Unlike other autoimmune blistering diseases, eosinophils appear to play a significant role in the pathophysiology of Bullous Pemphigoid (BP) as lesions have been shown to contain eosinophil-rich infiltrates in the upper dermis. Furthermore, successful therapy of BP has been demonstrated with the use of anti-IgE antibody treatment, an important aspect as eosinophils from BP patients express the high-affinity IgE receptor FcƐRI.1 The role of eotaxin in the recruitment of eosinophils into inflammatory sites has been described and the specific eotaxin receptor, CCR3, has been documented to be expressed on eosinophils, basophils, and Th2 cells. Eotaxin-1 binds CCR3 on eosinophils and ultimately drives chemotaxis and degranulation. The expression of both eotaxin-1 and CCR3 in lesional skin from patients with active BP and control subjects affected with pemphigus vulgaris (PV) was analyzed in a study.2 A strong immunostaining for eotaxin-1 and CCR3 was observed in BP skin specimens and eotaxin serum levels were significantly higher in BP patients when compared to healthy donors (P=0.003) and PV patients (P=0.01).
All these results confirm the role of eotaxin-1 in the recruitment of activated cells at inflammatory sites during BP and the expression of CCR3 further supports the involvement of Th2 cells in the pathogenesis of this disease.
Bertilimumab is an anti-eotaxin-1 antibody with a high affinity and specificity for eotaxin-1. It blocks eotaxin-mediated chemotaxis and inhibits eotaxin-1-induced eosinophil conformational change.
To evaluate the safety, efficacy, and pharmacodynamic effect of bertilimumab in patients with BP.
Type of study (NCT02226146)3
Primary Outcome Measure
Secondary Outcome Measure
Change in Bullous Pemphigoid Disease Area Index (BPDAI) score.
Present disclosure: The presenter has reported honoraria (grants/research funding) from Immune Pharmaceuticals.
Written by: Debbie Anderson, PhD
Reviewed by: Victor Desmond Mandel, MD