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Fibrotic Diseases and Unmet Needs

Idiopathic pulmonary fibrosis [IPF] is a form of progressive pulmonary fibrosis, or abnormal scarring of the lungs. As the disease progresses, the increased scarring leads to decreasing transfer of oxygen into the bloodstream, and ultimately, irreversible loss of lung function. The average life expectancy after an IPF diagnosis is 3-5 years.1 Because there is no cure and no therapy has been shown to halt or reverse the progressive deterioration of lung function, the primary goal of IPF treatment is to slow disease progression, maintain or improve quality of life, and prolong survival.

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.

human lungs, relevant to idiopathic pulmonary fibrosis (IPF), for which IBIO-100 candidates are in development for therapeutic applications

iBio’s Approach

The lead candidates are a fusion of the endostatin derived E4 antifibrotic peptide to the hinge and heavy chain of human IgG1. Based upon work by Dr. Carol Feghali-Bostwick, IBIO-100 candidates have been shown to be effective using infusion and oral administration schemes in animal models, a novel aspect of a biotherapeutic protein of its type2.

IBIO-100 candidates have been shown to address symptoms of fibrosis in highly diseased human tissue in addition to efficacy in preclinical models:
  • As measured by both hydroxyproline content assay and modified Ashcroft histopathology scoring, bleomycin induced lung fibrosis in mice was reduced
  • Reduced skin thickening and collagen content in mice (experimental design included osmotic pump delivery of bleomycin followed by pump delivery of IBIO-100)
  • IBIO-100 induced reduction of hydroxyproline content (a biomarker of fibrosis) of human lung tissue obtained after transplant of diseased, terminal-stage organs after only 72 hours.
  • Orphan drug designation was granted by the US FDA for the systemic scleroderma indication.
Sample results from preclinical development testing. Bleomycin increases skin and collagen thickness. Administration of IBIO-100 reduced thickness.
  1. Scelfo C, Caminati A, Harari S. Recent advances in managing idiopathic pulmonary fibrosis. F1000Res. 2017;6:2052. Published 2017 Nov 27. doi:10.12688/f1000research.10720.1
  2. Science translational medicine vol. 4,136 (2012): 136a72. doi:10.1126/scitranslmed.3003421