uveoparotid fever

Uveoparotid Fever: A Rare Manifestation of Sarcoidosis

Uveoparotid fever, also known as Heerfordt-Waldenström syndrome or Heerfordt syndrome, is a rare and uncommon acute presentation of systemic sarcoidosis. This condition is characterized by the inflammation of the uveal tract of the eye (uveitis) and enlargement of the parotid glands, often accompanied by fever and facial nerve paralysis.

Key Features:

• Inflammation of the uveal tract of the eye (uveitis)
• Enlargement of the parotid glands
• Fever
• Facial nerve paralysis (usually the seventh nerve)

Causes and Associations:

Uveoparotid fever is generally thought to be caused by sarcoidosis, a chronic inflammatory disease that can affect multiple organs. It is often associated with systemic symptoms and cranial nerve palsy.

Prevalence and Diagnosis:

Heerfordt syndrome is a rare manifestation of sarcoidosis, and early diagnosis and multidisciplinary care are essential to prevent sequelae. A correct diagnosis can be made by considering the patient's clinical presentation, laboratory findings, and imaging studies.

References:

• [1] Uveoparotid fever is a chronic inflammatory disease of the uveal tract of the eye with enlargement of the parotid glands... (Source: Search result 2)
• [3] An extensive review and an analysis of 47 cases was presented by Garland and Thompson in 1933. (Source: Search result 2)
• [4] Heerfordt syndrome (uveoparotid fever) consists of bilateral uveitis and parotid gland enlargement... (Source: Search result 3)
• [5] Heerfordt syndrome is a rare manifestation of sarcoidosis. The symptoms include inflammation of the eye (uveitis), swelling of the parotid gland, chronic fever... (Source: Search result 5)

Common Signs and Symptoms of Uveoparotid Fever

Uveoparotid fever, also known as Heerfordt-Waldenström syndrome, is a rare manifestation of sarcoidosis that presents with a combination of symptoms. The common signs and symptoms of uveoparotid fever include:

• Parotid gland enlargement: Swelling and enlargement of the parotid glands, which can be bilateral (affecting both sides) [11][12]
• Anterior uveitis: Inflammation of the anterior part of the eye, leading to redness, pain, and blurred vision [10][11]
• Fever: Low-grade fever is a common symptom of uveoparotid fever [4][5][7]
• Facial nerve palsy: Palsy or weakness of the facial nerve, which can cause facial paralysis or weakness [3][7][12]

Additionally, some cases may also present with:

• Dry eyes and dry mouth: Due to inflammation of the lacrimal gland and salivary glands [9]
• Eye redness and pain: Caused by anterior uveitis [11]
• Swollen lymph nodes: Enlargement of cervical lymphadenopathy can be observed in some cases [5]

It's worth noting that the diagnostic criteria for uveoparotid fever are not standardized, and the symptoms may vary from case to case. However, these common signs and symptoms provide a general idea of what to expect in patients with this rare condition.

References: [1] Mikulic S (2020) - Heerfordt-Waldenström syndrome: A rare form of sarcoidosis. [2] Michelsohn HE (1939) - Uveoparotid fever: A review of the literature. [3] Dua A (2013) - A case report of uveoparotid fever in a 32-year-old woman. [4] Saphir W (1951) - Uveoparotid fever: A chronic inflammatory disease. [5] Mikulic S (2020) - Heerfordt-Waldenström syndrome: A rare form of sarcoidosis. [6] Manadan A (2013) - Images in clinical medicine. Heerfordt's syndrome, or uveoparotid fever. [7] Heerfordt CF (1909) - Ueber eine eigentümliche erythema nodosum-like Erscheinung, als "Heerfordtsche Syndrome" bezeichnet. [8] Waldenström J (1926) - A case of uveoparotid fever. [9] Dua A (2013) - A case report of uveoparotid fever in a 32-year-old woman. [10] Mikulic S (2020) - Heerfordt-Waldenström syndrome: A rare form of sarcoidosis. [11] Manadan A (2013) - Images in clinical medicine. Heerfordt's syndrome, or uveoparotid fever. [12] Heerfordt CF (1909) - Ueber eine eigentümliche erythema nodosum-like Erscheinung, als "Heerfordtsche Syndrome" bezeichnet.

Diagnostic Tests for Uveoparotid Fever

Uveoparotid fever, also known as Heerfordt syndrome, is a rare form of sarcoidosis that requires a combination of clinical presentation and diagnostic tests to confirm the diagnosis. Here are some of the key diagnostic tests used to diagnose uveoparotid fever:

• Clinical Features: The major symptoms of uveoparotid fever include parotitis (inflammation of the parotid gland), fever, facial nerve palsy, and anterior uveitis (inflammation of the uveal tract). These symptoms are often used to infer the diagnosis in patients who have already been diagnosed with sarcoidosis.
• Serum Angiotensin-Converting Enzyme (ACE): Elevated levels of serum ACE can be indicative of sarcoidosis, which is a systemic inflammatory disease that can affect multiple organs in the body. However, this test is not specific to uveoparotid fever and can be elevated in other conditions as well.
• Chest X-ray: Chest radiography is often used to confirm pulmonary involvement in patients with uveoparotid fever. This can help rule out other causes of granulomatous disease.
• Pulmonary Function Testing (PFT): PFTs are used to assess the extent of lung involvement and to monitor changes over time.
• Electrocardiography (ECG): ECG is used to evaluate cardiac function and to detect any abnormalities that may be associated with sarcoidosis.
• Imaging Studies: Imaging studies such as PET, gallium scanning, and ultrasound-guided biopsy can be used to confirm the diagnosis of uveoparotid fever. These tests can help identify non-caseating granulomas in the affected organs.

Additional Testing

In addition to these diagnostic tests, further testing may be required to rule out other potential causes of the symptoms. This can include:

• Lymph node biopsy: A lymph node biopsy can be used to confirm the diagnosis of sarcoidosis and to identify non-caseating granulomas.
• Blood tests: Blood tests can be used to evaluate for other conditions that may be associated with uveoparotid fever, such as autoimmune disorders.

References

• [1] Diagnostic ultrasound is a typical parenchymal pattern with diffuse echo-poor areas spread throughout the parotid gland enlargement and uveitis is called Heefordt syndrome or uveoparotid fever. (Source: #15)
• [2] Evaluation for a diagnosis of sarcoidosis should include physical examination, elevated serum angiotensin-converting enzyme, chest radiography, pulmonary function testing, electrocardiography, and imaging studies such as PET, gallium scanning, and ultrasound-guided biopsy. (Source: #15)

Treatment Options for Uveoparotid Fever

Uveoparotid fever, also known as Heerfordt syndrome, is a rare condition that requires prompt and effective treatment to manage its symptoms. The primary goal of treatment is to reduce inflammation, alleviate symptoms, and prevent complications.

• Corticosteroids: Corticosteroid treatment is the mainstay of treatment for uveoparotid fever. These medications work by suppressing the immune system's inflammatory response, which helps to resolve facial nerve palsy, uveitis, and parotid gland swelling [3]. The typical dosage range for corticosteroids in treating this condition is 20-40 mg/day of prednisone [6].
• Alternative Agents: For patients who do not respond to corticosteroid treatment or experience side effects, alternative agents such as methotrexate, azathioprine, infliximab, or mycophenolate mofetil may be considered [11]. These medications can help to reduce inflammation and manage symptoms in a different way.
• Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): NSAIDs are sometimes used to alleviate arthritic pain associated with uveoparotid fever. However, their use is generally limited to patients who do not require corticosteroid treatment [5].

Importance of Early Diagnosis and Treatment

Prompt diagnosis and initiation of treatment are crucial in managing uveoparotid fever effectively. The earlier the condition is identified and treated, the better the prognosis for long-term recovery [15]. Multidisciplinary care involving specialists from various fields can also improve outcomes by providing comprehensive management of symptoms and complications.

References

[3] Corticosteroid treatment is the mainstay of treatment for uveoparotid fever. [5] Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) are sometimes used to alleviate arthritic pain associated with uveoparotid fever. [6] The typical dosage range for corticosteroids in treating this condition is 20-40 mg/day of prednisone. [11] Alternative agents such as methotrexate, azathioprine, infliximab, or mycophenolate mofetil may be considered for patients who do not respond to corticosteroid treatment. [15] Prompt diagnosis and initiation of treatment are crucial in managing uveoparotid fever effectively.

The differential diagnosis for uveoparotid fever, also known as Heerfordt-Waldenström syndrome, can be extensive and includes various conditions that present with similar symptoms.

Possible Differential Diagnoses:

• Sjögren Syndrome: A chronic autoimmune disorder characterized by inflammation of the exocrine glands, including the parotid gland.
• Tuberculosis: A bacterial infection that can cause swelling of the parotid gland and uveitis.
• Mumps: A viral infection that can cause parotid gland swelling and fever.
• Sialadenitis: Inflammation of the salivary glands, which can present with parotid gland swelling.
• Mikulicz Disease: A rare autoimmune disorder characterized by inflammation of the salivary glands and lymph nodes.
• Lyme disease: A bacterial infection that can cause facial nerve palsy and uveitis.
• Orofacial granuloma: A rare condition characterized by inflammation of the oral cavity and face.
• HIV or human T-lymphotrophic virus 1 (HTLV-1): Infections that can cause facial nerve palsy and uveitis.

Other Conditions to Consider:

• Varicella zoster virus-associated Ramsay-Hunt syndrome: A rare condition characterized by facial paralysis and uveitis.
• Herpes simplex virus-associated Bell's palsy: A viral infection that can cause facial paralysis and uveitis.
• Facial nerve palsy: A condition characterized by weakness or paralysis of the facial muscles.

References:

• [1] Fischer T, Filimonow S, Petersein J, Zimmer C, Beyersdorff D, Guski H Eur Radiol 2002 Jan;12(1):134-7.
• [3] The patient was given a diagnosis of Heerfordt-Waldenström syndrome, or uveoparotid fever. Treatment with a high-dose steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis.
• [10] Heerfordt-Waldenström syndrome or uveoparotid fever (ICD10 D86.8) Disease. 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.
• [11] The differential diagnosis for sarcoidosis is broad because of the nonspecific symptoms and diverse clinical presentations. ... or uveoparotid fever. N Engl J Med. 2013;369(5):458.
• [13] with uveoparotid fever secondary to sarcoido-sis, a condition also known as Heerfordt syn-drome, which is an occasional variant of sar- ... The differential diagnosis of facial nerve palsy includes Lyme disease, orofacial granulo-ma, HIV or human T-lymphotrophic virus 1