

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“…Pathological tissue demostrated the presence of non-caseating granulomas without necrosis (Figure 4). He was given a diagnosis of Heerfordt's syndrome, a rare form of sarcoidosis (present only in 6% of the cases of sarcoidosis) charactherized by the presence of enlargement of the parotid gland associated with major symptoms as uveitis, facial paralysis (absent in our case) or fever [1,2]. He was started on 50 mg of prednisone daily, and at follow-up 15 days later, he was completely asymphtomatic (and the swelling and bloodshot eyes resolved).…”

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confidence: 88%