Improving Outcomes for Patients with Scleroderma-related Lung Fibrosis

Monica Sanborn, Dr. Dinesh Khanna & Erica Bush
L to R: Monica Sanborn, RN, BSN; Dinesh Khanna, MD, MSc; Erica Bush, BS, CCRA

Monica Sanborn, RN, BSN, a Clinical Research Coordinator in the Division of Rheumatology, was recently recognized by several faculty members for her work on the Scleroderma Lung Study III. Dr. Dinesh Khanna, Director of the Scleroderma Program, stated “Monica was highlighted as the best coordinator for the study. Everyone is very impressed with her communication skills, prompt data entry, and overall conduct of the trial. We are fortunate to have her and are thankful for her contributions and helping us achieve the tripartite mission of U-M!”

Monica focuses her work on the study of Scleroderma and is involved with several clinical studies at Michigan Medicine. Below, she discusses the current Scleroderma Lung Study III, what they hope to achieve, and why it’s important.

What is the Scleroderma Lung Study III?

The Scleroderma Lung Study III (SLS III) continues the work we did on the Scleroderma Lung Study II (SLS II). It is a phase II study combining the anti-fibrotic effects of Pirfenidone (PFD) with Mycophenolate Mofetil (MMF) for treating Scleroderma-related Interstitial Lung Disease (ILD).

The primary hypothesis is that the rapid onset and anti-fibrotic effects of PFD, which have been observed in the treatment of Idiopathic Pulmonary Fibrosis (IPF), will complement the delayed anti-inflammatory and immunosuppressive effects of MMF, to produce a significantly more rapid and/or greater improvement in lung function over time than occurs in patients receiving control therapy with MMF and Placebo.

Who is involved?

The study was designed by principal investigator Michael Roth, MD, from the University of California, Los Angeles (UCLA) and co-principal investigator Dinesh Khanna, MD, MSc, from the University of Michigan (U-M). The study includes 17 centers in the US and we are currently expanding to include Canadian centers.

What do you hope to achieve?

The SLS III study builds upon 20 years of experience by the Scleroderma Lung Study investigators and is focused on improving clinical outcomes for patients with scleroderma-related lung fibrosis. While the preceding study, SLS II, demonstrated that around 75 percent of patients experience stable or improving pulmonary function when treated with MMF, overall improvements in the forced vital capacity were modest. Researchers hope that combining MMF with the anti-fibrotic drug, PFD, will show additional and clinically meaningful benefits.

What is Pirfenidone?

Pirfenidone is among a new class of drugs that have demonstrated efficacy in treating IPF. It works via a different mechanism than Mycophenolate Mofetil. Anti-fibrotic agents have been shown to slow lung deterioration, improve pulmonary function, and decrease the risk of progressive disease and death when used in the treatment of Idiopathic Pulmonary Fibrosis. In addition, Pirfenidone is known to have a rapid effect in Idiopathic Pulmonary Fibrosis patients, while Mycophenolate Mofetil has typically taken 3 to 6 months to improve pulmonary function in Scleroderma-related ILD.

Why is this study important?

The inflammation and scarring that targets the lungs of up to 70% of patients with scleroderma results in debilitating symptoms and is the leading cause of morbidity and mortality. SLS III investigators hope to demonstrate that the combined treatment will be well tolerated, produce a faster and more potent therapeutic effect, and that a higher percentage of scleroderma patients will experience meaningful improvements with respect to lung function, CT scan findings, skin changes, and quality of life measures.

Learn more about the Scleroderma Lung Study III and the previous Scleroderma Lung Studies.

Learn more about the University of Michigan Scleroderma Program.