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A3055 - A Catastrophic Presentation of Antisynthetase Syndrome with Rapidly Progressing Interstitial Lung Disease and Its Antibodies
Author Block: A. Arthur, A. Alaverdian; Pulmonary Critical Care, Nassau University Medical Center, East Meadow, NY, United States.
Antisynthetase Syndrome (AS) is an autoimmune disorder and an idiopathic inflammatory myositis, often presenting with severe and rapidly progressing interstitial lung disease (ILD). These patients can present with or without myopathy and are often intolerant to corticosteroids alone. Diagnosis is made by presence of antisynthetase antibodies (ARS), plus (one or more): ILD, arthritis, fever, Raynaud’s phenomenon, or mechanic’s hands. Different ARS have been associated with different presentations and outcomes.
A 29-year-old African American male with no past medical history, presented with worsening dyspnea for 5 weeks, previously treated as pneumonia. History was notable for dry cough, night sweats, persistent acid reflux and bilateral hand and wrist pain for last 6 months. There was no exposure history. He was hypoxic on room air and velcro crackles were auscultated. CT chest was as shown with associated axillary and mediastinal lymphadenopathy.
During hospitalization, the patient had rapid clinical deterioration, increasing oxygen requirements and persistent fevers despite antibiotics. Anti-Jo antibody and elevated aldolase were detected; CK 1726 and EMG confirmed a myopathic process. Labs and cultures were negative, including HIV and TB. Transbronchial biopsies showed non-specific interstitial fibrosis. Additional antibodies sent included Anti-SRP, Anti-Mi-2, Anti-MDA-5, Anti-PL 7, 12, anti-OJ and anti-Jo-1. The patient was started on pulse dose steroids while continuing to deteriorate, requiring high-flow oxygen. On day 9, cyclophosphamide was initiated and he received IVIG. Day 13, he required endotracheal intubation. Patient expired on day 14 on extracorporeal membrane oxygenation pending transfer to lung transplant center. Anti-Jo-1 ARS was later reported as positive.
Anti-Jo-1 is the most common ARS, often associated with concurrent muscle myalgias and weakness (though often undetectable in AS), especially when compared to anti-PL7/12. Patients with positive PL7/12, anti-KS and anti-OJ tend to have more severe and rapidly progressing ILD, often preceding any other symptomatology. Anti-Jo-1 portends a better prognosis, with a five-year survival at 90%, with poorer outcomes associated with the less common ARS. Currently, no standardized approach exists in the management of antisynthetase syndrome: it is guided by case reports and series. Any patient with antisynthetase syndrome and a rapidly progressing ILD despite therapy needs considered for a lung transplantation. Even though patients with anti-Jo-1 are reported to have a better prognosis, this case demonstrates that vigilance should be practiced for possible early lung transplant referral in a rapidly progressing ILD.