B cells and systemic sclerosis interstitial lung disease

Interstitial lung disease is an important complication of systemic sclerosis (SSc-ILD) with high mortality and morbidity. Recent clinical studies in SSc-ILD have led to FDA-approved therapies in SSc-ILD. Importantly, evidence from these studies has been extrapolated to guide management of interstitial lung diseases 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 centre 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 also risks associated with treatment.