To rule out the possible influence of diabetes on our results, we have analysed differences in fibrinolysis and coagulation parameters between BP patients and normal controls after the exclusion of the three diabetic BP patients and their RAD001 mw sex- and age-matched

controls. In the 17 BP patients with active disease, PAI-1 antigen and active PAI-1 levels were significantly higher (22·13 ± 8·68 ng/ml and 16·76 ± 5·55 ng/ml) than in the 17 sex- and age-matched healthy controls (8·65 ± 6·29 ng/ml and 6·21 ± 4·37 ng/ml) (P = 0·0001 for both). Plasma t-PA levels were also significantly higher in the 17 patients (36·91 ± 32·02 ng/ml versus 6·09 ± 4·45 ng/ml; P = 0·0001). Finally, plasma d-dimer and F1+2 levels were both markedly higher in

the 17 patients with active BP (2774 ± 3817 ng/ml and 631 ± 487 ng/ml) than in the 17 controls (183 ± 107 ng/ml and 106 ± 44 ng/ml) (P = 0·0001 for both). In the patients with active BP, disease severity (expressed as the percentage of involved body surface area) correlated significantly with the number of blood eosinophils (r = 0·705, P = 0·01) and the plasma levels of d-dimer (r = 0·713, P = 0·0001) and F1+2 (r = 0·703, P = 0·001). Plasma CRP levels correlated directly with the levels of PAI-1 antigen (r = 0·722, P = 0·0001), PAI-1 activity (r = 0·514, P = 0·021), t-PA antigen (r = 0·547, P = 0·012) and F1+2 (r = 0·450, P = 0·047) and the number of blood eosinophils MK 1775 correlated with PAI-1 antigen (r = 0·585, P = 0·046), PAI-1 activity (r = 0·680, P = 0·015) and d-dimer (r = 0·710, P = 0·010). Anti-BP180 autoantibody levels only correlated with d-dimer (r = 0·495,

P = 0·026) and F1+2 (r = 0·458, P = 0·042). In the 20 BP patients during remission after treatment, the levels of PAI-1 antigen and active Oxalosuccinic acid PAI-1 decreased significantly from 25·06 ± 8·88 ng/ml to 16·99 ± 7·05 ng/ml and from 15·65 ± 5·75 ng/ml to 11·19 ± 5·14 ng/ml (P = 0·008 and P = 0·006, respectively) (Fig. 1). The mean differences were 5·30 ng/ml [95% confidence interval (CI): 1·65–8·96 ng/ml] for PAI antigen and 4·00 ng/ml (95% CI: 1·66–6·35 ng/ml) for active PAI. There was an albeit not significant decrease in tPA levels (from 34·70 ± 33·22 to 32·74 ± 27·80 ng/ml). Plasma TAFI levels did not change significantly (Fig. 2), but there was a significant decrease in the plasma levels of d-dimer (from 2350 ± 3676 ng/ml to 571 ± 651; P = 0·0001) and F1+2 (from 551 ± 484 ng/ml to 188 ± 216; P = 0·0001). The mean differences were 2804 ng/ml (95% CI: 744–4865 ng/ml) for d-dimer and 414 ng/ml (95% CI: 191–638 ng/ml) for F1+2.