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This case highlights the effective management of anti-PL-12-associated anti-synthetase syndrome presenting as rapidly progressive interstitial lung disease.

May 20, 2026

C32-13 Anti-pl-12 Antibody-associated Anti-synthetase Syndrome With Rapidly Progressive Interstitial Lung Disease Successfully Treated With Immunosuppression

Key Points

This case highlights the effective management of anti-PL-12-associated anti-synthetase syndrome presenting as rapidly progressive interstitial lung disease.
Patient presented with respiratory failure and was diagnosed with anti-synthetase syndrome via serologic testing.
Received multi-agent immunosuppressive therapy including corticosteroids, plasma exchange, IVIG, and rituximab.
Follow-up involved imaging and clinical evaluations to assess recovery.
Marked improvement in oxygenation and significant resolution of lung opacities observed on follow-up CT images.
Pulmonary pressures normalized post-treatment, demonstrating the effectiveness of the therapy.
Patient was discharged to rehabilitation and no longer required supplemental oxygen by the 4-week follow-up.

Abstract

Abstract Introduction Anti-synthetase syndrome (AS) is a rare subset of idiopathic inflammatory myopathies characterized by antibodies directed against aminoacyl-transfer RNA synthetases. Clinical manifestations include interstitial lung disease (ILD), myositis, arthritis, Raynaud’s phenomenon, and characteristic cutaneous features. Among anti-synthetase antibodies, anti-PL-12 is uncommon (3%) and is frequently associated with amyopathic or hypomyopathic presentations and rapidly progressive ILD (RP-ILD). We present a case of anti-PL-12-associated RP-ILD with profound respiratory failure that responded to aggressive immunosuppressive therapy. Case Presentation A 67-year-old man with a history of Campylobacter-associated Guillain-Barré syndrome presented with subacute cough and dyspnea. Chest CT demonstrated diffuse bilateral ground-glass opacities and consolidation. He developed acute hypoxemic respiratory failure requiring intubation and mechanical ventilation. He was transferred to our tertiary center for consideration of extracorporeal membrane oxygenation (ECMO) and lung transplant evaluation. Comprehensive infectious testing, including respiratory cultures, was negative. Echocardiography revealed preserved left ventricular systolic function with elevated pulmonary artery pressures. Serologic testing demonstrated a high-titer anti-PL-12 antibody (137 SI; normal 11 SI), confirming the diagnosis of anti-synthetase syndrome-associated RP-ILD with a hypomyopathic phenotype. He received pulse-dose corticosteroids, plasma exchange, intravenous immunoglobulin (IVIG), and rituximab. Over several weeks, his oxygenation improved, and he was successfully extubated to high-flow nasal cannula. Follow-up CT imaging showed marked resolution of parenchymal abnormalities (Figure 1), and pulmonary pressures normalized on repeat echocardiogram. He was discharged to an acute rehabilitation facility on 5 L/min oxygen via nasal cannula and was off supplemental oxygen by his 4-week follow-up visit. Discussion This case illustrates the rare presentation of anti-PL-12-associated anti-synthetase syndrome manifesting as rapidly progressive ILD without overt myositis. The patient’s dramatic clinical and radiographic recovery following multi-agent immunosuppression underscores the importance of early recognition and aggressive therapy. Although RP-ILD in anti-PL-12-positive patients are often refractory to treatment and associated with high mortality, this case demonstrates that a combination of corticosteroids, plasma exchange, IVIG, and rituximab can induce substantial remission even in severe disease. Conclusion Anti-PL-12-associated anti-synthetase syndrome should be considered in patients presenting with rapidly progressive ILD of unclear etiology, particularly when infectious and other autoimmune causes are excluded. Early, aggressive, multimodal immunosuppression can lead to meaningful recovery and reversal of lung injury. Figure 1. Representative axial chest CT images before and after immunosuppressive therapy, showing marked improvement in bilateral ground-glass opacities. Reference 1. Elferjani B, Liaqat A, Zaman M, Sexton M. Anti-synthetase syndrome-related interstitial lung disease with anti-PL-12 antibodies. Cureus. 2021;13(2):e12936. doi:10.7759/cureus.12936 This abstract is funded by: None

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