Scleroderma Association of BC Research Project   -   First Quarter Report   -   August 2017


Enrolment of Scleroderma (SSc) patients with and without interstitial lung disease (ILD) to provide blood and skin tissue samples has closed ahead of schedule. This is an important milestone in the project. Another important milestone was the establishment of ``The Scleroderma Biobank” at St. Paul`s hospital in Vancouver ostensibly to store the collected samples and any derived products to be used in the course of this Project by Drs. James Dunne, Chris Ryerson, and Pearce Wilcox of The University of British Columbia and Dr. Kevin J. Keen of The University of Northern British Columbia.  A deliberate decision was taken in selecting the name of the Biobank to avoid a regional identification so to allow for growth of the storage of biological tissues for future use by any researchers using samples collected from anywhere in the globe.


Enrolment of patients with idiopathic pulmonary fibrosis (IPF) and normal controls is continuing with the time to completion of this task extended for another month until the end of September 2017.  IPF is the form of ILD when it occurs in patients unassociated as a secondary disorder due to another disease.  By comparing SSc-ILD tissues with IPF tissues essentially the IPF tissues are acting as a second control group in this scientific study. The delay in recruitment of the normal controls is due to a report received in June 2017 from an international review panel for the project that requested that normal controls additionally provide skin biopsies. With the weight of this opinion from an international group of peers, it was possible to revise the study design accordingly.  A skin biopsy on the forearm results in minor discomfort and a small scar with the risk of a treatable infection.  It was therefore necessary to call back normal controls who already had given blood samples.  In addition to extra time requested of voluntary controls for the procedure, additional clinician time and clinical space for the minor surgical procedure of a forearm skin biopsy had to be added to the initial study plan.  Understanding the flow of IPF patients through the Lung Clinic at St. Paul`s Hospital was another learning situation and it made sense to stretch out recruitment from among these patients.


The initially stated goal of the Project is to better understand the impact of known and novel micro ribonucleic acid (miRNA) sequences in regulating molecular pathways in IPF and SSc-ILD.  With the additional collection of skin tissue from normal controls added to the design of the study on the authority of the international review panel, the Project has been enhanced by the addition of a second goal:  to better understand the impact of known and miRNA sequences in regulating molecular pathways in skin damage in SSc.  Because the skin is not affected in IPF patients, it is not ethical to ask them to undergo a skin biopsy.  They have normal skin and the only source of normal skin for the Project need be from normal controls.  With Rodnan skin scores for the patients with the limited and diffuse forms of scleroderma as well as skin tissues from them and the controls, it will be possible to search for regulating impacts of miRNA sequences in skin and relate this to miRNA sequences in circulating blood components in a quest for a biomarker for skin damage detectable in a simple blood test.  The Project in its latest evolution can be restated as a hypothesis-generating study for the control of both lung damage in IPF and SSc-ILD patients and skin damage in SSc patients.