Marine-Lenhart syndrome is a relatively rare condition. Graves’ disease or another autoimmune thyreopathy associated with hyperthyroidism occur here along with warm thyroid nodules. Differential diagnosis is difficult; the symptoms of the syndrome are largely similar to those of Graves’ disease and hyperthyroidism.
What is Marine-Lenhart syndrome?
Marine-Lenhart syndrome is a variant of Graves’ disease in which coexistent autonomous thyroid nodules are present. It is also commonly referred to as Graves’ disease with coexistent multinodular goiter or nodular Graves’ disease, as it is considered by many to be a subtype of Graves’ disease. The syndrome occurs rarely, with a reported prevalence ranging from 1-4.1 percent in patients with Graves’ disease. The autoimmune disease with stimulating autoantibody occurs at the TSH receptor in a coexisting nodular gland. The syndrome was first described in 1911 by American surgeons David Marine and Carl H. Lenhart. In studies of goiter, they encountered eight cases in which goiter was associated with nodules in the thyroid gland.
Causes
Marine-Lenhart syndrome is an autoimmune disease of the thyroid gland. In this case, Graves’ disease is grafted onto a pre-existing goiter. On the one hand, this is caused by a genetic defect of the immune system. On the other hand, external influences such as smoking, viral infections or psychosocial stress intensify the disease. The interaction of several factors eventually leads to a breakdown of the thyroid gland‘s self-tolerance to antigens. This results in an autoimmune disease. The autoantibodies formed bind to the TSH receptor. Through their intrinsic activity, they stimulate the follicular epithelial cells of the thyroid. This leads to increased iodine uptake, increased production and secretion of thyroid hormones, and ultimately to hyperthyroidism. Thus, in Graves’ disease, the TSH receptors are damaged and destroyed, resulting in uncontrolled production of SD hormones. Marine-Lenhart syndrome results from the association of Graves’ disease with focal autonomy. These are demarcated nodules that produce SD hormones uncontrollably and independently.
Symptoms, complaints, and signs
Possible symptoms include all complaints that can also occur in hyperthyroidism. These range from the typical goiter to insomnia, fatigue, irritability and tremors. High blood pressure, heart fibrillation or unwanted weight loss can also be the consequences. Concomitantly, the mobilization of glycogen and fat reserves leads to glucose tolerance, possibly even hyperglycemia. Furthermore, the syndrome also makes itself felt through the symptoms of Graves’ disease. These include protruding eyeballs, myxedema, muscle weakness, osteoporosis, and cycle disturbances that can lead to temporary infertility. Heat intolerance, sweating and increased stool frequency may also be among the signs. When the nodes press on the windpipe, it results in difficulty swallowing and a tight feeling in the throat.
Diagnosis and disease progression
Differential diagnostic challenges arise in determining the presence of the disease. During primary hyperthyroidism, the nodules initially appear as scintigraphically cold. However, after therapy, they turn out to be normally storing or warm areas. A blood sample is initially taken for diagnosis. Here, the antibodies TPO and TG are searched for. These are specific antibodies used to detect Graves’ disease and thyroid disorders. An ultrasound examination is then performed to look for both nodules and thyroiditis. Features suggestive of thyroid inflammation include a diffusely enlarged gland, echo-deficient glandular tissue, and markedly increased blood flow, known in English as “thyroid inferno.” Although abnormalities on ultrasound examination are usually indistinguishable from symptoms of Hashimoto’s thyroiditis, the clinical picture and blood tests make a diagnosis easy. SD scintigraphy provides additional clarity.
Complications
Marine-Lenhart syndrome results in many different complaints that significantly reduce the quality of life of the affected person. In most cases, patients thereby suffer from severe fatigue, which is produced by insomnia. As a result, it is not uncommon for sufferers to be irritable and leiht aggressive. Likewise, high blood pressure occurs, so that the patients can also suffer a heart attack, which in the worst case can be fatal. It is not uncommon for weight loss and muscle weakness to occur. The patient’s daily life is severely restricted by Marine-Lenhart syndrome and sweating or swallowing difficulties occur. Furthermore, the swallowing difficulties can lead to a restricted intake of fluids and food, resulting in dehydration or various deficiency symptoms. In most cases, no surgical intervention is necessary for Marine-Lenhart syndrome and treatment can be carried out with the help of medications and radioactive preparations. There are no further complications or discomforts and the patient’s life expectancy is not reduced with early treatment. However, affected individuals are usually dependent on hormones for the rest of their lives.
When should you see a doctor?
Sleep disturbances, fatigue or tiredness are signs of an existing health condition. If the condition persists or increases in intensity, a doctor is needed. If there is internal agitation, irritability, or tremors, there is cause for concern. A physician is needed so that relief can be initiated by providing medical care to the individual. Disturbances of the heart rhythm, high blood pressure, an internal heat or interruptions of the heartbeat should be examined and clarified by a physician as soon as possible. If there is a sudden and unwanted decrease in body weight, this is a sign of an existing irregularity. Complaints of the swallowing cycle, a refusal to eat, and visual changes in the face should be presented to a physician. In case of sweating or a decrease in the usual physical performance, a visit to the doctor is advisable. If the patient is no longer able to perform his or her daily duties, if there is a decrease in participation in social activities, or if other behavioral problems occur, the patient needs help and support. Difficulty breathing or a feeling of tightness in the throat should be evaluated by a physician. Weakness of the muscles and, in sexually mature women, irregularity of the menstrual cycle, are other signs of a health disorder. Action is needed so that a diagnosis can be made.
Treatment and therapy
Initial therapy usually consists of taking thyrostatic drugs. However, this treatment should be given only for a limited time until eutheryosis, or normal thyroid function, is achieved. SD hormone-inhibiting drugs help about half of the patients to recover. Once thyroid hormone levels are under control, other treatment options are considered. If malignancy has been ruled out, radioiodine therapy can be used. This nuclear medicine procedure uses the radioactive isotope of iodine. Predominantly a beta emitter, it has a half-life of eight days. In the human body, it is found exclusively in cells of the thyroid gland. The beta rays damage the DNA surrounding the thyroid cells, eliminating the affected cells. Because patients affected by Marine-Lenhart syndrome have often developed resistance to radioiodine, a higher dosage is required. As a result, many affected individuals have been successfully treated in the past. In the past, surgery was considered the only effective therapy. Even today, in the case of multiple nodules, their surgical removal can lead to success. Removal of the entire thyroid gland is also conceivable. In this case, however, patients must take hormones for the rest of their lives.
Outlook and prognosis
Marine-Lenhart syndrome has an unfavorable prognosis. The disease is due to a genetic defect that has no cure to date. Alteration of human genetics is not permitted for legal reasons.Doctors and physicians focus their attention in a treatment on the use of symptomatic approaches. The individually occurring complaints are treated in the best possible way by different therapies. Hormonal preparations lead to about half of the patients becoming largely symptom-free. However, if the medication is discontinued, the symptoms are likely to return. Therefore, lifelong drug treatment is necessary to stabilize the quality of life of the affected person. If the course of the disease is unfavorable, secondary disorders occur. The cardiovascular system is subjected to enormous stress. Therefore, premature death due to heart failure is documented in some patients. In addition, malignant disease development may occur. Here, too, the affected person is threatened with a reduction of the average lifespan. In addition to the administration of hormones, a stable psyche is of particular importance as helpful and supportive. Since the affected person has to undergo a long-term therapy, measures for self-help as well as the avoidance of harmful substances are important. With a healthy and stable mental state, improvements in the overall situation can often be observed.
Prevention
Since Marine-Lenhart syndrome is related to Graves’ disease, the same risk factors apply to both conditions. Thus, psychosocial stress should be avoided as much as possible. Relaxation exercises can help defuse stressful situations. Smoking can also promote the development of the disease. In addition, viral diseases should be carefully treated and cured.
Aftercare
Marie-Lenhart syndrome may result in lifelong follow-up care. This is independent of the particular treatment method. It is also necessary to prevent ocular symptoms due to endocrine orbitopathy, which is possible in about 50% of all patients. In addition, follow-up treatment requires a lot of effort and patience. Thus, therapeutic strategies are medium- to long-term. In the case of conservative drug therapy, the patient receives thyrostatic drugs for one to two years. Depending on the initial situation, the risk of recurrence is 30 to 90 percent. Follow-up examinations must take place every four to eight weeks. Radioiodine therapy and surgery are considered the safest and fastest treatments for Graves’ disease. Following these procedures, however, it is necessary to take thyroid hormones for the rest of one’s life. This is the only way to compensate for the resulting hypothyroidism, i.e. a deficiency of thyroid hormones. If regular check-ups are necessary at the beginning, they are limited to one or two examinations a year as the disease progresses. Immediately after a surgical procedure on the thyroid gland, the patient receives thyroid hormones in a standard amount. How many hormones the patient ultimately needs is determined in the period after surgery and adjusted accordingly on an individual basis. Target levels vary and are determined by the patient’s primary care physician or an endocrinologist.
Here’s what you can do yourself
Marine-Lenhart syndrome is primarily treated with medication. Patients can support thyroid therapy by taking it easy and informing their doctor if they experience unusual symptoms. In general, it is advisable to keep a diary in which any symptoms of the disease and side effects of the medication used are noted. This makes it easier for the doctor to adjust the medication optimally to the patient’s individual condition. Radioiodine therapy can also be supported by the patient through good cooperation with the responsible physician. If these measures have no effect, surgery must be performed. After an operation, the diet must be changed to ensure a rapid healing process. Those affected should also avoid stimulants such as alcohol and caffeine and, if necessary, stop smoking. The individual symptoms must also be treated. A walk in the fresh air often helps against sweating. Irritability and trembling can be counteracted by targeted relaxation measures. Stress and physical exertion should be avoided as far as possible until the symptoms of Marine-Lenhart syndrome have subsided.If cardiovascular problems or cycle disturbances are added, other physicians may need to be consulted.
