Critical Care |

Stolen Identity: Adult Onset Still Disease (AOSD) Masquerading as Septic Shock FREE TO VIEW

Parth Rali, MD; Ahsan Iqbal, MD; Eric Bihler, MD; Marvin Balaan, MD
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Allegheny General Hospital, Pittsburgh, PA

Chest. 2014;146(4_MeetingAbstracts):270A. doi:10.1378/chest.1990678
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SESSION TITLE: Critical Care Cases I

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Monday, October 27, 2014 at 11:00 AM - 12:00 PM

INTRODUCTION: We report a rare case of AOSD in a 23 y/o F presenting with Multi Organ Dysfunction Syndrome (MODS).

CASE PRESENTATION: A 23 y/o woman was transferred from a local hospital to the MICU at our institution after she presented with respiratory failure and shock. She was seen at another facility 6 weeks prior with quotidian fever, chills, arthralgia, and diffuse salmon colored rash. She was admitted to the referring hospital and was treated for community acquired pneumonia. A chest CT scan showed diffuse lymphadenopathy. Results of an inguinal lymph node biopsy were pending at time of her transfer. On presentation, patient was intubated at OSH. Key findings on admission included a Hgb of 6.0 WBC count of 45,000, platelets of 800,000, ferritin of 46,000 and. metabolic acidosis with pH of 6.88, lactic acidosis of 20, pericardial effusion with tamponade physiology. She underwent immediate pericardiocentesis and was started on broad spectrum antibiotics, multiple pressors, and stress dose steroids. The patient improved dramatically within the next 48 hours. The inguinal lymphnode biopsy was reported as showing giant histiocytes. A diagnosis of AOSD was made based on leukocytosis, severe anemia, hyperferritinemia, seronegativity, pleuropericarditis, hepatomegaly, arthritis, rash, and giant histiocytes on biopsy.The patient was given tapering course of steroids and was discharged within next few days

DISCUSSION: AOSD is an inflammatory disorder characterized by daily fever, arthralgia, rash. Etiology is unknown but there is association with HLA 17,18,35 and DR2. Ferritin count >3000 in absence of infection or malignancy is classic finding. Diagnosis is made with Yamaguchi clinical criteria: Severe pleuro-pericarditis seen in 30-40% of cases. Reactive hemophagocytic syndrome with diffuse lymphadenopathy, hepato-splenomegaly, anemia , thrombocytosis, and leukocytosis mimicking lymphoma/leukemia is commonly seen. Reactive giant histiocytes on lymph node biopsy is a classic finding. Treatment is with steroids. Refractory cases can be treated with methotrexate and infliximab

CONCLUSIONS: Diagnostic bias can occur when a patient presents with a picture of septic shock. Other entities that mimic sepsis should be considered when the appropriate clinical clues are present

Reference #1: Efthimiou et al,Diagnosis and management of adult onset Still's disease.Ann Rheum Dis 2006; 65:564

Reference #2: Kontzias et al, Adult-onset Still's disease: pathogenesis,clinical manifestations and therapeutic advances.Drugs 2008;68:319

DISCLOSURE: The following authors have nothing to disclose: Parth Rali, Ahsan Iqbal, Eric Bihler, Marvin Balaan

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