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Case Report: Acute interstitial pneumonia as the initial presentation of Anti-EJ antisynthetase syndrome: a case of severe respiratory failure with rapid response to cyclophosphamide
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DOI：10.3389/fimmu.2026.1877724.png)
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Abstract
En 中文
BackgroundAntisynthetase syndrome (ASyS) associated with anti-EJ antibodies (directed against glycyl-tRNA synthetase) is increasingly recognized as an ILD-dominant phenotype with a propensity for rapidly progressive ILD (RP-ILD). Acute interstitial pneumonia (AIP)-like presentations may mimic severe infection; posing significant diagnostic challenges.Case presentationA 55-year-old man with hypertension and a 20-pack-year smoking history presented with a fever (39°C) and rapidly progressive dyspnea. Laboratory findings revealed markedly elevated inflammatory markers. CT pulmonary angiogram demonstrated diffuse bilateral ground-glass opacities; consolidations; and interlobular septal thickening consistent with AIP-pattern. Despite broad-spectrum antibiotics; the patient developed refractory respiratory failure requiring mechanical ventilation; hemodynamic instability requiring vasopressors; and acute kidney injury. Bronchoscopy with transbronchial biopsy confirmed acute interstitial inflammation. After more than one week in the intensive care unit (ICU) without improvement; rheumatologic evaluation revealed positive for anti-EJ and anti-Th/To antibodies; supporting ASyS. High-dose of immunosuppressive drug were initiated; resulting in marked improvement in oxygenation; hemodynamics; and muscle strength within 48 hours. Follow-up CT at three weeks demonstrated significant regression of ground-glass opacities with residual findings consistent with nonspecific interstitial pneumonia/usual interstitial pneumonia (NSIP/UIP) overlap.ConclusionASyS should be considered in patients with severe pneumonia-like illness who fail to respond to appropriate antibiotic therapy. Anti-EJ antibodies are associated with AIP-like patterns and fulminant respiratory failure. Early serologic testing and prompt immunosuppression can be life-saving. The recently published 2024 Classification Criteria for Anti-Synthetase Syndrome (CLASS) provide a contemporary framework for the classification of ASyS; particularly in ILD-dominant presentations.
Keywords:
cyclophosphamide
rapidly progressive interstitial lung disease
antisynthetase syndrome
acute interstitial pneumonia
Anti-EJ antibodies
glycyl-tRNA synthetase
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