Adult onset Still's disease (AOSD) is a rare inflammatory condition characterized by high spiking fevers, evanescent rash, and joint inflammation. Its cause is unknown but may be triggered by viral or bacterial infections. Diagnosis is based on clinical features including quotidian fevers over 39C, arthritis for 2+ weeks, salmon-pink rash, and exclusion of other conditions. Treatment involves NSAIDs, glucocorticoids, or immunomodulators depending on severity.

1. 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
-Rubella
-Ebstein Barr
-Staphylococcus
-Parvovirus
-Yersinia enterocolitica
Brucela abortus
-Mycoplasma
-Borelia burgdoferi
-Cytomegalovirus
-Mumps
-Parainfluenza

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

11.  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)
RASH

12. 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.

16. 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

17.  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

19. 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

20. 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 hilar nodes may be discovered
during diagnostic imaging

21.  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.

22.  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

23. Laboratory
Investigations

24. Absence of antinuclear antibodies
Absence of rheumatoid factor,
Elevated ESR and C-reactive protein
Neutrophilic leukocytosis
Elevated serum amyloid A
Thrombocytosis
Elevated serum ferritin and
glycosylated ferritin
Elevations the hepatic enzymes
Hypoalbuminemia

25.  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.

26.  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.

27. Diagnosis

28.  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

29.  Yamaguchi et al 1992
 AOSD Total of > 5 criteria (including 2 major)
 Major Criteria Minor Criteria
Fever > 39°C Sore throat
Arthralgia > 2 wks. LN or splenomegaly
Still's rash Liver dysfunction
Neutrophilic leukocytosis Negative RF & ANA
 specificities greater than 92%, the sensitivity of Yamaguchi
(96%)

30. Treatment

31. 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.

32.  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

33.  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