Clinical Findings
• 1-2 times a day, >= 39 °C, intermittent fever is seen.
• With fire; On the trunk, a maculopapular, salmon-colored rash appears. It disappears with the decrease of fever.
• Oligo-Polyartricular joint involvement is observed.
• Most patients have lymphadenopathy and/or hepatosplenomegaly.
• Sore throat (non-suppurative pharyngitis) is a common finding
• Pleural and/or pericardial effusion may be present.
laboratory findings
• Erythrocyte sedimentation rate and CRP were significantly increased.
• Normochromic normocytic anemia, leukocytosis (neutrophilic) and thrombocytosis are expected.
• Ferritin levels are too high.
• ANA and RF are often negative (seronegative).
• Liver enzymes are often elevated.
Diagnosis
• The diagnosis of adult-onset Still's disease is a diagnosis of exclusion.
Yaroaguchi Criteria
Major criteria
- Fever (intermittent, >= 39 °C, >=1 week)
- Characteristic rash
- Arthritis/ Arthralgia (>= 2 weeks)
- Leukocytosis (neutrophil dominance)
Minor criteria
- Throat ache
- Lymph adenopathy
- Hepatomegaly / Splenomegaly
- KCFT height
- ANA and RF negativity
For diagnosis;
* Infection, malignancy, other rheumatological diseases should be excluded
* A total of 5 criteria are required, with at least 2 major criteria.
Treatment
• NSAIDs can be given in the presence of mild and moderate inflammatory symptoms.
• Corticosteroids are preferred for severe inflammatory symptoms.