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Prospective study in bullous pemphigoid: association of high serum anti‐ BP 180 IgG levels with increased mortality and reduced Karnofsky score
Author(s) -
Holtsche M.M.,
Goletz S.,
Beek N.,
Zillikens D.,
Benoit S.,
Harman K.,
Walton S.,
English J.,
Sticherling M.,
Chapman A.,
Levell N.J.,
Groves R.,
Williams H.C.,
König I.R.,
Schmidt E.
Publication year - 2018
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.16553
Subject(s) - bullous pemphigoid , medicine , autoantibody , pemphigoid , serology , prospective cohort study , gastroenterology , immunology , antibody
Summary Background Bullous pemphigoid ( BP ) is a subepidermal blistering disease characterized by autoantibodies against the two hemidesmosomal proteins, BP 180 (type XVII collagen) and BP 230. The multicentre prospective BLISTER (Bullous Pemphigoid Steroids and Tetracyclines) trial randomized 253 patients with BP to compare the benefits and harms between initial treatment with doxycycline or prednisolone. Objectives To analyse distinct autoantibody profiles for the prediction of the disease course in a well‐characterized cohort of BP sera. Methods One hundred and forty‐three patients of the BLISTER trial consented to participate in this serological study. Sera taken at baseline were analysed by (i) indirect immunofluorescence, (ii) anti‐ BP 180 NC 16A (16th noncollagenous domain) and anti‐ BP 230 enzyme‐linked immunosorbent assay and (iii) immunoblotting with various substrates. Results were then linked with clinical parameters including age, Karnofsky score, number of blisters, related adverse events and mortality. Results Disease activity correlated with immunoglobulin (Ig)G anti‐ BP 180 levels but not with levels of anti‐ BP 230 IgG and anti‐ BP 180 IgE. High levels of both anti‐ BP 180 IgG and anti‐ BP 230 IgG were associated with a low Karnofsky score. The presence of anti‐ BP 230 IgG was more frequent in older patients. Those with higher total IgE serum levels suffered from fewer adverse events. Higher IgG anti‐ BP 180 levels were associated with an increased 1‐year mortality rate. Conclusions Analysis of the autoantibody profile is not only of diagnostic relevance but may also be helpful in predicting the course of the disease.

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