Systemic sclerosis is a rare chronic disease of uncertain etiology characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs. A number of medications can slow the progression of specific existing symptoms or temporarily reduce the development of new symptoms, but there remains an unmet need for more effective treatment.
IBIO-100 is a fusion of the endostatin derived E4 antifibrotic peptide to the hinge and heavy chain of human IgG1. Based upon the work (Science translational medicine vol. 4,136 (2012): 136ra72. doi:10.1126/scitranslmed.3003421) by iBio's collaborator Dr. Carol Feghali-Bostwick, IBIO-100 has been shown to be effective using infusion and oral administration schemes in animal models, a novel aspect of a biotherapeutic protein of its type. It has been granted orphan-drug designation by the FDA for treatment of systemic sclerosis.
In preclinical studies, IBIO-100 has been shown to reduce fibrotic symptoms, including:
Further studies are being conducted to support an Investigational New Drug (IND) application. The next milestones will be toxicology and cGMP manufacturing in our Bryan, Texas facility.