# Anti‐Eukaryotic Initiation Factor 2B‐Positive Systemic Sclerosis‐Associated Interstitial Lung Disease With Progressive Fibrosis: A Case Report

## TL;DR

A rare case of systemic sclerosis with lung disease linked to anti-eIF2B antibodies is reported, showing treatment challenges and progression despite therapy.

## Contribution

This case adds new insights into treatment strategies and disease progression for anti-eIF2B-positive systemic sclerosis-associated interstitial lung disease.

## Key findings

- The patient showed progressive fibrotic lung disease despite initial treatment with mycophenolate mofetil.
- Adding nintedanib to the treatment regimen was considered due to worsening lung function.
- Testing for minor autoantibodies like anti-eIF2B is important when major SSc autoantibodies are negative.

## 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.

We report the case of a patient with anti‐eIF2B‐positive SSc‐ILD managed with stepwise mycophenolate titration and subsequent nintedanib addition, accompanied by serial biomarkers and clinical follow‐up to help delineate the therapeutic course within this rare serological phenotype.