Ménétrier syndrome is characterized by hyperplastic mucosal folds in the stomach area and is characterized by protein loss and upper abdominal pain. The risk of degeneration of the mucosal folds is about ten percent, so patients must participate in close monitoring. Treatment is symptomatic.
What is Ménétrier syndrome?
In Ménétrier syndrome, the gastric mucosa has noticeably large hyperplasias that give the stomach a grossly wrinkled appearance. The syndrome is also called Ménétrier’s disease or hypertrophic gastropathy and is a rather rare disorder, with no precise data on its prevalence. The disease is considered to be one of the exudative gastroenteropathies characterized by a loss of protein in the gastrointestinal tract. The syndrome affects mainly middle-aged men and owes its name to the first describer, Pierre E. Ménétrier. Due to its low prevalence, the disease has not yet been conclusively researched. The causes, for example, can only be speculated about. The disease can vary in severity and therefore does not always have to be symptomatic, but can also be largely silent.
Causes
The causes of Ménétrier syndrome have so far been rather speculative. For example, there is a connection with a Helicobacter pylori infection, which is often symptomatic of the disease. In addition, the affected persons often report an infection with the cytomegalovirus, which they suffered in childhood. However, both the concomitant finding of Helicobacter pylori and the cytomegalovirus infection do not necessarily have to be causally related to Ménétrier syndrome. To establish such a link, controlled studies and case reports are mainly lacking so far. A genetic disposition at least does not seem to be related to the disease. Familial clustering, for example, has not been observed. A sporadic occurrence is to be suspected. Thus, a genetic cause with a hereditary basis is largely excluded.
Symptoms, complaints, and signs
The stomach undergoes pathologic changes in Ménétrier syndrome. Macroscopically, the gastric mucosa appears thickened and has markedly coarsened folds, which are most apparent in the large curvature of the stomach. Cystic enlargement and elongation are evident at the foveolae. Often, the cysts are slimy. The gastric glands are affected by degeneration and lose their acid-producing vestibular cells. The mucosa of the stomach is distended by edema and is populated by inflammatory cells. Eosinophilia is often present. Single muscle cell bars extend into the gastric mucosa. Achlorhydria may also be symptomatic. Inflammatory reactions at the gastric mucosa are accompanied by loss of plasma protein and often result in hypoproteinemia. When parietal cells are destroyed, anemia is especially often present. Diarrhea is one of the most common symptoms. Depending on the severity, individual symptoms may be absent.
Diagnosis and course of the disease
To diagnose Ménétrier’s disease, the physician performs a gastroscopy. During this procedure, he takes biopsies from the most conspicuous areas. Histologic examination of the biopsies should be accompanied by testing for Helicobacter pylori. In individual cases, the diagnosis can be made on suspicion by ultrasound examination of the stomach. This can be followed by gastroscopy to confirm the suspected diagnosis. The course of the disease depends on its severity. The most serious complication for Ménétrier syndrome is malignant degeneration of the hyperplasias. The risk of transition to gastric carcinoma is reported to be about ten percent. However, regular control examinations can largely prevent severe complications of this type. Lethal consequences are rather not to be expected for this reason.
Complications
First and foremost, those affected with Ménétrier syndrome suffer from relatively severe pain in the upper abdomen. These can thus also lead to a loss of appetite and further to underweight or deficiency symptoms. In many cases, this pain has a very negative effect on the psyche of the affected person, so that the patients suffer from depression or other psychological upsets.The risk of cancer is also significantly increased due to Ménétrier’s syndrome, so that those affected are usually dependent on various regular examinations in order to avoid complications in the process. Furthermore, the formation of cysts or inflammations also occurs. In most cases, these are associated with pain in the stomach. Diarrhea can also be associated with Ménétrier syndrome and has a very negative effect on the quality of life of the affected person. Sometimes this results in permanent dehydration. Treatment of Ménétrier syndrome is symptomatic in most cases and is not associated with complications. Sufferers can fight the infections with the help of antibiotics. However, in the case of a tumor, it must be removed with the help of surgery. It may also reduce the patient’s life expectancy.
When should you go to the doctor?
Ménétrier syndrome does not always cause noticeable symptoms. In some cases, a prolonged period of symptomlessness is documented, making diagnosis difficult. Digestive tract disorders, diarrhea, or a general feeling of illness should be investigated and treated. Often, pain is persistent until noticed by the affected individual. As soon as changes or irregularities occur, a physician should be consulted. If there is pain, a loss of appetite or a decrease in body weight, the affected person needs help. If various deficiency symptoms occur, or if there are diffuse functional disorders, cramps or internal weakness, a visit to the doctor is advisable. If the physical complaints are accompanied by emotional or psychological problems, if there is a withdrawal from social life or if the patient suffers from mood swings, a doctor is needed. If there are behavioral abnormalities, an increased need for sleep or rapid fatigue when performing light activities, a visit to the doctor is recommended. Irregularities are present, the cause of which must be determined. Swellings on the body, palpable lumps or indurations should always be presented to a doctor. If they increase in size or frequency, a visit to the doctor should be initiated immediately.
Treatment and therapy
A causative therapy for Ménétrier syndrome does not yet exist. Therefore, the disease is treated symptomatically. Drug therapy may be used for upper abdominal pain. If an infection with Helicobacter pylori has been detected and chronic gastritis type B is present, treatment of the infection is the focus of symptomatic therapy. The findings may regress after successful treatment of the Helicobacter. Therefore, a few weeks after therapy, the patient’s stomach is monitored for improvement. If the original findings persist despite successful treatment of the infection, the patient will henceforth participate in closely scheduled gastroscopies to monitor the findings. If changes are seen during these check-ups, biopsies are taken. If these biopsies show progressive changes and progressively increasing dysplasia, a gastrectomy must be performed as a precaution. This resection of the stomach corresponds to a surgical procedure in which all altered areas are removed. Subsequently, reconstruction of the affected passages may be performed if necessary. Resection is a preventive measure against possible degeneration. If regular checks of the stomach show no more changes and the disease is thus not progressive but has come to a standstill, resection of the stomach is not usually performed. In this case, the patient receives symptomatic treatments depending on his or her symptoms, which are primarily intended to improve his or her quality of life.
Outlook and prognosis
Ménétrier syndrome is treated purely symptomatically. The patient can support the therapy by adopting a low-impact lifestyle and dietary changes. Because of the severe gastrointestinal symptoms, warming compresses are a good option. Acute relief is also provided by natural painkillers. Their use should first be discussed with the doctor in order to avoid complications. Rest and recuperation are recommended after surgery. Patients should follow the doctor’s instructions to avoid complications. This also includes a change in diet.After a gastrectomy, heavy or particularly irritating foods must first be avoided. Alcohol, nicotine and caffeine must also be avoided so as not to further irritate the gastrointestinal tract. The details of the change in diet should be discussed with a nutritionist or the specialist in charge. Sporting activities may be resumed a few days after the operation. Here, too, it is advisable to consult the doctor first, as there is a risk of serious complications if the body is put under strain again too soon. If, despite these measures, gastrointestinal complaints, upper abdominal pain or other typical symptoms occur again, the physician must be informed.
Prevention
Ménétrier syndrome is of as yet unknown etiology. For this reason, there is no promising way to prevent the disease. However, patients with the syndrome can at least prevent malignant degeneration of the hyperplastic gastric mucosa by regular checkups and biopsies of the stomach and, if necessary, have a gastric resection performed in time.
Follow-up
In most cases, very few measures or options for follow-up care are available to the affected person with Ménétrier syndrome. Here, early diagnosis is primarily necessary to prevent further worsening of symptoms. As a rule, self-healing cannot occur in Ménétrier syndrome, so that the patient should ideally consult a doctor at the first signs and symptoms. Those affected are dependent on taking various medications. Regular intake with the correct dosage should always be observed. In case of ambiguities or questions, a doctor should always be consulted first. Furthermore, frequent checks and regular examinations by a doctor are very important in this disease in order to detect further damage at an early stage. The diet of the affected person should also be adjusted, and the doctor can also create a diet plan. If Ménétrier’s syndrome is treated by surgery, it is recommended for the affected person to rest and take care of the body after the surgery. In this regard, efforts or physical activities should be refrained from. Likewise, only light food should be taken, although the body can readapt to ordinary food over time.
This is what you can do yourself
These patients are followed up closely because there is a high risk that their disease will degenerate. The checkups can also detect and treat other complications of the disease at an early stage, which is why it is essential to keep these examination appointments. The fact that the disease is rare and still largely unexplored can be unsettling for those affected. However, they can find the latest information on the self-help portal for rare diseases (www.orpha-selbsthilfe.de). There they can also make contact with other sufferers, which experience has shown can be helpful. Depending on the severity of the disease, those affected suffer from sometimes severe pain as well as diarrhea. They may therefore take little part in normal social life. This can wear patients down and trigger depression or other psychological disorders. Psychological therapy support is therefore urgently recommended. Patients with Ménétrier syndrome must be careful to drink enough fluids, especially if they are prone to diarrhea. Otherwise, their body will dehydrate, which will weaken it at the same time. Despite possible pain, patients should also eat enough food to avoid provoking deficiency symptoms. The diet should consist of healthy and natural foods, but also be easily digestible. In times of crisis, cooked and pureed foods are recommended. Old home recipes, such as gruel, can be spiced up tastily, are nutritious and well tolerated.
