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The Mikulicz syndrome is the symptom of a general or systemic disease and occurs particularly frequently in the context of diseases such as tuberculosis, syphilis, Hodgen’s lymphoma and sarcoid. Patients’ parotid and lacrimal glands swell in what is believed to be an autoimmune process. The treatment of the syndrome usually corresponds to a causal therapy of the underlying disease.

What is Mikulicz Syndrome?

The parotid glands are paired and form the largest salivary glands in the human body. The serous glands can be affected by various diseases, such as viral diseases and, in older people, tumor diseases. See AbbreviationFinder for abbreviations related to Mikulicz Syndrome.

Reaction – like swelling of the parotid glands is called Mikulicz syndrome . In most cases, the swelling is associated with lacrimal gland swelling. Mikulicz syndrome is usually the symptom of a primary disease and can hardly be considered a primary disease itself.

Various diseases can be responsible for the swelling of the glands. The term Mikulicz syndrome goes back to Johann Freiherr von Mikulicz-Radecki, who first described the pathological condition in 1892. The terms Dacryo-sialo-adenopathia atrophicans, Mikulicz-Sjögren syndrome and Mikulicz-Radecki syndrome are used as synonyms.

Causes

The cause of the Mikulicz syndrome lies in an overriding general or systemic disease. Systemic diseases affect entire organ systems and are not limited to a single body region. Systemic diseases with symptomatic Mikulicz syndrome are primarily chronic lymphocytic leukemia, Hodgkin’s lymphoma and non-Hodgkin’s lymphoma.

Sometimes there is also a reactive swelling of the parotid and lacrimal glands in the context of sarcoidosis. Less commonly, the swelling is caused by tuberculosis, hyperthyroidism, or syphilis. It is not yet known which processes cause the swelling in detail.

According to speculation, the glands swell in the context of the diseases mentioned as a result of an autoimmune process. Since Mikulicz syndrome is similar to Sjogren’s syndrome, some researchers suspect that the two diseases have an identical basis.

Symptoms, Ailments & Signs

Mikulicz syndrome is characterized by a more or less severe swelling of the parotid glands, which in some cases leads to a general enlargement of the glands. Since the parotid glands are the largest glands in the body, the swelling usually causes various accompanying symptoms.

Accompanying symptoms, for example, can suddenly be extreme dry mouth with swallowing difficulties and tooth decay. In addition, the lacrimal glands often swell at the same time. Decreased tear production may occur. Swellings in other glands of the body are also conceivable. pain does not exist.

As a rule, the swellings are not sensitive to pressure, but merely hardened. The individual symptoms do not necessarily have to exist for a long time, but can also disappear and reappear periodically. In individual cases, depending on the underlying disease, there may be many other accompanying symptoms.

Diagnosis & disease progression

Mikulicz syndrome is usually diagnosed using a tissue sample from the affected glands. In most cases, the doctor first performs a palpation and ultrasound examination to determine other possible causes of the swelling. In the majority of all cases, the patients were diagnosed with a systemic disease long before the glands became swollen.

In such a case, the doctor already has a first suspicion of the syndrome in the anamnesis. However, differential diagnostics must still exclude diseases such as mumps or Heerfordt syndrome. Tumors must also be ruled out, usually histologically.

The prognosis for patients with Mikulicz syndrome depends on the primary cause of the glandular swelling. Since the syndrome is often triggered by malignant cancer, the time of diagnosis and the stage of the disease play a central role in the chances of recovery.

Complications

Those affected by Mikulicz syndrome suffer from a very dry mouth. Above all, this complaint occurs very suddenly and unexpectedly. The syndrome also causes significant swallowing difficulties, so that the patient is usually no longer able to eat and drink normally.

This can lead to dehydration or other deficiency symptoms that have a very negative effect on the patient’s health. Furthermore, patients often suffer from tooth decay, which can lead to pain in the teeth and other problems in the oral cavity. In general, Mikulicz syndrome significantly reduces the patient’s quality of life. However, the further course of the Mikulicz syndrome depends heavily on the underlying disease and its severity.

If it is cancer, it can spread to other parts of the body. Chemotherapy is then used in the treatment, which can lead to various side effects. Likewise, various transplants are necessary to save the life of the affected person. In many cases, the patient’s life expectancy is reduced by the underlying disease that triggers Mikulicz syndrome.

When should you go to the doctor?

Since Mikulicz syndrome is a congenital disease, a doctor should always be consulted with this disease if the respective symptoms and signs appear. There is no self-healing and in some cases the symptoms worsen. In general, Mikulicz syndrome is characterized by discomfort in the mouth, making it appear dry. Many patients also suffer from swallowing difficulties and thus problems in taking food and liquids. In most cases, these complaints also lead to being underweight or to various deficiency symptoms.

If these symptoms occur, a visit to a doctor is definitely necessary. Furthermore, glands on the body can be swollen and thus indicate Mikulicz syndrome. The severity of the symptoms can change over time. First and foremost, Mikulicz syndrome can be identified and diagnosed by a general practitioner. In the further treatment, however, various specialists are necessary to alleviate the symptoms completely and permanently.

Treatment & Therapy

When treating patients with Mikulicz syndrome, the focus is usually not on the symptom but on the actual cause of the swelling. In CLL, both conventional chemotherapy and antibody therapy usually fail. For this reason, a bone marrow transplant or stem cell transplant is usually carried out, which in principle can enable healing.

In the case of causative tuberculosis, therapy consists of a four-fold combination of rifampicin, isoniazid, pyrazinamide and ethambutol. In this case, the treatment lasts at least half a year. In children and in milder cases of tuberculosis in adults, a double combination is used instead of a quadruple combination.

If Mikulicz syndrome is due to sarcoidosis, there are no causal therapies available to cure the underlying disease. In such a case, the patient’s complaints are only treated symptomatically. If, for example, too little tear fluid and saliva is produced, replacement fluids are used in therapy.

Saliva and tear substitutes can usually prevent the glands from drying out and the associated symptoms. Although in the case of causes such as leukemia or tuberculosis, the focus of treatment is on the causal therapy of the systemic disease, a combined symptomatic treatment of the Mikulicz syndrome is usually carried out in addition to the causal therapy steps, even for these causes. Basically, the swelling recedes as soon as the causative systemic disease has healed. ´

Outlook & Forecast

The prognosis of Mikulicz syndrome is linked to the underlying cause of the health problems. Spontaneous healing is not to be expected with this disease. Rather, serious illnesses can develop if no medical help is sought. If the swelling of the glands can be reduced by the administration of medication, freedom from symptoms is possible within a short time.

In the case of an existing cancer, the further development is strongly dependent on the stage of the underlying disease. In most cases, cancer therapy must be initiated. Chemotherapy is often used to achieve long-term recovery. The treatment method is associated with numerous complications and side effects. This circumstance must be taken into account when making the forecast.

If a bone marrow transplant is necessary, various disorders or secondary diseases can also occur. If the procedure is carried out under the best possible conditions, the patient has a good chance of permanent relief from the symptoms. In the case of a chronic underlying disease, the chances of recovery are extremely slim. Physicians and medical professionals focus on minimizing symptoms and reducing the intensity of existing health problems. Long-term therapy is necessary so that any irregularities can be reacted to as quickly as possible. In addition, an increase in symptoms can be expected in the case of chronic underlying diseases without medical care.

Prevention

So far, Mikulicz syndrome can only be prevented to a limited extent. When it comes to preventing systematic swelling, the same preventive measures apply as for the causative systemic diseases chronic lymphocytic leukemia, Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, sarcoid, syphilis and tuberculosis.

The processes that cause the swelling of the glands in the context of the systemic diseases mentioned have so far been controversial. Since the connections are still largely in the dark, prevention of the primary diseases has so far been the only preventive measure for Mikulicz syndrome.

Aftercare

Mikulicz syndrome can lead to a number of different complications if left untreated or if treatment is started very late. As a rule, early detection and subsequent treatment has a very positive effect on the further course of the disease and can also prevent further deterioration of the symptoms.

In most cases, the syndrome leads to a significant enlargement of the glands in the body of the person affected. This leads to severe swallowing difficulties and also to a very dry mouth. The symptoms do not go away on their own and have a very negative effect on the quality of life of those affected. In some cases, the glands may swell.

It is not uncommon for Mikulicz syndrome to lead to caries, which can lead to problems when eating or drinking liquids. If the symptoms occur over a longer period of time, depression or other psychological upsets can also occur. As a rule, however, the disease can be treated well, so that the life expectancy of the affected person is usually not reduced.

You can do that yourself

In many cases, self-help is not possible with Mikulicz syndrome. Those affected are dependent on medical treatment in any case, which diagnoses and combats the underlying disease.

Since many of those affected are dependent on chemotherapy and suffer from the side effects of this therapy, the help and support of friends and acquaintances can help and lead to a positive course of the disease. Likewise, the person concerned should not exert himself excessively during the therapy and should offer the body rest and relaxation. Since the Mikulicz syndrome also often leads to caries, the teeth must be specially protected. The person concerned should have these checked regularly by a dentist in order to avoid complications.

Despite the severe difficulty in swallowing, the patient must take care to consume enough food and fluids to avoid dehydration or underweight and deficiency symptoms. Usually, low salivation can be treated with medication. However, the further course of the disease depends very much on the underlying disease, so that it is not generally possible to predict the course and the options for treatment.