Autoantibody to scaffold attachment factor B (SAFB): A novel connective tissue disease-related autoantibody associated with interstitial lung disease

•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.

ObjectiveTo identify and characterize a novel connective tissue disease (CTD)-related autoantibody (autoAb) directed against scaffold attachment factor B (SAFB).MethodsAutoAb specificity was analyzed using RNA and protein-immunoprecipitation assays. Autoimmune targets were affinity purified using patients' sera and subjected to liquid chromatography mass spectrometry.ResultsBy immunoprecipitation assay, 10 sera reacted with a protein with a molecular weight of approximately 160 kDa. Liquid chromatography mass spectrometry of the partially purified autoantigen and additional immunoblot-based analyses revealed that the Ab specifically recognized SAFB. Anti-SAFB Abs were detected in 2 of 646 patients with systemic sclerosis (SSc) (0.3%), 1 of 1570 patients with polymyositis/dermatomyositis (0.06%), 4 of 270 patients with interstitial lung disease (ILD) (1.5%), 1 of 43 patients with overlap syndrome (2.3%) and 2 patients with other diseases including primary Raynaud's disease and eosinophilic pneumonia. Five patients with anti-SAFB Abs had Raynaud's phenomenon and 3 had nail fold punctate hemorrhage. Of note, 8 of the 10 patients (80%) suffered from ILD. None of the patients with anti-SAFB Abs had pulmonary arterial hypertension, heart disease, or renal involvement.ConclusionsAnti-SAFB Ab is a novel CTD-related autoAb possibly associated with ILD.