The trajectory of pulmonary function tests in patients with systemic sclerosis

  • Hinchcliff, Monique E (PD/PI)

Project: Research project

Project Details


Because interstitial lung disease (ILD) is the leading cause of mortality in patients with systemic sclerosis (SSc), research to identify therapeutics that halt SSc-ILD progression and/or foster resolution of lung fibrosis is essential. Several studies show that immune modulatory treatment in patients with SSc improves FVC. The Scleroderma Lung Study I that xxx showed xxxx. The Scleroderma Lung Study II that xxx showed xxxx. Evidence also suggests that autologous stem cell transplant in patients with SSc improves FVC and reduces radiographic evidence of ILD on HRCT (Burt Lancet paper) (this sentence might be better in a discussion since the ASSIST trial compared ASCT to IV cytoxan not MMF). These studies appropriately recruited patients with ILD and FVC reductions; however, with the emergence of a growing body of immune suppressive medications that appear to safe in patients with SSc, the question of whether to implement immune suppressive treatment in patients with radiographic evidence of ILD with reserved pulmonary functions emerges. While results have shown favorable association between immune suppression and the trajectory of forced vital capacity%-predicted (FVC%) these studies have been small in sample size with specific inclusion/exclusion criteria.[1, 2] It remains to be determined if the findings from the aforementioned studies can be generalized to the SSc patient population, irrespective of baseline PFT, HRCT, or disease duration. The purpose of this cross-sectional study is to identify the trajectory of pulmonary function testing in patients with SSc (this is your Aim 1) and to determine the effect of immune suppression on PFT progression in SSc patients (this has already been done in SLS I and II?) you need to tell what we are doing that is different from those studies. We hypothesize that patients receiving immunosuppressive medications at baseline PFT will experience a more favorable PFT trajectory compared to patients not receiving these medications (this had been shown already). In addition, we hypothesize that initiation of immune suppression (ie for skin, joints) despite normal FVC% will also improve PFT trajectory (this is new). Subjects who fulfill the American College of Rheumatology (ACR) 2013 SSc criteria and have at least two PFT separated by> 12months will be studied recruited (this is a cross-sectional study so we are not recruiting anyone) from the Northwestern Scleroderma Program registry. We will determine the natural (will it be natural pattern in all patients? What about those that are treated?)pattern of PFT changes between baseline and 3 year follow up in this SSc cohort. We will determine the effect that immune suppression has on PFT trajectory adjusting for xxxxx. We will dichotomize the group into those patients taking immune suppression with normal versus reduced baseline FVC% and assess the PFT trajectory in order to (you need to say what you will learn). Results of these studies will provide insight into the natural?? history of SSc pulmonary disease. Moreover, our results will assess the utility of immune suppression in slowing lung function decline (this has been done by others) what are we doing that is different from SLS I and SLS II. Finally, this analysis will determine if early initiation of immune suppression prior to development of ILD is beneficial in halting disease onset and progression (yes!).
Effective start/end date10/1/173/29/19


  • Rheumatology Research Foundation (AGREEMENT 9/1/17)


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