Autoantibody (autoAb) against scaffold attachment factor B (SAFB) is a novel connective tissue disease-related autoAb. Anti-SAFB Ab is detected in a small number of patients with systemic sclerosis and/or idiopathic inflammatory myopathy. Interstitial lung disease was observed in patients with anti-SAFB Ab as a relatively common manifestation. Patients with anti-SAFB Ab have Raynaud’s phenomenon and nail fold punctate hemorrhage at a constant rate.