Mikulicz syndrome is a symptom of a general or systemic disease and is particularly common in the setting of diseases such as tuberculosis, syphilis, Hodgen’s lymphoma, and sarcoidosis. Patients’ parotid and lacrimal glands swell in what is thought to be an autoimmunologic process. Treatment of the syndrome usually corresponds to causal therapy of the causative 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 age, tumor diseases. Mikulicz syndrome is a reaction-like swelling of the parotid glands. In most cases, the swelling is associated with lacrimal gland swelling. Mikulicz syndrome is usually a symptom of a primary disease and can hardly be considered a primary disease itself. Different diseases may be responsible for the swelling of the glands. The name Mikulicz syndrome is derived from 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 Mikulicz syndrome is an overarching general or systemic disease. Systemic diseases affect entire organ systems and thus are not confined to a single region of the body. Systemic diseases with symptomatic Mikulicz syndrome are primarily chronic lymphocytic leukemia, Hodgkin’s lymphoma, and non-Hodgkin’s lymphoma. At times, reactive swelling of the parotid and lacrimal glands also occurs in the setting of sarcoidosis. Somewhat less commonly, the swelling is caused by tuberculosis, hyperthyroidism, or lues. The specific processes that cause the swelling are as yet unknown. According to speculations, the glands swell in the context of the mentioned diseases as a result of an autoimmune process. Because Mikulicz syndrome resembles Sjögren’s syndrome, some researchers suspect identical basis behind the two diseases.
Symptoms, complaints, and signs
Mikulicz syndrome is characterized by more or less severe swelling of the parotid glands, which in some cases results in general enlargement of the glands. Since the parotid glands are the largest glands in the body, the swelling usually causes various accompanying symptoms. For example, accompanying symptoms may include sudden extreme dry mouth with difficulty swallowing and tooth decay. In addition, the lacrimal glands often swell at the same time. Decreased tear production may occur. Swelling of other glands of the body is also conceivable. There is no pain. As a rule, the swellings are not sensitive to pressure, but merely harden. The individual symptoms do not necessarily have to persist for a long time, but can also disappear and reappear periodically. In individual cases, many other accompanying symptoms may be present, depending on the underlying disease.
Diagnosis and course of the disease
Mikulicz syndrome is usually diagnosed by tissue sampling from the affected glands. In most cases, the physician first performs palpation and ultrasonography to determine possible other causes for the swelling. In a majority of cases, patients have been diagnosed with a systemic disease long before the glandular swelling occurs. In such a case, the physician already has a first suspicion of the syndrome in the medical history. However, differential diagnosis must still exclude diseases such as mumps or Heerfordt’s syndrome. Tumors must also be excluded, usually histologically. The prognosis for patients with Mikulicz syndrome depends on the primary cause of the glandular swelling. Because the syndrome is often triggered by malignant cancers, the timing of diagnosis and the stage of the disease play a key role in the prospects for cure.
Complications
As a result of Mikulicz syndrome, affected individuals suffer from a very dry mouth. This complaint occurs mainly very suddenly and unexpectedly.Likewise, the syndrome causes significant swallowing difficulties, so that the usual intake of food and liquids is usually no longer possible for the patient. This can lead to dehydration or other deficiency symptoms, which have a very negative effect on the patient’s health. Furthermore, it is not uncommon for patients to suffer from tooth decay, so there may be pain in the teeth and other oral discomfort as well. In general, Mikulicz syndrome significantly reduces the patient’s quality of life. However, the further course of Mikulicz syndrome strongly depends on the causative underlying disease and its severity. If the disease is cancerous, it can also spread to other regions 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 causes Mikulicz syndrome.
When should you see a doctor?
Since Mikulicz syndrome is a congenital disease, a doctor should always be consulted when the particular symptoms and complaints occur. There is no self-healing and, in some cases, worsening of the symptoms. 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 with taking food and liquids. In most cases, these complaints also lead to underweight or various deficiency symptoms. If these symptoms occur, a visit to a doctor is definitely necessary. Furthermore, glands on the body may also be swollen, indicating Mikulicz syndrome. The expression of symptoms may change over time. In the first instance, Mikulicz syndrome can be detected and diagnosed by a general practitioner. However, further treatment requires various specialists to provide complete and lasting relief of symptoms.
Treatment and therapy
Treatment of patients with Mikulicz syndrome usually focuses 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, bone marrow transplantation or stem cell transplantation is usually performed, which in principle can provide a cure. In case of causative tuberculosis, the therapy consists of fourfold 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 two-drug combination is used instead of a four-drug combination. If the Mikulicz syndrome is due to sarcoidosis, there are no causal therapies available to cure the underlying disease. In such a case, the patient’s symptoms are treated symptomatically only. If, for example, too little tear fluid and saliva is produced, replacement fluids are used in the therapy. Saliva and tear substitutes can usually prevent drying of the glands and the associated secondary symptoms. Although in the case of causes such as leukemia or tuberculosis, the causal therapy of the systemic disease is the focus of treatment, combined symptomatic treatment of Mikulicz syndrome is usually carried out in addition to the causal therapy steps for these causes as well. Basically, the swellings regress as soon as the causal systemic disease is cured. ‘
Outlook and prognosis
The prognosis of Mikulicz syndrome is tied to the presenting cause of the health disorders. Spontaneous recovery is not to be expected in this disorder. Rather, severe disease progression can occur if medical help is not sought. If the swelling of the glands can be reduced by the administration of medication, freedom from symptoms is possible within a short time. If cancer is present, 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 determining the prognosis. If bone marrow transplantation is necessary, various disorders or secondary diseases may also occur. If the procedure proceeds under the best possible conditions, the patient has a good chance of permanent relief of symptoms. If the underlying disease is chronic, the chances of recovery are extremely slim. Doctors and physicians concentrate on minimizing the symptoms and reducing the intensity of existing health impairments. Long-term therapy is necessary in order to be able to react as quickly as possible in the case of existing irregularities. In addition, if there are chronic underlying conditions without medical management, the symptoms are likely to increase.
Prevention
To date, Mikulicz syndrome can be prevented only to a limited extent. In preventing systematic swelling, the same preventive measures apply as for the causative systemic diseases chronic lymphocytic leukemia, Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, sarcoidosis, syphilis, and tuberculosis. Which processes cause the swelling of the glands in the context of the above-mentioned systemic diseases is still controversial. Because the relationships are still largely in the dark, prevention of the primary diseases has been considered the only preventive measure for Mikulicz syndrome.
Follow-up
Mikulicz syndrome can lead to a number of different complications if it is not treated or if treatment is initiated very late. In this regard, early detection and subsequent treatment usually have a very positive effect on the further course of the disease and, in the process, can also prevent further worsening of the symptoms. In most cases, the syndrome leads to a significant enlargement of the glands in the body of the affected person. This leads to severe swallowing difficulties and also to a very dry mouth. The symptoms do not disappear by themselves and have a very negative effect on the quality of life of the affected person. In some cases, swelling can occur at the glands. It is not uncommon for Mikulicz syndrome to lead to tooth decay, which can cause discomfort when taking food or liquids. If the symptoms occur over a longer period of time, depression or other psychological upsets may also occur. However, the disease can usually be treated well, so that there is usually no reduced life expectancy of the affected person.
What you can do yourself
In many cases of Mikulicz syndrome, self-help is not possible. In any case, those affected are dependent on medical treatment to diagnose and combat the underlying disease. Since many affected persons are dependent on chemotherapy and suffer from the side effects of this therapy, the help and support of friends and acquaintances can help here and lead to a positive course of the disease. Likewise, the affected person should not exert himself excessively during the therapy and offer the body rest and recuperation. Since Mikulicz syndrome also frequently leads to caries, the teeth must be particularly protected. The affected person should have them examined regularly by a dentist to avoid complications. Despite the severe swallowing difficulties, the patient must be careful to take in enough food and fluids to avoid dehydration or underweight and deficiency symptoms. Usually, the low salivary flow can be treated with the help of 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.
