Epidemiology of Addison's disease in GermanyMorbus Addison's is a chronic disease caused by an autoimmune disease of the adrenal glands in more than 80% of developed countries like Germany. Autoimmune disease is a disorder based on the fact that the immune system mistakenly fights the body's own tie as a foreign body. In the first half of 20. At the beginning of the twentieth century, the most common cause of Addison's disease was tuberculosis affecting both adrenal glands, even in our country. In the meantime, adrenal tuberculosis is very rare in Germany, but still represents an important cause of Addison's disease worldwide, especially in less developed countries.
Number of affected persons
Addison's disease is rare. This very fact makes it very difficult to determine how many affected people there are in a country. Most of the figures available so far are based on extrapolations of often quite small patient groups and are thus ultimately relatively rough estimates. In Sweden and Norway, there are national patient registries in which all diseases of the population are recorded. On the basis of these registers, quite exact figures for Addison's disease can be determined for the Scandinavian countries, which are about 140 patients per 1 million inhabitants. However, we know that autoimmune diseases are more frequent in Scandinavia than in Germany, so that this figure cannot simply be transferred to our country. Various studies indicate that the disease Addison's disease seems to be increasing somewhat.
In order to gain more detailed knowledge about the prevalence of Addison's disease in Germany, the insurance data available there were evaluated in a cooperation with the Techniker Krankenkasse1. About 11-12% of the German population is insured by Techniker Krankenkasse, a large statutory health insurance company. The collaboration therefore offered the opportunity to survey the occurrence of such a rare disease in a very large group of people, so that meaningful extrapolations to the entire population were possible. Analysis of the data showed an incidence of autoimmune Addison's disease of 82-87 per 1 million population. Women were about 1.5 times more likely to develop Addison's disease than men, with 96-108 Addison's patients per 1 million population, compared with 63-68 per 1 million for men (Figure 1). Based on these figures, the number of people with autoimmune Addison's disease in Germany is about 7.000 people with autoimmune Addison's disease can be identified. The disease is therefore undoubtedly very rare. This becomes particularly clear when compared with a very common disease such as type 2 diabetes mellitus, which affects about 6 million people in our country. In addition, the analysis of the data actually showed a slight increase in the number of cases of the disease over the years studied, 2008-2011. This was particularly noticeable among women. Here, the number of people affected increased by 2.7% per year in the years studied.
Autoimmune Addison's disease often does not occur alone
It is not uncommon for patients with autoimmune Addison's disease to also have one or sometimes several other autoimmune diseases. Examination of insurance data1 showed this to be the case in 46.5%, or nearly half, of Addison's patients. The risk of having another autoimmune disease is up to 30 times higher in people with Addison's disease than in the general population. The spectrum of possible other autoimmune diseases is large. However, many of these diseases are rare. Remain this even with increased risk. By far the most common other autoimmune disease is Hashimoto's autoimmune thyroiditis, which is present in more than one third of Addison's patients. Additional type 1 diabetes mellitus could be detected in about 12% of the patients.
Addison's disease is not a hereditary disease. However, we know that there are genetic characteristics that increase the risk of developing Addison's disease and possibly other autoimmune diseases. Addison's disease occurs frequently in combination with other autoimmune diseases, because the genetic risk constellations for many of these diseases are similar. These characteristics can be inherited by the offspring. Children of people with Addison's disease therefore have – statistically – a slightly increased risk compared to the general population to also get the disease. However, Addison's disease remains a very rare disease, even for the children of those affected.
Epidemiology of Addisonian crises
Addisonian crises can occur as a potentially life-threatening complication of Addison's disease when an increased need for cortisone during infections, accidents, surgery, or in significant psychological stress situations is not adequately covered by a dose increase of hydrocortisone. The same is true when cortisone tablets cannot be properly absorbed into the body, especially during vomiting and/or diarrhea. In recent years, various studies show that Addisonian crises are probably more common than previously thought. In a large study of the University Hospital of Wurzburg, in which more than 400 patients with adrenal insufficiency were closely examined and interviewed over a period of 2 years, a crisis frequency of 8.3 per 100 patient-years was determined2. Thus, in a group of 100 patients, 8.3 Addisonian crises occurred within one year. By evaluating the insurance data of the Techniker Krankenkasse3, very similar figures were shown for patients with Addison's disease with 7.9 crises per 100 patient-years. The number of crises was even higher in patients who had at least one other autoimmune disease. Here, 10.9 crises per 100 patient-years were found. This study identified people with Addison's disease and type 1 diabetes as a subgroup at particular risk of crisis, with 12.5 crises documented per 100 patient-years (Figure 2).
These figures make it clear how important it is to develop strategies to prevent the occurrence of Addisonian crises as far as possible. In particular, this includes adequate, comprehensible and regular training of patients and their relatives and appropriate provision of emergency cards and emergency kits, as well as improvements in medical care in the event of impending or already occurring crises.
Dr. med. Gesine Meyer Hospital of the Goethe University ZIM, Med. Clinic 1 Focus Endocrinology Theodor-Stern-Kai 7 60590 Frankfurt, Germany
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