Northern California Scleroderma Research Consortium

Lorinda Chung, MD, MS
Stanford University

Paul Wolters, MD
University of California, San Francisco

Project Summary:

The Northern California Scleroderma Research Consortium (NCSRC) comprises a group of investigators at UCSF and Stanford who collaborate on efforts to understand the clinical characteristics and molecular mediators of patients with scleroderma. A core feature of this collaboration has been the creation of a longitudinal registry of patients and a database of detailed clinical information linked to biological samples obtained from scleroderma patients. This registry is being used to advance the understanding of scleroderma by investigating pathobiological and clinical endpoints in scleroderma patients. Members of the consortium use the registry, database and associated biospecimens for joint or independent research projects.

This summary focuses on the work in Dr. Paul Wolters’ group. Our research has focused on understanding the pathobiology of scleroderma-associated interstitial lung disease (SSc-ILD) and whether SSc-ILD shares clinical characteristics or underlying biological mechanisms with idiopathic pulmonary fibrosis (IPF), a genetically mediated, age-associated, interstitial lung disease. By comparing these diseases, we developed a model (SADL model) that can be used to predict survival in patients with SSc-ILD. The SADL model predicts 1, 2 and 3-year risk for mortality from SSc-ILD and was recently published (Morisset et al. Chest 2017).

In collaboration with Dr. Richard Locksley at UCSF, we recently reported (Van Dyken et al. Cell 2017) that the accumulation of environmental chitin (a polysaccharide found in the cell walls of fungi or arthropods) in the lungs of mice or humans can lead to lung fibrosis. Interestingly, we found that chitin accumulates in the lungs of patients with either SSc-ILD or IPF. We are now in the process of trying to understand whether this accumulation contributes to, or is the consequence of lung fibrosis in patients.

In addition, we have been comparing serum levels of circulating molecular markers that predict survival in patients with IPF in patients with SSc-ILD, in order to determine whether there is a subset of patients with SSc-ILD whose disease results from biological mechanisms similar to those seen in IPF. We have found several IPF biomarkers to be elevated in SSc-ILD patients. We are now in process of determining whether they predict outcomes in SSc-ILD patients.

How This Work Will Impact Patients:

The SADL model can be used to advise patients with SSc-ILD on their individual risk for progression, and possibly identify those at highest risk for progression and who may benefit from lung transplantation.

Dr. Wolters on Why Research Matters:

The only way to advance knowledge and improve patient care is through research. The SRF is the most important philanthropic organization dedicated to developing novel treatments for patients with scleroderma.”

Dr. Chung on the SRF and Collaboration:

The SRF has been instrumental in connecting our group with other top scleroderma researchers in the country. The SRF facilitated communication between researchers and introduced Stanford to investigators and industry sponsors with similar interests but different research skill sets in order to build the strongest research teams possible. Specifically, the SRF supported the development and growth of our collaborative research group with UCSF, the Northern California Scleroderma Research Consortium. Multiple promising studies have arisen from our collaboration with UCSF, and the group has grown to triple its initial size over the past few years.

 

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