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Article Excerpt
Byline: Neeta S. Ogden, MD, Carla M. Ward, MD, Apostolos Kontzias, MD, Sheron T. Wyatt, MD and Petros Efthimiou, MD
ABSTRACT: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease characterized by evanescent rash; daily high, spiking fevers; and arthritis. Diagnosis necessitates exclusion of other processes, including neoplastic and infectious causes and other autoimmune diseases. Laboratory tests are nonspecific and reflect heightened immunologic activity, with leukocytosis, elevated acute phase reactant levels, and elevated serum ferritin levels. Abnormal serum liver function test results are common; rheumatoid factor and antinuclear antibodies usually are absent. Treatment traditionally consisted of a combination of low-dose corticosteroids and disease-modifying antirheumatic drugs or intravenous gamma globulin. The recent application of anticytokine therapy with biologic agents has shown efficacy in refractory cases and has provided insight into the pathophysiology. (J Musculoskel Med. 2006;23:788-800)
Adult-onset Still disease (AOSD) is a systemic inflammatory disorder the cause of which is unknown. The clinical manifestations include the classic triad of evanescent rash, fever, and articular involvement, as well as sore throat and serositis. AOSD is a rare condition; occasionally, the symptoms are severe.
Bywaters1 first described AOSD as a distinct clinical syndrome in 1971. AOSD most often occurs in persons aged 16 to 35 years2; in a Japanese series, the mean patient age was 38.1 years.3 However, the disorder may affect persons of all ages. AOSD occurs more frequently in women than in men (female to male ratio, 60:40),4 but men are more likely to have disease onset at a younger age.5 The disease is associated with unusual physical or emotional stress in the year preceding the onset of symptoms.6
The diagnosis of AOSD is primarily clinical and requires exclusion of other processes, including neoplastic and infectious causes and other autoimmune diseases. The diagnostic criteria proposed by Yamaguchi and associates7 are most widely used and validated (Table). A newer set of classification criteria, that proposed by Fautrel and coworkers,8 does not have exclusion criteria and incorporates newly described serologic markers, such as serum ferritin and its glycosylated fraction.
AOSD treatment traditionally consisted of a combination of low-dose corticosteroids and disease-modifying antirheumatic drugs (DMARDs), as well as NSAIDs as adjunct therapy for symptomatic control. However, recent advances in basic research have enhanced understanding of the disease pathophysiology and emphasized the role of cytokines. The application of anticytokine therapy with the tumor necrosis factor a (TNF-a) inhibitors, originally developed for patients with rheumatoid arthritis, may revolutionize therapy for patients with AOSD-if proved effective in rigorous controlled trials.
In this article, we provide 20 "clinical pearls," or insights, to help primary care physicians make the diagnosis of AOSD. We also describe new and promising treatments.
1. When AOSD should be suspected
The onset of AOSD often is heralded by a sore throat and other constitutional symptoms. The sore throat, which may occur days to weeks before the onset of rash or fever, is seen in more than 70% of patients.9 The constitutional symptoms that follow may include myalgia or arthralgia, fatigue, anorexia, nausea, and weight loss. The latter, which may be dramatic and rapid, tends to parallel the inflammatory activity, as measured by decreasing serum albumin values and decreasing serum hemoglobin levels.9
2. Focus on the fever pattern
The fever typically is high and spiking, usually quotidian (recurring every day) and, occasionally, double quotidian.10 Temperature generally exceeds 39 degrees C (102.2 degrees F), often is accompanied by the characteristic "Still's rash," and decreases spontaneously. The spike occurs in late afternoon or evening, and the patient's temperature may be normal (or even lower than normal) between temperature spikes.11 Typically, the duration of fever is brief and the temperature drops rapidly. In one retrospective study, 100% of patients with AOSD presented with fever.12 AOSD often is the underlying cause of fever of unknown origin.
3. Recognizing the rash
The Still's rash that is characteristic of AOSD is seen in more than 92% of patients.9 It is evanescent, lasts for hours, often appears during febrile attacks, and can change from day to day. Typically, Still's rash is a salmon-colored, morbilliform (measles-like) cutaneous eruption that may be found on the trunk, neck, and extensor surfaces of the extremities (Figure 1); often, the face is spared and the eruption is nonpruritic.10,12
A Koebner-like phenomenon-the appearance of new lesions in uninvolved skin resulting from minor trauma or pressure-may be present. The rash is more prominent in areas that...
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