- Quotidian fever, frequently >39°C.
- Evanescent, salmon-colored macular rash on trunk and extremities, often coincident with fever spikes.
- Clinical manifestations include pharyngitis, polyarthralgias, lymphadenopathy, splenomegaly, and serositis.
- Leukocytosis and elevations of the erythrocyte sedimentation rate, serum C-reactive protein, and serum ferritin are common laboratory abnormalities.
Adult-onset Still disease (AOSD) is a multisystem inflammatory disease of unknown cause. It affects women and men equally and is rare, with a reported incidence of 0.16 cases per 100,000 in one series. AOSD typically begins in the late teenage to early adult years, but disease onset occurs after the age of 35 in approximately 25% of patients. The onset of AOSD is systemic, with fever and rash; however, the subsequent disease course is variable. AOSD may consist of single (monocyclic) or recurrent (polycyclic) episodes of systemic disease lasting weeks to months. Alternatively, chronic articular disease, with or without recurrent systemic flares, may follow the initial systemic episode. Pediatric patients with systemic-onset juvenile idiopathic arthritis can have a recurrence of active Still disease at any age into adulthood. There are no definitive laboratory findings or diagnostic histologic abnormalities. The diagnosis is made on clinical grounds and often is one of exclusion (Table 16–1).
Table 16–1. Yamaguchi Classification Criteria for Adult-Onset Still Disease. ||Download (.pdf)
Table 16–1. Yamaguchi Classification Criteria for Adult-Onset Still Disease.
- Fever ≥39°C, intermittent, lasting 1 week or more
- Arthralgias or arthritis lasting 2 weeks or more
- Characteristic rash
- WBC ≥10,000/mcL with neutrophils ≥80%
- Pharyngitis or sore throat
- Hepatomegaly or splenomegaly
- Liver enzyme abnormalities
- Negative tests for rheumatoid factor and antinuclear antibodies
- Absence of infection
- Absence of malignant diseases
- Absence of inflammatory disease
|Classification as adult-onset Still disease requires the presence of five or more criteria, of which at least two must be major criteria.|
Fever is a hallmark of AOSD. Typically, the fever is quotidian, with spikes to ≥39°C (often in the afternoon or evening) followed by return to normal temperature in the absence of antipyretic medications. Shaking chills and sweating can accompany these daily temperature swings, which can be as high as 4°C. Occasionally, the fever is double quotidian (two spikes daily). In a small percentage of patients, a low-grade baseline fever persists between spikes. Untreated, the fever of AOSD is relentless, frequently lasting weeks at a time. Constitutional symptoms of profound fatigue, weight loss, and anorexia are common.
The rash of AOSD is a salmon-colored, macular or maculopapular eruption (Plate 34). Occasionally, it has an urticarial appearance. The rash usually affects the trunk and the extremities and spares the face, palms, and ...