Heerfordt syndrome (also perceived as uveoparotid fever and Heerfordt-Waldenström syndrome) is a critical syndromal presentation of sarcoidosis. Multiple nodular sores identify sarcoidosis in the skin, eyes, internal organs, salivary glands, and lymph node enlargement. In 1909, an ophthalmologist from Denmark, C. F. Heerfordt, first described the indications – uveitis, parotid gland enlargement, and cranial nerve paresis.
Sarcoidosis describes a granulomatous disease of in-explicit etiology. This condition occurs throughout the world, affecting people of all genders, cultures, and ages. It consistently shows a preference for people under age 40, with a peak rate of 20 to 29. There is a second peak occurrence rate in Japan and Scandinavian countries in women aged 50. Most researches suggest a slightly greater disease frequency for women. Sweden, Denmark, and the USA seem to have the greatest prevalence rates globally.
What is Heerfordt Syndrome?
As previously described, Heerfordt’s syndrome is an exceptional subacute irregularity of sarcoidosis. It is defined as an aggregate of low-grade fever, facial palsy, uveitis, and parotid swelling in sarcoidosis cases. Facial palsy may be a factor of this disease, but all practitioners do not confirm it. The state of single facial palsy with no apparent cause is usually diagnosed as Bell’s palsy and attended empirically.
In approximately 6% of sarcoidosis cases, the granulomatous inflammatory reaction causes parotid gland enlargement. Granulomas, along with a peripheral lymphocyte deficit, are found in the anatomic diagnostics of impaired organs. It is usually observed self-controlling, with cure achieved between 1 to 3 years, but some prolonged incidents have also been reported.
Heerfordt Syndrome – Symptoms
Sarcoidosis disease may exhibit in different ways. After several reported cases, it has been analyzed that hilar lymphadenopathy is found to be in 90% of the cases, head and neck exhibitions in 15%, and enlargement of parotid glands in 6% of the related cases. Also, the salivary glands are seen to be less generally involved, with unilateral or bilateral swelling of the parotid gland and reported in only 6% to 8% of sarcoidosis patients.
The reported cases to date revealed the initial symptoms to be; involvement of the eyes, nervous system, and salivary glands frequently occurs in sarcoidosis condition. These symptoms must all present in the progression to fit the criteria of this syndrome and fever events.
Skin lesions and evidence of thoracic complications are some other symptoms of sarcoidosis that coexist with the syndrome. It represents a form of neurosarcoidosis, which is an inflammatory disease and vigorously affects our nervous system. There may be different neurological studies such as meningism or other cranial tissue lesions affecting ophthalmoplegia, pupillary reflex dysfunction, or other damages. In sarcoidosis, facial palsy seems to be the most frequent peripheral nervous system indication. Some other manifestations like vertigo, hearing impairment, and tinnitus may also be observed in the absence of other neurologic displays.
Heerfordt Syndrome – Causes
The precise cause of this catastrophic disease has not been identified. However, it has been reported that a minority of patients have a close family member with the syndrome. Most patients present as young or middle-aged adults between 20-40 years, although the disease may present at any age. There is some evidence to show Heerfordt syndrome may result from a combination of environmental factors and possibly a hereditary component. However, the evidence is still insufficient to support the hypothesis. Also, the most common presentation of the syndrome has been seen in women than in men. The disease is more prevalent in black than white patients and occurs in the third and fourth decades of life.
Heerfordt Syndrome – Treatment
The diagnostics of this syndrome are clinical, and medication depends on the development of systemic impairment. Oral corticosteroids are designated the first-line treatment selection. At the same time, the mortality rate varies between 1 and 5% of the patients.
The cause of sarcoidosis is not accurately understood, so the fundamental defending approaches do not exist. Sarcoidosis patients are likely to relapse and may benefit from long-term medical help. They are advised to seek immediate help if they experience indications possibly attributable to sarcoidosis.
Heerfordt Syndrome – Prognosis
The prognosis of sarcoidosis alters according to the disease term. The disease’s development is mutable as irresistible remission may occur in over half of the cases, while others may require more continuous management with corticosteroids. Facial paralysis tends to improve or react to steroids. One American research stated that out of all deaths reported in 1988-2007, 0.05% listed sarcoidosis as the main cause.