Case‐Based Immunology: B Cells and Systemic Sclerosis Interstitial Lung Disease

This is the authors' abstract. We don't add key points for this paper.

Interstitial lung disease (ILD) is an important complication of systemic sclerosis (SSc), with high mortality and morbidity. Recent clinical studies in SSc-ILD have led to US Food and Drug Administration-approved therapies in SSc-ILD. Importantly, evidence from these studies has been extrapolated to guide management of ILDs of other systemic autoimmune rheumatic diseases. Pathogenesis of SSc-ILD involves interplay between fibroblasts and the innate and adaptive immune system. A central role for the B cell compartment is supported by clinical and translational studies. We use a case from our center as a basis to discuss the pathogenesis of SSc-ILD, autoantibodies in SSc-ILD, and the role of B cells in the disease. We go on to consider treatment options for the case, the decision-making algorithm for treatment, and risks associated with treatment.