Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid

Giovanni Di Zenzo, Sybille Thoma-Uszynski, Lionel Fontao, Valentina Calabresi, Silke C Hofmann, Thomas Hellmark, Nathalie Sebbag, Cristina Pedicelli, Francesco Sera, Jean-Philippe Lacour, Jörgen Wieslander, Leena Bruckner-Tuderman, Luca Borradori, Giovanna Zambruno, Michael Hertl
Clinical Immunology: the Official Journal of the Clinical Immunology Society 2008, 128 (3): 415-26
Bullous pemphigoid (BP) is an autoimmune bullous disease, associated with autoantibodies directed against the hemidesmosomal components BP180 and BP230. In this study for the first time different laboratories have analyzed the autoantibody profile in the same group of 49 prospectively recruited BP patients. The results show that: 1) disease severity and activity correlated with levels of IgG against the BP180-NC16A domain, but also against a COOH-terminal epitope of BP180, 2) distinct epitopes of the BP180 ectodomain other than BP180-NC16A were recognized by 96% of the BP sera; and 3) the combined use of BP180 and BP230 ELISA led to the detection of IgG autoantibodies in all the BP sera. These results demonstrate the usefulness of the combined ELISAs based on various BP180 and BP230 fragments in establishing the diagnosis of BP and support the concept that BP180 is the major autoantigen of BP.

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