Systemic Scleroderma and Other Fibrotic Diseases



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:

  • Bleomycin induced lung fibrosis in mice, as measured by both hydroxyproline content assay and modified Ashcroft histopathology scoring
  • Reduction of skin thickening and collagen content in mice in which fibrosis was produced by osmotic pump delivery of bleomycin followed by pump delivery of IBIO-100
  • Reduction of hydroxyproline content of human lung tissue obtained after transplant of diseased, terminal-stage organs. Tissue fragments exhibited a significant reduction of hydroxyproline, a biomarker of fibrosis, when cultured in the presence of IBIO-100 after only 72 hours. This final result demonstrates IBIO-100 therapeutic potential in highly diseased human tissue in addition to its efficacy in animal models.

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.