TY - JOUR A1 - Recke, Andreas A1 - Konitzer, Sarah A1 - Lemcke, Susanne A1 - Freitag, Miriam A1 - Sommer, Nele Maxi A1 - Abdelhady, Mohammad A1 - Amoli, Mahsa M. A1 - Benoit, Sandrine A1 - El-Chennawy, Farha A1 - Eldarouti, Mohammad A1 - Eming, Rüdiger A1 - Gläser, Regine A1 - Günther, Claudia A1 - Hadaschik, Eva A1 - Homey, Bernhard A1 - Lieb, Wolfgang A1 - Peitsch, Wiebke K. A1 - Pföhler, Claudia A1 - Robati, Reza M. A1 - Saeedi, Marjan A1 - Sárdy, Miklós A1 - Sticherling, Michael A1 - Uzun, Soner A1 - Worm, Margitta A1 - Zillikens, Detlef A1 - Ibrahim, Saleh A1 - Vidarsson, Gestur A1 - Schmidt, Enno T1 - The p.Arg435His Variation of IgG3 With High Affinity to FcRn Is Associated With Susceptibility for Pemphigus Vulgaris-Analysis of Four Different Ethnic Cohorts JF - frontiers in Immunology N2 - IgG3 is the IgG subclass with the strongest effector functions among all four IgG subclasses and the highest degree of allelic variability among all constant immunoglobulin genes. Due to its genetic position, IgG3 is often the first isotype an antibody switches to before IgG1 or IgG4. Compared with the other IgG subclasses, it has a reduced half-life which is probably connected to a decreased affinity to the neonatal Fc receptor (FcRn). However, a few allelic variants harbor an amino acid replacement of His435 to Arg that reverts the half-life of the resulting IgG3 to the same level as the other IgG subclasses. Because of its functional impact, we hypothesized that the p.Arg435His variation could be associated with susceptibility to autoantibody-mediated diseases like pemphigus vulgaris (PV) and bullous pemphigoid (BP). Using a set of samples from German, Turkish, Egyptian, and Iranian patients and controls, we were able to demonstrate a genetic association of the p.Arg435His variation with PV risk, but not with BP risk. Our results suggest a hitherto unknown role for the function of IgG3 in the pathogenesis of PV. KW - immunology KW - dermatology KW - autoantibodies KW - allotype KW - pemphigus KW - Diagnose KW - pemphigoid KW - half-life KW - functional genetics Y1 - 2018 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-225073 VL - 9 ER - TY - JOUR A1 - Schmidt, Enno A1 - Sticherling, Michael A1 - Sárdy, Miklós A1 - Eming, Rüdiger A1 - Goebeler, Matthias A1 - Hertl, Michael A1 - Hofmann, Silke C. A1 - Hunzelmann, Nicolas A1 - Kern, Johannes S. A1 - Kramer, Harald A1 - Nast, Alexander A1 - Orzechowski, Hans‐Dieter A1 - Pfeiffer, Christiane A1 - Schuster, Volker A1 - Sitaru, Cassian A1 - Zidane, Miriam A1 - Zillikens, Detlef A1 - Worm, Margitta T1 - S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid: 2019 update JF - JDDG: Journal der Deutschen Dermatologischen Gesellschaft KW - pemphigus vulgaris KW - pemphigus foliaceus KW - S2k guidelines Y1 - 2020 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-217806 VL - 18 IS - 5 SP - 516 EP - 526 ER -