A Case of Antisynthetase Syndrome Causing Critical Digital Ischemia

Antisynthetase syndrome (ASS) is a heterogeneous group of systemic autoimmune diseases caused by autoantibodies against aminoacyl-transfer RNA synthetases. Although the hallmark characteristics of ASS include Raynaud phenomenon, critical digital ischemia and digital ulcers resembling systemic sclerosis are rarely reported in this condition. We hereby report an anti-Jo-1 positive ASS patient syndrome that demonstrated the typical clinical features of myositis, interstitial lung disease, “mechanic’s hand”, Raynaud phenomenon, intermittent fever and inflammatory arthralgia. She was initially treated with methylprednisolone and mycophenolate mofetil. During the follow up, she presented with critical digital ischemia causing severe cyanosis and digital ulcers. Upper extremity Doppler ultrasound was normal. Patient was hospitalized and intravenous iloprost infusion and acetylsalicylic acid were initiated. Unfortunately, patient developed a respiratory failure, she was intubated and was admitted to intensive care unit, where she passed away, before the efficacy of iloprost treatment could be assessed. This article also discusses the very limited amount of literature about ASS patients with severe digital ischemia.