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A6756 - A Rare Disease Associated with Acute Respiratory Failure in an ICU Patient
Author Block: R. Westman; Pulmonary and Critical Care, The Ohio State University, Columbus, OH, United States.
Introduction: Kikuchi Disease is a rare, idiopathic disease that presents with constitutional symptoms and lymphadenopathy. Diagnosis is confirmed with lymph node biopsy showing necrotizing lymphadenitis. The disease has rarely been associated with acute hypoxemic respiratory failure. In this case, we describe a young female who develops multi-organ dysfunction with progressive respiratory failure who is ultimately diagnosed with Kikuchi disease. Case: A 23 year old, previously healthy female, presented to the emergency department with three weeks of fevers, night sweats, weight loss and a rash. In the emergency department, she was found to be febrile to 103°F, tachycardic to 102 with the remainder of her vital signs normal. Her exam was notable for a thin, ill appearing female with a macular rash of her chest and face. Cardiopulmonary exam was normal. Splenomegaly and cervical lymphadenopathy were noted. Her labs were significant for WBC 1.9, Platelets 131, ALT 202, AST 357, LDH 1095, Ferritin 8999. She was admitted for further work up. On hospital day three, she became progressively tachycardic, tachypneic and hypoxemic requiring supplemental oxygen at 15 L/min via nasal cannula. She was transferred to the Medical ICU. Chest x-ray showed bilateral infiltrates and effusions. The patient was started on broad spectrum antibiotics. Her infectious and autoimmune work up was unremarkable. Bone marrow biopsy was non diagnostic. CT scan was performed of her neck, chest, abdomen and pelvis. Her imaging revealed cervical and axillary lymphadenopathy. The patient underwent excisional lymph node biopsy of an enlarged axillary lymph node. Pathology was compatible with Kikuchi disease, also known as histiocytic necrotizing lymphadenitis. The patient was started on steroids and her condition rapidly improved. She was discharged home from the hospital several days later. Discussion: Kikuchi Disease is a rare, benign disease that is usually self-limiting. The cause of Kikuchi disease remains unknown but is thought to be an immune response of T-cells and histiocytes to an infectious agent. It has rarely been associated with significant hypoxemia as in this case. Previous reports of hypoxemia related to Kikuchi disease have been attributed to lymphatic obstruction in the chest. In patients presenting with constitutional symptoms and lymphadenopathy, Kikuchi disease should be maintained in the differential diagnosis as it is often overlooked and can lead to progressive organ dysfunction. There is no recommended treatment for Kikuchi Disease, but there is anecdotal evidence of success with corticosteroids and IVIG in patients who are severely symptomatic.