Adult-Onset Still Disease (AOSD)

Article Figures & Data

Download figure
Open in new tab
Figure 1.
Magnetic resonance image of the left wrist. Gadolinium-enhanced, coronal, T1-weighted image demonstrates synovial proliferation and active synovitis (bright signal between and contiguous to the carpal bones and involving the radioulnar joints), as well as contiguous osseous erosions (arrows).
Download figure
Open in new tab
Figure 2.
Proposed therapeutic strategies in adult-onset Still disease (AOSD). NSAIDs, nonsteroidal anti-inflammatory drugs; IL, interleukin; CBC, complete blood count; ESR/CRP, erythrocyte sedimentation rate/C-reactive protein; LFT, liver function tests; TNF, tumor necrosis factor.

Table 1.

Laboratory Values of the Patient during the Course of Treatment

Component	First Admission	Second Admission	After Methotrexate	Reference Range
WBC (k/UI)	15.8	18.1	10.7	5.5–13
Hemoglobin (g/dL)	11.3	8.9	14.4	13.2–15.6
Hematocrit (%)	34.2	27.8	45.8	41–53
Platelet (k/ul)	253	558	235	150–400
Neutrophils (%)	60	41	67	28–78
Bands (%)	27	36	0	<10
C-reactive protein (mg/dL)	16	17.6	<0.5	<0.9
Erythrocyte sedimentation rate (mm/hr)	79	121	1	<16
Total protein (g/dL)	5.5	6.8	6.6	6.2–8.3
Albumin (g/dL)	2	2.3	4.1	3.4–4.8
Bilirubin, direct (mg/dL)	1.2	1	0.2	0.1–0.3
Bilirubin total (g/dL)	2.5	2.1	1.1	0.1–1.5
Alkaline phosphatase (IU/L)	262	291	89	50–190
ALT (IU/L)	143	142	42	7–40
AST (IU/L)	141	138	15	7–40
INR	1.3	1.3		0.9–1.1
Prothrombin time (sec)	14.4	15.2		10–12.5
LDH (U/L)	Not done	416	Not done	50–220
Ferritin (ng/mL)	Not done	46709.9	431.6	11.5–300
HIV	Negative	–	–	Negative
Cytomegalovirus	Negative	–	–	Negative
ANA	Negative	–	–	Negative
RF	Negative	–	–	Negative
ASO titer (IU)	Not done	177	–	<133
EBV VCA Ig M	Positive	Negative	–	Negative
EBV VCA Ig G	Positive	Positive	–	Negative
EBV early antigen	Negative	Equivocal	–	Negative
EBV nuclear antigen	Negative	Negative	–	Negative
Mycoplasma IgG AB	Positive	Positive	–	Negative
Mycoplasma IgM AB	Positive	Negative	–	Negative

WBC, white blood cell; ALT, alanine amino transaminase; AST, aspartate aminotransferase; INR, international normalized ratio; LDH, lactic dehydroginase level; HIV, human immunodeficiency virus; ANA, antinuclear antibody; RF, rheumatoid factor; ASO, antistreptolysin O; EBV, Epstein-Barr virus; VCA, viral capsid antigen; Ig, immunoglobulin; AB, antibody.

Table 2.

Yamaguchi Criteria*

Major Criteria	Minor Criteria	Exclusion Criteria
• Arthralgia, >2 weeks	• Sore throat	• Infection, especially sepsis
• Fever, >39°C, intermittent ≥1 week	• Lymphadenopathy and/or splenomegaly	• Epstein-Barr infection
• Typical rash (macula popular, nonpruritic)	• Abnormal liver function test	• Malignancy
• WBC >10,000 (>80% granulocytes)	• Rhematoid factor and ANA negative	• Inflammatory disease

* The Yamaguchi criteria require the presence of 5 criteria to diagnose Still disease, and at least 2 must be major criteria.¹²
WBC, white blood cell; ANA, antinuclear antibody.