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
ETIOLOG
Y
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.
Implicated organisms in
AOSD
-Rubella -Mycoplasma
-Ebstein Barr -Borelia burgdoferi
-Staphylococcus -Cytomegalovirus
-Parvovirus -Mumps
-Yersinia enterocolitica -Parainfluenza
Brucela abortus
Clinical Features
Common Clinical
Features
*Fever of at least
*Arthralgias 39ºC lasting
or arthritis lastingone
twoweek or or
weeks
longer
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
* 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
FEVE
Rin the late afternoon or early evening.
Quotidian or "double-quotidian" with a brief peak
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
0C
FEVE
R
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
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)
RAS
H
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.
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
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
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
Lymphadenopath
y
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
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.
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
Laboratory
Investigations
Absence of antinuclear antibodies Elevated serum amyloid
A
Absence of rheumatoid factor, Thrombocytosis
Elevated ESR and C-reactive protein Elevated serum ferritin and
glycosylated ferritin
Neutrophilic leukocytosis Elevations the hepatic enzymes
Hypoalbuminemia
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 and 50% have
mm/hr ESR > 90 mm/hr. and
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.
Diagnosi
s
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
Yamaguchi et al 1992
AOSD Total of > 5 criteria (including 2
major)
Major Criteria Minor Criteria
Fever > 39°C Sore throat
Arthralgia > 2 LN or
wks. Still's rash splenomegaly
Neutrophilic Liver dysfunction
leukocytosis Negative RF & ANA
specificities greater than 92%, the sensitivity of
Yamaguchi (96%)
Treatment
Treatme
nt
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 .
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
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
