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ADULT ONSET STILL DISEASE

ADULT ONSET STILL DISEASE. Dr. Müge Bıçakçıgil Kalaycı. ADULT ONSET STILL DISEASE. Multi-system inflammatory disease begins with a sore throat may develop days to weeks before the typical quotidian fever Rash Joint pains. ETIOLOGY. no etiologic factor has been identified Infectious ??

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ADULT ONSET STILL DISEASE

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  1. ADULT ONSET STILL DISEASE Dr. Müge Bıçakçıgil Kalaycı

  2. ADULT ONSET STILL DISEASE • Multi-system inflammatory disease • begins with a sore throat • may develop days to weeks before • the typical quotidian fever • Rash • Joint pains

  3. ETIOLOGY • no etiologic factor has been identified • Infectious?? • prodromal sore throat • fever • Possible mechanism; - viral agent initiates a cascade of the immunological events resulting in the characteristic clinical syndrome of AOSD.

  4. Implicated organisms in AOSD

  5. Clinical Features

  6. Common Clinical Features *Fever of at least 39ºC lasting one week or longer *Arthralgias or arthritis lasting two weeks or longer *Characteristic rash which is a macular or maculopapular, nonpruritic, salmon-pink eruption usually apparent over the trunk or extremities during febrile episodes * Leukocytosis (10,000/µL or greater) with 80 percent or more granulocytes

  7. * Sore throat * Recent development of significant lymph node swelling * Hepatomegaly or splenomegaly *Abnormal liver function studies, particularly aminotransferases and lactate dehydrogenase *Negative tests for antinuclear antibody and rheumatoid factor

  8. FEVER • Quotidian or "double-quotidian" with a brief peak in the late afternoon or early evening. • Temperature swings can be dramatic, with changes of 4ºC occurring in four hours. • Approximately 20 percent of cases, fever persists between spikes. • Over 99 % of patients manifest with fever > 39 0 C

  9. FEVER • The febrile paroxysms are cyclic and tend to recur every 24 or sometimes every 12 hours. Characteristically very high in the evening, returning to normal by morning. • Paroxysms are heralded by shaking chills, followed by 2-4 hours of high fever (> 104°F), and ending with defervescence and drenching sweats

  10. RASH • Still's rash is seen in 86% of patients • Periodic appearance and location • Appears during febrile attacks and may last for several hours • It is typically salmon-colored (infrequently erythematous),maculopapular and may be confluent or show areas of central clearing. • Trunk, neck, extremity( extensor surface)

  11. RASH • Usually the face, palms, and soles are spared. • Dermatographism: is an exaggerated cutaneous urticarial response to cutaneous stimuli (ie, the scratch test). • Rash is typically nonpruritic.

  12. Articular Manifestations • Arthralgias dominate the early clinical picture • During the first 6 mos. the onset of polyarthritis is expected in > 90% of patients and may involve large and small articulations • Myalgias

  13. Affected joints: the knees, wrists, ankle, elbow, shoulder, PlPs, DlPs, TMJ and cervical spine. • Bony ankylosis of carpal, carpometacarpal. Intertarsal joints • Erosive and destructive polyarthritis, especially in those with a chronic polyarticular course

  14. Reticuloendothelial Disease • Splenomegaly • Very common early in the disease and reflects tissue infiltration with inflammatory cells and heightened immunologic activity within the reticuloendothelial system (RES). • Palpable or radiographic demonstration of splenomegaly is seen in 42% of individuals • Hepatomegly • 40% of patients are found to have hepatomegaly • 70% demonstrate abnormalities of hepatic enzymes at some time during their illness

  15. Lymphadenopathy • 65% of AOSD patients. • Generalized mild to moderate nodal enlargement of nontender lymph nodes located in the cervical, axillary, epitrochlear, or inguinal regions. • Mesenteric, para-aortic and hilarnodes may be discovered during diagnostic imaging

  16. SEROSITIS • Pleuritis(40%) • Pleural effusions are usually bilateral, seldom large enough to be symptomatic, and rarely produce pleural thickening. • Thoracentesis often yields bloody, exudative effusions with white blood cell counts ranging from 3-20 x 103/mm3 with a polymorphonuclear predominance.

  17. Pneumonitis • Pneumonitis is found in over 20% of patients • These individuals often appear septic with complaints of fever, dry cough, dyspnea and are found to have pulmonary infiltrates that are unresponsive to anti-infective therapy • Infiltrates tend to be bilateral more commonly than unilateral, alveolar or interstitial in pattern and responds well to anti-inflammatory therapy with steroids

  18. Laboratory Investigations

  19. Leukocytosis • Leukocytes counts generally range between 12,500-40,000 cells/mm3, with the highest recorded to be 69,000 • ESR • 90% of AOSD patients have an ESR > 50 mm/hr and 50% have and ESR > 90 mm/hr.

  20. Hyperferritinemia • It has been suggested that extreme elevations of the acute phase reactant, ferritin, may be of diagnostic value in assessing patients with AOSD • Hyperferritinemia with values between 4000­ ­ 30,000 mg/ml have often been reported in association with the onset and/or flare of disease activity • Levels as high as 250,000 mg/ml have been reported AOSD.

  21. Diagnosis

  22. Diagnosis • Still disease lacks serologic test or histopathology and thus, remains a clinical diagnosis of exclusion. • AOSD is now being considered earlier in the course of evaluation of patients with fever, dermatitis and arthritis. • Diagnostic steps should include a comprehensive, noninvasive workup, documentation of fever pattern

  23. Yamaguchi et al 1992 • AOSD ­ Total of > 5 criteria (including 2 major) • Major CriteriaMinor Criteria Fever > 39°C                       Sore throat Arthralgia > 2 wks.               LN or splenomegalyStill's rash                              Liver dysfunction Neutrophilicleukocytosis    Negative RF & ANA • specificities greater than 92%, the sensitivity of Yamaguchi (96%)

  24. Treatment

  25. Treatment • NSAIDS or Aspirin • Mild disease with no life- threatening visceral involvement • 20-25 % respond (good prognosis group with mild disease activity) • Aspirin or an NSAID should be continued for one to three months following disease remission.

  26. GLUCOCORTICOSTEROIDS • Patients with very high fever, • Joint involvement that is disabling • Potentially life-threatening visceral involvement (myocarditis) • Starting dose of 0.5 to 1.0 mg/kg per day PO

  27. Immunomodulating drugs • There are no controlled trials assessing the efficacy of any of the immunomodulating drugs in ASD • * Intramuscular gold salts • * Hydroxychloroquine, • * Azathioprine, • * Cyclophosphamide, • * Cyclosporine, • * Sulfasalazine, • *Methotraxate • * Intravenous immune globulin, • * Anti-TNF-alpha agents

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