Persisting CD19.CAR-T cells in combination with nintedanib: clinical response in a patient with systemic sclerosis-associated pulmonary fibrosis after 2 years
Wolfgang Merkt, Manuel Röhrich, Eleni Mavriopoulou, Ayla Nadja Stütz, Jörg H. W. Distler, Anita Schmitt, Markus Polke, Claus Peter Heußel, Michael Schmitt, Hanns‐Martin Lorenz
Abstract
Interstitial lung disease (ILD) associated with progressive pulmonary fibrosis is the leading cause of mortality in systemic sclerosis (SSc).1 Standard therapy of SSc-ILD at best decelerates progression.2–4 Treatments that profoundly improve SSc-ILD do not exist.5 Important components of SSc-ILD pathophysiology are fibroblast-driven fibrosis and B cell-driven autoimmunity with evidence of SSc-specific autoantibodies such as anti-Scl70. Therapeutic B cell-depletion using CD19-targeting chimeric antigen receptor (CD19.CAR)-bearing autologous T cells has the potential to change the therapeutic landscape in systemic autoimmune diseases.