Heerfordt-Waldenström syndrome or uveoparotid fever (ICD10 D86.8)
Heerfordt-Waldenström syndrome (HWS) was firstly described in 1909 by Christian Frederick Heerfordt. It is characterized by the presence of anterior uveitis, parotid gland enlargement, facial palsy and fever. In 1937 Jan G.Waldenström, classified it as a distinct manifestation of sarcoidosis. (1) HWS is a rare subtype of sarcoidosis, accounting for 4.1–5.6% of patients with sarcoidosis. (2)
Etiology and Pathophysiology
While the exact aetiology and pathogenesis of HWS are yet to be fully elucidated, the basic pathology of HWS is the underlying granulomatous inflammatory reaction. (3) A study of 1,000 sarcoidosis patients showed correlation between the HLA-DRB1*04 allele and HWS. (4)
HWS tends to present ambiguously, and the classical diagnostic criteria may not occur concomitantly. The clinical presentation varies from asymptomatic disease to acute illness, to chronic insidious organ failure. Most patients have a low-grade fever. Eye involvement occur in about 11-83 % of patients, most commonly with anterior uveitis. Facial nerve palsy is an integral component with an incidence of 25-50% in this syndrome. Parotid gland involvement occurs in about 6% percent and is usually bilateral. (5,6,7) Literature review also revealed variable presentation of HWS that includes progressive multifocal leukoencephalopathy, trigeminal nerve palsy, pericardial effusion, bilateral eyelids swelling, xerostomia and keratoconjunctivitis sicca (2).
Parotid gland enlargement, peripheric facial palsy and anterior uveitis. Asymptomatic conjunctival nodules may be present. (4)
HWS is fundamentally a clinical diagnosis and there are two presenting variants. In complete type, all the four components (anterior uveitis, parotid enlargement and facial nerve palsy and fever) are present while the incomplete type requires the presence of at least two out of three findings (anterior uveitis, parotid enlargement and facial nerve palsy).
Clinical features along with serum angiotensin-converting enzyme, chest X-ray and tissue biopsy provides adequate basis for diagnosis of HWS. (4)
- Sjögren Syndrome
- Herpes simplex virus–associated Bell's palsy
- Varicella zoster virus–associated Ramsay-Hunt syndrome
Corticosteroid treatment is the mainstay of treatment and will usually lead to the resolution of facial nerve palsy, uveitis, and parotid gland swelling. Alternative agents such as methotrexate, azathioprine, infliximab, or mycophenolate mofetil may be given to patients who do not respond to corticosteroids or who are unable to tolerate steroids. Artificial tears may also be implemented when facial paralysis with lagophthalmos is present. (4,7,8)
It is normally self-limiting, with cure achieved between 12 and 36 months, but some prolonged cases have been reported. The likelihood of permanent paralysis increases in patients who are not treated with corticosteroids. (8) The mortality rate ranges between 1 and 5% of cases. (4)
(1)- Fujiwara K, Furuta Y, Fukuda S. Two cases of Heerfordt’s Syndrome: a rare manifestation of sarcoidosis. Case Rep Otolaryngol 2016;2016:3642735.
(2)- Fukuhara K, Fukuhara A, Tsugawa J, et al. Radiculopathy in patients with Heerfordt’s syndrome: two case presentations and review of the literature. Brain Nerve 2013;65:989–92.
(3) - Ramasamy, K. Heerfordt-Waldenström Syndrome: An under-recognised form of sarcoidosis. Postgrad. Med. J. 98, 72 (2022).
(4)- Da Cunha Filho, R. R., Gervini, R. L. & Cartell, A. Do you know this syndrome? An. Bras. Dermatol. 80, 639–642 (2005).
(5)- Joseph FG, Scolding NJ. Neurosarcoidosis: A study of 30 new cases. J Neurol Neurosurg Psychiatry 2009; 80:297–304. doi: 10.1136/jnnp.2008.151977.
(6) - Longo N, Ghaderi M. Primary parotid gland sarcoidosis: Case report and discussion of diagnosis and treatment. Ear Nose Throat J 2010; 89:E6–10.
(7)- Chappity P, Kumar R, Sahoo AK: Heerfordt’s syndrome presenting with recurrent facial nerve palsy: case report and 10-year literature review. Sultan Qaboos Univ Med J. 2015, 15:124-128
(8)- Denny, M. C. & Fotino, A. D. The Heerfordt-Waldenström Syndrome as an Initial Presentation of Sarcoidosis. Baylor Univ. Med. Cent. Proc. 26, 390–392 (2013).