At the Antibody Deficiency Syndrome (AMS) is a collective term for congenital and acquired immunodeficiencies, which are particularly characterized by a deficiency in immunoglobulin G. As a result of this immune defect, there is an increased susceptibility to infection. Treatment is particularly indicated in the case of continually occurring severe infections.
What is Antibody Deficiency Syndrome?
The term antibody deficiency syndrome stands for a large number of congenital and acquired immunodeficiencies that are accompanied by a deficiency in antibodies. The congenital immune deficiencies are collectively referred to as variable immunodeficiency syndrome (CVID). A frequency of one in approximately 25,000 people is given for CVID.
It is assumed that around 800 to 3200 people in Germany suffer from the congenital form of the disease. Accordingly, CVID is a very rare syndrome. However, it is most common in relation to other congenital immunodeficiency diseases. Acquired AMS is much more common and can be caused by a variety of pre-existing conditions.
In the context of the antibody deficiency syndrome, too few antibodies of the immunoglobulin G type are formed. Immunoglobulin G is effective against bacteria and viruses. Therefore, the lack of immunoglobulin G results in a high susceptibility to infections, which mainly leads to respiratory infections. Antibody deficiency syndrome is most commonly diagnosed in both infancy and early adulthood.
causes
Antibody deficiency syndrome is a group of several genetic or acquired diseases. However, most of the gene mutations in the innate AMS are still unknown. In a few cases, however, the gene location could already be localized. It was found that various mutations of the TNFRSF13B gene on chromosome 17 can lead to immune deficiencies.
The inheritance of most immune defects is also not known. Both sporadic and familial cases of illness were noted. Various underlying diseases, poor living conditions, chemotherapy or radiation therapy can also lead to an acquired deficiency in antibodies.
As already mentioned, the main characteristic of the antibody deficiency syndrome is the lack of immunoglobulin G, which acts against bacteria and viruses. If it is missing, bacterial or viral infections can spread unchecked. The antibody deficiency is caused by defects in the regulation of the B cells. The severity of the symptoms within the syndrome varies.
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➔ Medicines to strengthen the defense and immune systemSymptoms, ailments & signs
A variety of symptoms can occur with antibody deficiency syndrome. In addition to chronic respiratory diseases, there are many other infections, digestive system disorders, skin diseases, lymph node swellings, granulomas, autoimmune diseases and tumors. Encapsulated bacteria such as Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis dominate respiratory diseases.
Enteroviruses can cause brain inflammation. Lamblia often cause diarrheal diseases and mycoplasmas often infect the urinary tract. Due to the constant diarrhea, the nutrients are no longer sufficiently absorbed. Deficiency symptoms can occur.
Sometimes the lower airways expand (bronchiectasis), which leads to constant coughing fits and sputum. Bronchiectasis is often accompanied by chronic bacterial infections that can further destroy the bronchial wall. The spleen and liver also enlarge. So-called granulomas often form in the lungs, spleen, liver and bone marrow.
These are foci of inflammation with a special structure. Skin changes such as white spot disease, hair loss or granulomas on the skin can also occur. In many cases there are also autoimmune diseases.Rheumatic joint inflammation, immunologically-related platelet or blood deficiency and pernicious anemia are often observed.
Tumors of the thymus, the lymphatic system or the stomach can also accompany the antibody deficiency syndrome. In general, it must be assumed that the life expectancy of patients with antibody deficiency syndrome is somewhat lower than that of the normal population.
However, due to the rarity of the congenital form of the disease, little statistical data is available here. Acquired forms of antibody deficiency are curable by treating the underlying disease as opposed to the congenital forms.
Diagnosis & course
In the case of recurring infectious diseases, the doctor can make the suspected diagnosis of AMS. The diagnosis is confirmed when too little immunoglobulin G is found in the blood. The immunoglobulins A and M are often also low. To distinguish between congenital and acquired AMS, other examinations are carried out, such as determining the protein excretion in the urine or the protein loss via the intestine.
When should you go to the doctor?
A doctor should be consulted immediately if the antibody deficiency syndrome is suspected. Anyone who suddenly discovers digestive system disorders, skin diseases or problems with the respiratory tract that cannot be traced back to any other cause must have this checked out medically. If the antibody deficiency syndrome is detected early, it can usually be cured without complications. If the syndrome remains undetected, however, the infections will continue to increase as the disease progresses.
At the latest when severe complaints and an increasing physical or emotional discomfort are noticed, you must see a doctor with the symptoms. In the event of organ failure or an anaphylactic shock, the emergency doctor must be consulted immediately.
People who have cases of AMS in their families should have routine check-ups and additional information about diseases of the immune system. If unusual symptoms occur that do not go away after one to two weeks at the latest, you should see your doctor. Other contact persons are rheumatologists, immunologists and specialists for the respective immunodeficiency.
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Treatment & Therapy
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For congenital antibody deficiency syndrome, treatment is only required for people who have symptoms. With this form of AMS there is no possibility of causal therapy. Patients must receive intravenous or subcutaneous infusions of immunoglobulins for life, and the infusions should take place regularly.
Intravenous infusions are given every two to six weeks. With these infusions between 200 and 600 milligrams of immunoglobulins per kilogram of body weight are injected. In the case of subcutaneous infusion, significantly fewer immunoglobulins have to be administered on a weekly basis.
Existing bacterial infections are fought with antibiotics. If there is an acquired AMS, the underlying condition needs treatment. In these cases, the AMS can completely heal.
Aftercare
The need for follow-up care for an antibody deficiency syndrome often arises from a plasmacytoma or multiple myeloma, lymphoma or blood cancer. These severe tumor diseases require professional treatment. The therapy must also keep an eye on the resulting antibody deficiency syndrome in the follow-up care.
The lack of antibodies increases the risk of infection. In an organism weakened by tumors, infections can be much more fatal than in a body that can produce enough antibodies. In addition, the radiation or chemotherapy also attack healthy cell material. This further weakens the organism that is struggling to survive.
The aftercare measures are intended to convey to the patient that he is under medical observation. This enables recurrences or changes in the tumor causing the problem to be detected more quickly. Regular follow-up care is essential if you have an antibody deficiency syndrome. The general disease risks are greatly increased.
In addition, the tumors mentioned can cause secondary damage. Regular follow-up appointments should therefore be made. Through surveys and various check-ups, they ensure that everything has been done for the quality of life of those affected.
The primary or secondary antibody deficiency syndrome can, however, also be triggered by long-term malnutrition. As a result, bacterial respiratory infections or infections in the gastrointestinal tract must be followed up. At the same time, the underlying disease or the nutritional situation that triggered it must be eliminated.
Outlook & forecast
People who are affected by congenital antibody deficiency syndrome may be symptom-free for life. You do not experience any impairments and do not have to undergo any medical treatment.
On the other hand, sick people with complaints experience recurring health problems, for which no lasting relief can be achieved. You must give yourself infusions at regular intervals so as not to experience any deterioration in your health. If the infusions are used continuously, the organism can be adequately supplied with the missing antibodies.
However, since these are not reproduced by the body to a sufficient extent and are broken down again within weeks, repeated treatment must take place in order to maintain health. If this is exposed, the state of health deteriorates considerably within a short time.
In the case of an acquired antibody deficiency syndrome, the prognosis prospects are more optimistic than in the case of the congenital syndrome. Here the organism only needs to be temporarily supplied with sufficient antibodies. Depending on the underlying disease, a single or multiple infusions can occur in the healing process.
As soon as the underlying disease has been cured or the organism has been sufficiently stabilized, it independently produces the necessary amount of the vital immunoglobulin. This leads to a permanent healing of the antibody deficiency syndrome and freedom from symptoms.
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➔ Medicines to strengthen the defense and immune systemprevention
A congenital antibody deficiency syndrome cannot be prevented. Measures can only be taken to prevent infectious diseases. This includes reducing the risk of infection. Immunocompromised people should avoid large crowds, especially in times of increased risk of infection.
A healthy lifestyle with a balanced diet and plenty of exercise helps to avoid the acquired form of AMS. You should also refrain from alcohol and smoking. A healthy lifestyle can also support therapy for the underlying disease and improve the chances of recovery.
Aftercare
The need for follow-up care for an antibody deficiency syndrome often arises from a plasmacytoma or multiple myeloma, lymphoma or blood cancer. These severe tumor diseases require professional treatment. The therapy must also keep an eye on the resulting antibody deficiency syndrome in the follow-up care.
The lack of antibodies increases the risk of infection. In an organism weakened by tumors, infections can be much more fatal than in a body that can produce enough antibodies. In addition, the radiation or chemotherapy also attack healthy cell material. This further weakens the organism that is struggling to survive. The aftercare measures are intended to convey to the patient that he is under medical observation.
This enables recurrences or changes in the tumor causing the problem to be detected more quickly. Regular follow-up care is essential if you have an antibody deficiency syndrome. The general disease risks are greatly increased. In addition, the tumors mentioned can cause secondary damage. Regular follow-up appointments should therefore be made. Through surveys and various check-ups, they ensure that everything has been done for the quality of life of those affected.
The primary or secondary antibody deficiency syndrome can, however, also be triggered by long-term malnutrition. As a result, bacterial respiratory infections or infections in the gastrointestinal tract must be followed up. At the same time, the underlying disease or the nutritional situation that triggered it must be eliminated.
You can do that yourself
The antibody deficiency syndrome (AMS), which is noticeable through a relative deficiency in immunoglobulins G, also known as gamma globulins, means a sensitive weakening of the immune system against bacterial and viral infections. Gamma globulins make up the majority of antibodies in blood plasma. They are each geared towards a specific pathogen that the immune system has already been confronted with and which provides the corresponding immune response via the immunoglobulins M.
An adaptation of behavior in everyday life and effective self-help measures require that the factors causing the disease are known. AMS can be genetic or triggered by certain circumstances such as extreme protein deficiency or by chemotherapy or radiation therapy.
If the disease was caused by genetic factors, self-help measures mainly consist of staying away from sources of infection. This means that contact with people who obviously have a cold should be avoided because the immune system is unable to counter the ingested infectious germs.
The same behavior in everyday life is also expedient for an acquired AMS if the causes are known but cannot be prevented for certain reasons, for example to achieve other health effects. In those cases in which other serious health disorders such as autoimmune diseases or tumors may be the cause of AMS, these must be clarified quickly so that an efficient therapy can be started as early as possible.
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