[Diagnosis, progression and prognosis, aetiology and treatment of adult-onset Still's disease].

DIAGNOSTIC DOUBTS: Despite an increased awareness of the clinical features of the disease, adult onset Still's disease (AOSD) remains a diagnosis of exclusion. Many diagnostic criteria have been published, and the most popular are those proposed by Yamaguchi, even though they do not consider the presence of hyperferritinemia or the decrease of it glycosylated fraction, and they include exclusion criteria that are difficult to satisfy. UNPREDICTABLE PROGRESSION: Three evolutive forms have been described: monocyclic, intermittent with articular and/or systemic flares, chronic, usually in the form of chronic polyarthritis. Vital prognosis is sometimes compromised by severe systemic manifestations or the occurrence of amyloidosis, whilst destructive polyarthritis, particularly common in the chronic form, may compromise the function. The aetiology of AOSD remains unknown. THERAPEUTIC UNCERTAINTIES: Treatment is largely empirical in the absence of randomized clinical trials because of the rarity of the disease. Nonsteroidal anti-inflammatory drugs could be used in moderately severe forms of the disease, but they rare rarely sufficient. Around 80% of the patients require corticosteroids. Among the disease modifying drugs, methotrexate still remains the most effective. The role of the new anti-TNF molecules remains to be specified.