Anti-synthetase Antibody Syndrome with Rapidly Progressive Interstitial Lung Disease and Neuropsychiatric Manifestations

A BSTRACT A 68-year-old female presented with rapidly progressive dyspnea and neuropsychiatric symptoms. Investigations revealed bilateral ground-glass opacities on chest high-resolution computed tomography and electroencephalogram abnormalities suggestive of encephalopathy. Serological tests, extensive workup for alternative diagnoses, and exclusion of malignancy led to a diagnosis of anti-synthetase syndrome (ASS) with interstitial lung disease (ILD) and rare neuropsychiatric involvement. Treatment with high-dose corticosteroids and rituximab resulted in improvement of respiratory function and partial resolution of cognitive symptoms. This case highlights the importance of considering ASS in patients with rapidly progressive ILD, even when presenting with atypical features such as neuropsychiatric symptoms.