Systemic sclerosis (SSc) is a rare connective tissue and autoimmune disease associated with inflammation of the skin and internal organs. Interstitial lung disease (ILD), a frequent complication of SSc with highly variable patient course, is associated with increased risk of morbidity and mortality. Mechanistic, mathematical modeling approaches have been applied to a similar disease area, idiopathic pulmonary fibrosis (IPF), to support the development of new drug treatments by improving understanding of disease pathophysiology, identifying mechanistic drivers of disease, interpreting clinical treatment results, and assessing efficacy for novel treatments as monotherapies or in combination with existing treatments.

ILDsym, a quantitative systems pharmacology (QSP) model of SSc-ILD, was built by leveraging the design framework from IPFsym. New sub-models were developed and components updated where appropriate to represent SSc-ILD. Aspects of this development process and simulated patient generation will be discussed, along with how standard of care treatments have been represented in the model which set the stage for novel treatment assessment.

Presented by Principal Scientist Zackary Kenz.