[Systemic chronic juvenile arthritis (Still's disease) in adults. Review of the literature].

UNLABELLED

The entire literature up to the present of 119 patients described as having adult Still's disease is reviewed. Clinical, laboratory, radiological and histological findings, treatment, follow-up and outcome are presented. 1.

CLINICAL FINDINGS

All patients had arthralgias and/or myalgias. Seven did not fulfill strict diagnostic criteria as they did not have overt arthritis. All had fever, and 93% had septic fever with temperature peaks of greater than or equal to 39 degrees C. The characteristic maculo-papular rash was present in 89%. Other findings were sore throat, lymphadenopathy, splenomegaly (in about 50% each), weight loss, pericarditis, hepatomegaly, pleuritis (in 1/4 to 1/3 of the cases each). Less frequent were alopecia, pneumonitis and abdominal pain (in less than 10% each). 2.

LABORATORY FINDINGS

The ESR was uniformly elevated. Over 90% of the patients exhibited neutrophilic leukocytosis. Anemia, hypoalbuminemia and pathologic liver function tests were found in 60-80%. IgM-RF and ANA were both negative in 95% of the patients and hence were not more frequently present than in the normal population. 3. X-ray findings: There were no pathognomonic radiological abnormalities of the joints. Nevertheless, the tendency to ankylosis, especially of the carpus, seems to be typical. A few patients developed severe destructive lesions, especially of the hip and shoulder joints. 4. Biopsies: Many biopsies were performed and did not reveal specific histologic abnormalities. Nonetheless, they sometimes are necessary in order to exclude other disease entities. 5. Treatment, follow-up, prognosis: Therapeutically high-dosage ASA and (if necessary) systemic steroids are recommended for the acute phase. The efficacy of this treatment is controversial. Even after successful control of the acute disease, exacerbations are frequent for many years. Despite the initial optimism, there is significant long-term morbidity due to recurrent disease flare-ups on the one hand, and articular destructions in some patients on the other.