Open Label Extension (OLE) for the Patients Treated in the ISD002-P144-07 Study
Status:
Completed
Trial end date:
2010-12-01
Target enrollment:
Participant gender:
Summary
Transforming growth factor-beta 1 is consistently over expressed in most fibrotic diseases
and displays a variety of profibrotic effects in fibroblasts(25, 26). Activation of TGF-beta
receptors induces the activation of several kinase signalling cascades leading to the
phosphorylation of SMAD proteins as well as to the activation of SMAD-independent kinases
that collectively activate ECM synthesis and fibroblast growth and differentiation into
myofibroblasts. TGF-beta 1 i one of the main mediators in the fibrotic process, associated to
both scarring and a long list of pathologies related to chronic inflammation and which affect
all type of organs and tissues. An increase in TGF-beta1 mRNA and protein levels has been
described in these processes. Peptide 144 (P144)is a acetic salt of a 14mer peptide from
human TGF-beta1 type III receptor (betaglycan). P144 TGF-beta1-inhibitor has been
specifically designed to block the interaction between TGF-beta1 and TGF-beta1 type III
receptor, thus blocking its biological effects. P144 has shown significant antifibrotic
activity in mice receiving repeated sucutaneous injections of bleomycin, a widely accepted
animal model of human scleroderma, and could contribute to the development. The aim of the
study is to asses the long-term safety of topical application of P144 cream in the treatment
of skin fibrosis in patients with systemic sclerosis in an extension open-label treatment
period of 6 additional months.