Successful treatment of rapid progressive interstitial lung disease in a case of anti‐Zo antibody positive anti‐synthetase syndrome

Abstract Background Anti‐synthetase syndrome (ASS) is a chronic multisystemic autoimmune disease characterized by detectable anti‐aminoacyl‐transfer‐RNA antibodies. Interstitial lung disease (ILD) in anti‐synthetase syndrome patients is often severe and rapidly progressive. Anti‐Zo (phenylalanyl) antibody is reported rarely in ASS. Therefore, the appropriate treatment of anti‐Zo positive ASS is unclear. Case presentation Here we present a case of anti‐Zo‐positive ASS with rapid progressive ILD (RP‐ILD) in a Chinese patient successfully treated with a combination of systemic corticosteroids and tacrolimus. Conclusion We reviewed 13 anti‐Zo‐positive ASS patients (including our case) and summarized clinical features that have some differences with other ASS. Anti‐Zo‐positive ASS is a rare autoimmune disease with a high burden of ILD, is often severe and rapidly progressive. Corticosteroids with tacrolimus may improve patient outcomes in anti‐Zo antibody positive ASS with RP‐ILD.