ABSTRACT Anti‐eukaryotic initiation factor 2B (anti‐eIF2B) is a rare systemic sclerosis (SSc)‐related autoantibody. Longitudinal treatment data for anti‐eIF2B‐positive SSc‐associated interstitial lung disease (ILD) remain limited. We herein describe the case of a 46‐year‐old man with limited cutaneous SSc who developed lower‐lobe–predominant fibrotic ILD. Major SSc autoantibodies were negative, and an antigen‐specific panel identified anti‐eIF2B. Mycophenolate mofetil was initiated; however, serial high‐resolution computed tomography showed incremental fibrotic progression, and the diffusing capacity declined, consistent with progressive SSc‐ILD, prompting the addition of nintedanib to the ongoing therapy. This case supports testing for minor specificities when SSc‐ILD is suspected despite negative results for major autoantibodies and adds new information on the post‐treatment disease course and therapeutic selection in this serological subset. Further case accumulation with standardised longitudinal outcomes is needed to define the prognosis and treatment response.
Ikeda et al. (Thu,) studied this question.