In patients with bullous pemphigoid (BP), high serum levels of immunoglobulin G (IgG) autoantibodies against BP180 (anti-180) are associated with increased mortality and low Karnofsky scores, according to the results of a prospective, multicenter study of a subgroup of patients from the randomized Bullous Pemphigoid Steroids and Tetracyclines study. Results of the current study were published in the British Journal of Dermatology.
The investigators sought to identify serologic markers that might predict the disease course and clinical outcomes of BP. Of the 253 patients with BP enrolled in the original trial, a total of 143 (69 women, 74 men; mean age, 77) consented to participate in the current serologic study. Among the participants, 42 had mild disease (<10 blisters), 59 had moderate disease (10-30 blisters), and 42 had severe disease (>30 blisters) at study entry.
Serum obtained at baseline from the participants was analyzed with indirect immunofluorescence, anti-BP180 NC16A and anti-BP230 enzyme-linked immunosorbent assay, and immunoblotting with different substrates. Results of the serologic analysis were then linked with such clinical parameters as age, Karnofsky score, number of blisters, related adverse events, and mortality.
Pretreatment anti-BP180 NC16A IgG serum levels were significantly associated with disease severity (P <.001) but not with levels of anti-BP230 IgG and anti-BP180 NC16A IgE. Moreover, high levels of both anti-BP180 NC16A IgG and anti-BP230 IgG were significantly linked with a lower Karnofsky score (P <.001 and P =.002, respectively).
The presence of anti-BP230 IgG was significantly more common among older participants (P =.016). In addition, those with higher total IgE serum levels experienced fewer adverse events. A significantly increased 1-year mortality rate was reported among patients with higher anti-BP180 NC16A IgG levels (P =.021).
Study limitations included that serum was obtained from the participants at baseline only and not during the course of their disease.
The investigators concluded that in patients with BP, analysis of their autoantibody profile is of diagnostic relevance and may also assist in predicting the course of their disease.
Holtsche MM, Goletz S, van Beek N, et al; BLISTER Study Group. Prospective study in bullous pemphigoid: association of high serum anti-BP180 IgG levels with increased mortality and reduced Karnofsky score [published April 1, 2018]. Br J Dermatol. doi: 10.1111/bjd.16553