Heerfordt(-Waldenström) syndrome or uveoparotid fever is a variant of sarcoidosis, comprising of:
- parotid enlargement
- facial palsy
- ocular involvement (anterior uveitis)
The exact prevalence is unknown, as only isolated case reports exist. Neurologic involvement may occur in ~5% of patients with sarcoidosis, with facial nerve palsy dominating 2-4. Interestingly, rarity sections hold a case with the subsequent development of progressive multifocal leukoencephalopathy PML 3.
It may present as complete or incomplete (two out of the last three) 3. This holds also true for the single symptoms facial nerve palsy and anterior uveitis 2.
Although no specific imaging characteristics exist, imaging is nonetheless important to show the systemic nature of the disease and/or possible biopsy sites.
Enlargement of the parotid gland(s) and enlargement of cervical lymphadenopathy seem to be typical 2 and may be observed clinically and by various imaging modalities.
Treatment and prognosis
The constellation of symptoms along with blood samples and biopsy usually allows the diagnosis. Prognosis equals the underlying systemic disease, as such marked improvement of symptoms often occurs after a few days of immunomodulating therapy, e.g. steroids 2-4. Possible associated diseases need independent treatment 3.
History and etymology
The first to describe the constellation of symptoms was the Danish ophthalmologist Christian Frederik Heerfordt (1871-1953) in 1909 5-6. Association with sarcoidosis was observed by the Swedish internist Jan Gösta Waldenström (1906-1996) in 1937 7-8.
- misdiagnosing facial paralysis as simple Bell palsy prevents possible treatment of the underlying systemic disorder 2-4
- 1. Koyama T, Ueda H, Togashi K et-al. Radiologic manifestations of sarcoidosis in various organs. Radiographics. 24 (1): 87-104. doi:10.1148/rg.241035076 - Pubmed citation
- 2. Denny MC, Fotino AD. The Heerfordt-Waldenström syndrome as an initial presentation of sarcoidosis. Proc (Bayl Univ Med Cent). 2013;26 (4): 390-2. Free text at pubmed - Pubmed citation
- 3. Yagi T, Hattori H, Ohira M et-al. Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir. Clin Neurol Neurosurg. 2010;112 (2): 153-6. doi:10.1016/j.clineuro.2009.10.005 - Pubmed citation
- 4. Tamme T, Leibur E, Kulla A. Sarcoidosis (Heerfordt syndrome): a case report. Stomatologija. 2007;9 (2): 61-4. Pubmed citation
- 5. Heerfordt C. Über eine Febris uveo-parotidea subchronica. Von Graefe‘s Archiv fur Opthalmologie 1909; 70:254-73.
- 6. http://www.whonamedit.com/doctor.cfm/2946.html
- 7. Waldenström JG. Some observations of uveoparotitis and allied conditions with special reference to the symptoms from the nervous system. Acta Medica Scandinavica. 1937;91(1–2):53–68
- 8. http://www.whonamedit.com/doctor.cfm/2618.html
- systemic manifestations
- pulmonary and mediastinal manifestations (chest x-ray staging)
- cardiac manifestations
- musculoskeletal manifestations
- head and neck manifestations
- central nervous system manifestations
- abdominal manifestations
- cutaneous manifestations