Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of excessive inflammation and tissue destruction secondary to abnormal immune activation. The term macrophage activation syndrome (MAS) is used when HLH develops in the setting of systemic juvenile idiopathic arthritis (SJIA; formerly known as Still's disease), adult-onset Still's disease, or any other rheumatologic disorder. We present a case of a 21-year-old female with a known history of SJIA who presented to the hospital with fever, chills, myalgia, nausea, vomiting, and hypotension. Initial evaluation at the time of presentation suggested sepsis likely due to acute pyelonephritis, and the patient was started on antibiotics and intravenous fluid hydration. However, further workup suggested that her symptoms were non-infectious and were likely due to MAS, a rare complication of SJIA. We promptly diagnosed her, and she received a course of steroids and made an uneventful recovery.
We report a 58-year-old female with severe throat pain, difficulty swallowing, choking on solid meals, coughing, and hoarseness. CT angiography of the chest revealed vascular compression of the esophagus by an aberrant right subclavian artery (ARSA). The patient underwent thoracic endovascular aortic repair (TEVAR) and revascularization to address the ARSA. The patient experienced significant improvement in her symptoms following the surgical intervention.Dysphagia lusoria is a rare condition involving compression of the esophagus and airway by an ARSA. While medical management is the first line of treatment for mild symptoms, surgical intervention is often necessary for severe cases or those unresponsive to conservative management. TEVAR with revascularization is a feasible and minimally invasive option for treating symptomatic non-aneurysmal ARSA, potentially resulting in favorable outcomes.
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