Heerfordt syndrome is a bilateral mumps with uveitis and fever, presumably caused by sarcoidosis or a viral infection. It is possible to involve the respiratory tract, lymph nodes and facial nerve in the pathological process. The clinical picture is dominated by complaints of an increase in body temperature, visual impairment, enlargement of the parotid glands and lymph nodes. Diagnostics is based on biomicroscopy, visometry, ophthalmoscopy, radioisotope scanning and ultrasound of the affected glands. Treatment tactics are reduced to the use of glucocorticosteroids, NSAIDs and analgesics.
D86.8 Sarcoidosis of other specified and combined localizations
Heerfordt syndrome (uveoparotitis, uveoparotitis fever) was first described in 1909 by the Danish ophthalmologist K. F. Heerfordt. According to statistics, the prevalence of the disease in sarcoidosis reaches 21-56%. In 6% of patients, a lesion of the salivary glands is diagnosed, in 25-36% – the eyes. Anterior uveitis is detected 2 times more often than posterior. Pathology is mainly found in middle-aged people. Predisposition to the disease in women is higher than in men. Spontaneous healing is observed in 90% of cases. Only 10% of patients suffer from chronic uveoparotitis fever with a tendency to relapse.
The etiology of the disease is not fully understood. Scientists believe that uveoparotitis develops against the background of infectious or autoimmune pathologies. The main risk factors include immunodeficiency conditions, a recent mumps infection. Genetic predisposition to uveoparotite fever has not been proven. The most common causes of the syndrome are:
- Sarcoidosis. Benier-Beck-Schaumann disease is a multisystem granulomatous disease with unclear etiology and pathogenesis, accompanied by damage to the nervous system. It can manifest itself by the clinic of Heerfordt syndrome.
- Viral diseases. Uveoparotitis occurs against the background of a recently transferred epidemic mumps caused by an RNA-containing virus of the paramyxovirus family.
- Bacterial infections. A possible cause is infection with Mycobacteria or Propionobacteria. Cases of combination of tuberculous uveitis with fever and mumps are described.
The morphological substrate of sarcoidosis is considered to be an epithelioid cell granuloma, which is a cluster of mononuclear phagocytes. Patients have a nonspecific sarcoidosis reaction in the form of epithelioid cell granulomatosis. Similar foci located in lymph nodes, mumps glands, respiratory tract, other organs and tissues presumably provoke the development of Heerfordt syndrome. With the infectious nature of the disease, the pathogenesis is based on the replication of the virus in glandular tissue with damage to regional lymph nodes. Researchers believe that the bacterial flora acts as a trigger that stimulates the development of the disease, but the pathogenesis of this phenomenon has not been studied.
Patients often have a history of chronic calculous mumps. The syndrome develops acutely. The first manifestation of pathology is an increase in temperature to febrile values. The parotid glands are enlarged, painless when chewing and touching. Patients note redness of the conjunctiva, deterioration of vision, the appearance of “floating opacities” in front of the eyes. In some cases, the sublingual salivary glands increase. When the mucous membrane of the upper and lower respiratory tract is involved, a cough occurs, which is often mistaken for neurogenic. Shortness of breath is increasing. In severe cases, the nervous system is involved, there is a lesion not only of the visual, but also of the vestibular analyzer. Patients complain of double vision, loss of visual fields, dizziness, instability when walking, hearing impairment. Subsequently, the symptoms are reduced and disappear. A recurrent course of the disease is possible.
If the mucous membrane of the respiratory tract is affected, there is a high risk of developing bronchitis or pneumonia. Frequent complications of the syndrome are retinal detachment and optic neuritis. Floating opacities in front of the eyes may be the first sign of vitreous destruction. With the penetration of pathological agents through the blood-brain barrier, meningoencephalitis may occur. With paralysis of the vestibular-cochlear nerve, a clinic of temporary deafness is formed. Enlargement of lymph nodes is often replaced by lymphadenitis. The spread of the process to the salivary glands leads to sialoadenitis.
Diagnosis requires a comprehensive examination using physical techniques and specific diagnostic methods. On palpation, the parotid glands are hypertrophied, painless. The lymph nodes are enlarged, not soldered to the surrounding tissues, the skin above them is not changed. Specific diagnostic methods include:
- Biomicroscopy of the eye. The injection of conjunctival vessels is determined. The transparency of the cornea of the eyeball is preserved. Small precipitates on the endothelium are visualized. Pigment is found on the anterior capsule of the lens, an inflammatory suspension is found in the vitreous cavity.
- Ophthalmoscopy. Examination of the fundus reveals focal changes in the form of retinal edema and optic disc. The arteries of the inner shell of the eye are narrowed, the veins are full-blooded, convoluted. In patients with iridocyclitis, ophthalmoscopy is difficult due to damage to the anterior segment of the eyeball.
- Ultrasound of the eyes. The sonogram shows acoustically heterogeneous inclusions of the vitreous body, which look like flakes that are not attached to the inner shell of the eyeball. The lens is of normal shape. The longitudinal axis of the eye corresponds to the nature of ametropia, more often enlarged.
- Visometry. There is a decrease in visual acuity. With the auxiliary use of computer refractometry, it is possible to diagnose the myopic type of clinical refraction. Mild or moderate myopia.
- Ultrasound examination of the parotid glands. The glandular tissue is hypertrophied, the structure is heterogeneous, hypoechoic foci are visible. With calculous mumps, concretions are determined in the excretory duct. A puncture biopsy is performed under ultrasound control to confirm the diagnosis.
- Radioisotope scanning of salivary glands. Gallium-67 is used for the study. Patients with uveoparotitic fever are characterized by increased capture of the isotope by the parotid glands. If there are contraindications, sialography is performed.
Differential diagnosis is carried out with mumps and Mikulich syndrome. With epidemic mumps, there is a clear stage of the infectious process, the soreness of the parotid glands increases when chewing, talking, pain radiates into the ear area. With Mikulich syndrome, hypertrophy is noted not only of the salivary glands, but also of the lacrimal glands. In addition to an ophthalmologist, patients need to consult a maxillofacial surgeon.
In most cases, spontaneous recovery is observed within 2-3 months. In order to prevent the chronization of the process and the occurrence of relapses of the disease, conservative treatment is indicated. Instillations of antibacterial agents are necessary only with the development of complications. Therapeutic tactics are reduced to the appointment:
- Hormonal drugs. The use of glucocorticosteroids is justified in confirmed sarcoidosis. Treatment with prednisone is recommended to start with a shock dose (pulse therapy) with a further reduction in dosage. Hormone instillations promote partial resorption of precipitates.
- Nonsteroidal anti-inflammatory drugs (NSAIDs). Medications of this group can reduce the severity of the inflammatory process and local edema. If the body temperature does not normalize, analgesics-antipyretics are additionally used.
- Immunomodulators. Leukocyte interferon is prescribed in drops every 4 hours. The drug stimulates the phagocytic activity of macrophages, has cytotoxic and antiviral effects.
Prognosis and prevention
The prognosis for Heerfordt syndrome is favorable for life and work capacity. The probability of chronicling the process reaches 10%. After 2-3 months, in most cases, complete clinical remission occurs. Specific preventive measures have not been developed. Nonspecific prevention is based on the timely treatment of diseases of the parotid gland, including mumps. With the development of pathology against the background of sarcoidosis, patients should be registered with an ophthalmologist at the dispensary. An annual examination with mandatory fundus examination, visometry and biomicroscopy of the eye is shown.