CRA10 WHEN NOTHING WORKED- UNTIL STEROIDS DID: A CASE OF RAPIDLY PROGRESSIVE INTERSTITIAL LUNG DISEASE MIMICKING CARDIAC FAILURE IN ANTISYNTHETASE SYNDROME

Ad Rian Chong1, Khai Lip Ng1, Nur Husna Mohd Aminudin1, Kasuma Mohamed Nordin1
1 Division of Respiratory Medicine, Department of Internal Medicine, Melaka Hospital, Melaka, Malaysia

Introduction
Antisynthetase syndrome is a rare autoimmune condition that poses a significant diagnostic challenge due to its diverse and often subtle clinical manifestations.

Case Report
We report the case of a 53-year-old man with diabetes and hypothyroidism who presented with progressive dyspnea, orthopnea, and peripheral edema. Initial evaluation suggested acute heart failure, supported by elevated BNP levels and chest radiographic findings. Despite optimal heart failure therapy, including diuretics and vasodilators, his condition deteriorated rapidly, culminating in respiratory failure requiring mechanical ventilation and multiorgan dysfunction necessitating dialysis. Imaging showed extensive ground-glass opacities with septal thickening, initially interpreted as pulmonary edema with possible infection. Echocardiography revealed preserved left ventricular ejection fraction (64%), moderate tricuspid regurgitation, and elevated pulmonary artery pressure, without a definitive cardiac explanation. A respiratory consultation noted bilateral mechanic’s hands, prompting autoimmune workup. Anti-Jo-1 antibodies were detected, confirming the diagnosis of antisynthetase syndrome. High-dose corticosteroid therapy (methylprednisolone 1 mg/kg) was initiated, resulting in dramatic clinical improvement, including respiratory recovery and reversal of organ dysfunction. He was successfully extubated, weaned off oxygen, and discharged on tapering steroids and mycophenolate mofetil.

Discussion and Conclusion
This case underscores the importance of considering autoimmune etiologies such as antisynthetase syndrome in patients with unexplained respiratory failure and multiorgan involvement, particularly when they do not respond to standard treatments for heart failure or infection. Subtle clinical signs like mechanic’s hands, alongside serologic testing, can provide critical diagnostic clues. Early recognition and initiation of immunosuppressive therapy are essential to improve outcomes in this potentially life-threatening condition.