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Fever of Unknown Origin and Adult Onset Still s Disease AOSD

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Fever of Unknown Origin and Adult Onset Still s Disease AOSD

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    1. Fever of Unknown Origin and Adult Onset Still’s Disease (AOSD) AM Report Eric Edwards, M.D. September 4, 2007

    3. Fever of Unknown Origin: Definition NOT febrile illness without initially obvious etiology “Classical” definition (Petersdorf and Beeson, 1961): Fever > 38.3 on several occasions Duration>3 weeks Failure to reach a diagnosis after one week of inpatient investigation*

    4. Patient Subtypes Classical Nosocomial (Hospitalized>24h, no fever PTA) C. Difficile, PE, drugs Immune Deficient (ANC<500) Bacteremia, Fungal, HSV HIV M. Avium, PCP, CMV, lymphoma, Kaposi’s, drugs

    5. Differential Diagnosis Infections Malignancies Autoimmune Disease Miscellaneous Drugs Hepatitis DVT

    8. Causes of FUO (in India) Infectious 53% #1: TB (45%) Neoplasm: 17% #1: NHL (47%) Collagen Vasc.: 11% #1 SLE: 45% Miscellaneous: 5% Undiagnosed: 14%

    9. FUO by the Decades

    10. Minimal Diagnostic Criteria H+P CBC & Diff Blood Cultures x 3 Chem10 LFTs U/A and Microscopy Urine culture Chest X-ray Hepatitis serologies (if abnormal LFTs)

    11. Other Basic Tests ESR/CRP Peripheral Smear ANA Rheumatoid Factor HIV CMV IgM Mono Spot PPD

    12. Imaging Abdominal CT Chest CT Nuclear Imaging Lower Extremity Dopplers TTE/TEE

    13. Invasive Procedures Lumbar Puncture Liver Biopsy Temporal Artery Biopsy Bone Marrow Biopsy Lymph Node Biopsy Surgical Exploration of the Abdomen

    15. Adult Onset Still’s Disease Epidemiology Rare (0.16/100000) ~60% female Affects all ages Pathogenesis Poorly understood Genetic component? Infectious trigger? Characteristics Daily, high spiking fevers (85-100%) Arthritis (68-94%) Evanescent rash (51-87%) No specific diagnostic study Diagnosis is based on the presentation of characteristic features and the exclusion of similar conditions

    17. Diagnostic Criteria (Yamaguchi) Major Fever>39°, lasting >1 week Arthralgias or arthritis lasting >2 weeks Typical rash WBC>10,000 with >80% PMNs Minor Sore throat Lymphadenopathy and/or splenomegaly Abnormal LFTs Negative ANA and RF Exclusions Infections Malignancy Rheumatic Disease

    18. AOSD and Ferritin Ferritin is an acute phase reactant 80% have >5x elevation in ferritin Non-specific Low Glycosylated ferritin (GF) is more specific GF<20% + Ferritin >5x nl=93% specific Only 43% sensitive

    19. Treatment NSAIDs Monotherapy is effective in only ~10% Steroids 75% will respond favorably Methotrexate TNF blocking agents Etanercept Infliximab

    20. Prognosis Three distinct patters (~1:1:1) Self limited Most patients achieve remission within one year Intermittent Recurrent flares with complete remission between Flares may be years apart Recurrences tend to be milder than initial episode Chronic Articular manifestations can be severe 2/3 may need at least one total joint replacement

    21. References 1. Roth AR et al. Approach to the Patient with Fever of Unknown Origin. Am Fam Physician. 2003 Dec 1;68(11):2223-28. 2. Mourad O et al. A Comprehensive Evidence Based Approach to Fever of Unknown Origin. Arch Int Med. 2003 Mar 10;163:545-51. 3. Bor, DH. Approach to the Adult with Fever of Unknown Origin. www.utdol.com. 4. Kejariwal D et al. Pyrexia of Unknown Origin: A Prospective Study of 100 Cases. J Postgrad Med. 2002 Apr-Jun;48(2):155-6. 5. Efthimiou P et al. Diagnosis and Management of Adult Onset Still’s Disease. Ann Rheum Dis. 2006 May;65:564-72. 6. Uppal SS et al. Ten Years of Clinical Experience with Adult Onset Still’s Disease: Is the Outcome Improving? Clin Rheumatol. 2007 Jul;26(7):1055-60.

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