Autoimmune Polyglandular Syndrome Type 1

Dear colleagues,

Autoimmune polyglandular syndrome type 1 (APS-1), also known as Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), is a rare autoimmune endocrinopathy, which can also include a number of nonendocrine features ([Vogel et al., 2002]). Most patients with APS-1 are from Sardinia, Finland, or belong to the Iranian Jewish population. A few cases have been reported in Germany. However, many colleagues are unaware of this syndrome and we believe it is likely to be considerably underdiagnosed in Western European countries. The main symptoms include adrenocortical failure, hypopararathyroidism and candidiasis. The diagnosis is established when two out of these three symptoms are present. Moreover, APECED can be accompanied by a number of additional symptoms (see Fig. [1]): Quite frequently alopecia or hypogonadism are observed in these patients. Vitiligo, hepatitis, dysfunction of the gastrointestinal tract, anaemia, thyreopathies and diabetes mellitus are less common.

Fig. 1 Incidence of clinical disorders in APECED patients.

Increasingly, the APS-1 is diagnosed in Germany, which may mean that this syndrome is more frequent in Western European countries than previously assumed. That is why we are interested in compiling data of patients with APS-1, to establish markers of early detection and to learn about its progress in order to perform possible therapeutic studies.

If you are aware of any patients with APS-1 or patients with the above-mentioned symptoms please contact us under the address mentioned below.

M. P. Manns, C. P. Straßburg, T. Lankisch

Reference

• 1 Vogel A, Straßburg C P, Brabant G, Manns M P. Autoimmun polyglanduläre Syndrome.  Deutsches Ärzteblatt. 2002;  99 A1428-1434