Treatment in Antisynthetase Syndrome-Associated Interstitial Lung Disease

Purpose of review Describe the treatment of antisynthetase syndrome-associated interstitial lung disease (AS-ILD). Recent findings The pathogenesis of AS-ILD involves the abnormal activation of the innate and adaptive immune systems, including activation of NK cells, dendritic cells, T-cells, and B-cells. Immunosuppressive agents targeting these pathways can treat AS-ILD patients; however, there are no randomized clinical trials evaluating the effect of these medications. Current recommendations are based on retrospective studies with therapies that have been shown to stabilize the lung function of other autoimmune-related ILDs. The selection of the agent is based on expert opinion and varies according to center preference and patient profile. Glucocorticoids are the first-line therapy in patients with active AS-ILD disease, typically, in combination with azathioprine or mofetil mycophenolate. Calcineurin inhibitors represent an alternative agent as second-line therapy. Cyclophosphamide, rituximab, and human immunoglobulins may be used for resistant and severe disease, but their role as first-line therapy is unknown. Non-pharmacologic therapies include pulmonary rehabilitation and oxygen supplementation is also frequently used along with the aforementioned medications. Treatment recommendations for AS-ILD are mainly based on retrospective studies of immunosuppressive drugs that have shown benefit in the stabilization of lung function. Further studies, ideally multicentered randomized clinical trials, are required to better guide the treatment in AS-ILD.