부산대학교 도서관

Summary: Background: Bullous pemphigoid (BP) is a subepidermal blistering disease characterized by autoantibodies against the two hemidesmosomal proteins, BP180 (type XVII collagen) and BP230. 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‐BP180 NC16A (16th noncollagenous domain) and anti‐BP230 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‐BP180 levels but not with levels of anti‐BP230 IgG and anti‐BP180 IgE. High levels of both anti‐BP180 IgG and anti‐BP230 IgG were associated with a low Karnofsky score. The presence of anti‐BP230 IgG was more frequent in older patients. Those with higher total IgE serum levels suffered from fewer adverse events. Higher IgG anti‐BP180 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. What's already known about this topic? Serum levels of immunoglobulin (Ig)G autoantibodies against BP180 correlate with disease activity in bullous pemphigoid (BP).Reports about the correlation of serum anti‐BP180 IgE with disease severity are inconclusive.Karnofsky score is a predictor of death in BP but to date has not been shown to be related to serum autoantibody levels. What does this study add? High serum anti‐BP180 IgG levels are associated with an increased mortality in BP.Patients with BP with a lower Karnofsky score show higher serum levels of anti‐BP180 and anti‐BP230 IgG.High levels of total serum IgE appear to be related to a lower number of treatment‐related adverse events.Patients with BP with high anti‐BP180 IgG may be at risk of fatal outcomes, while in those with high total IgE, fewer adverse events can be expected. Linked Comment: Hashimoto and Tsuruta. Br J Dermatol 2018; 179:820–821. Plain language summary available online Respond to this article [ABSTRACT FROM AUTHOR]