Adult-onset Still's Disease with a Rare Life-Threatening Complication of Acute Pulmonary Embolism: A Case Report and Literature Review

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disorder with unknown etiology that most commonly affects young female adults. The characteristic signs and symptoms of AOSD are high-spiking fever, evanescence salmon-pink rash and arthritis. Atypical cutaneous manifestations of AOSD can present with persistent pruritic papules and plaques, flagellate erythema, linear plaques or vesicular eruptions. Two serious complications which can occur in patients with AOSD are reactive hemophagocytic syndrome and thrombotic thrombocytopenic purpura. Pulmonary embolism is one of the cardiovascular emergencies which are rarely reported as a complication of AOSD. Here in, we report the case of a 35-year-old Burmese woman presented with subacute intermittent high-grade fever with sore throat, polyarthritis, and persistent pruritic rashes with flagellate erythema for 3 weeks. She fulfilled the criteria for the diagnosis of AOSD. During the admission, she developed a sudden onset of dyspnea subsequent investigation revealed an intraluminal filling defect at the lateral and posterior right lower lobes that was most compatible with pulmonary embolism. She was treated with oral naproxen, prednisolone 30 mg/day (0.6 mg/kg/day), cyclosporin A 50 mg/day, topical betamethasone cream 0.1% for AOSD, and received enoxaparin 0.6 mg SC twice daily for 1 week, then oral warfarin 2 mg/day for pulmonary embolism. After 4 weeks of switching treatments, her clinical condition was partially improved.