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STILL'S DISEASE

Eleanor J. Rutherford, MD

Still's disease is a subset of juvenile rheumatoid arthritis which has systemic features as the major manifestations. Fever, rash, leukocytosis, lymphadenopathy, pleuropericarditis, sore throat, and hepatosplenomegaly are common in addition to the arthritis. Adults, usually under age 40, may be affected and commonly present with fever of unknown origin. The characteristic evanescent, macular, salmon-colored rash is usually on the torso and upper arms and may be brought out by mechanical stimulation of the skin (Kobner's phenomenon). It is most evident at times of fever, which characteristically occur in the evening, with resolution by the following morning. Hectic fever curves with two or more spikes per day are also common.

The arthritis may affect large joints more commonly than small joints and may lead to significant morbidity. Many patients will require total joint arthroplasty for severe destructive changes. Wrist involvement commonly leads to joint destruction and bony ankylosis. Amyloidosis may develop after years of disease, more commonly in adult-onset disease.

An accelerated ESR and leukocytosis are seen in over 90% of patients. Anemia, hypoalbuminemia, and abnormal liver function tests are seen in a majority of patients. ANA and RF are present in less than 10%. Curiously, extreme elevation of ferritin (>1000 µg/l) may be seen in a majority of patients; such elevations are not generally seen in other forms of inflammatory arthritis, suggesting a relative specificity of this finding. Ferritin levels may fluctuate with disease activity, as does the ESR.

Treatment is aimed at controlling the inflammatory process. An initial trial of salicylates is warranted, although most patients will eventually require corticosteroids for control of systemic symptoms. Second-line anti-inflammatory drugs (gold, methotrexate) have been used successfully to control the synovitis.

References

1. Bywaters EGL. Still's disease in the adult. Ann Rheum Dis 1974; 30:121-132.

2. Ohta A, Yamaguchi M, Kaneoka H, Nagayoshi T, Hiida M. Adult Still's disease: review of 228 cases from the literature. J Rheumatol 1987; 14:1139-1146.

3. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992; 19:424-430.

4. Gonzalez-Hernandez T, Martin-Mola E, Fernandez-Zamorano A Balsa-Criado A, deMigeul-Medieta E. Serum ferritin can be useful for diagnosis in adult onset Still's disease. J Rheumatol 1989; 16:412-413.

5. Schwarz-Eywill M, Heilig B, Bauer H, Breitbart A, Pezzuto A. Evaluation of serum ferritin as a marker for adult Still's disease activity. Ann Rheum Dis 1992; 51:683-685.