Objectives: To describe the clinical, laboratory and radiological features, treatment and prognosis of patients with adult onset Still's disease (AOSD).
Methods: Specific clinical features were retrospectively recorded in 41 patients fulfilling the Yamaguchi criteria. Patients were reviewed in two academic hospitals with a referral area of 700,000-1,000,000 inhabitants. Laboratory tests including haemogram, ferritin, biochemistry and autoimmunity were reviewed. Radiological studies, treatment and ACR functional class were determined.
Results: Forty-one patients with AOSD were identified, 25 of whom were female. Mean age at diagnosis: 38.19 years (range 17-68). Feverish polyarthritis was the most common clinical presentation. Acute phase reactants were invariably high in all patients. Serum ferritin levels were elevated in 86% of patients. Anti-cyclic citrullinated peptide antibodies (anti-CCP antibodies) were negative in all patients except one. The course of the disease was monocyclic in 44% of the patients, polycyclic in 26%, and chronic articular in 30%. ACR class was as follows: 29 (72.5%) class I, 7 (17.5%) class II, 2 (5%) class III and 2 (5%) class IV. As for the treatment received, aspirin or NSAIDs controlled the disease in eight patients (19.5%) and high-dose corticosteroids (0.5-1 mg/kg/day) in 32 (78%). Almost half of the patients (49%) required an additional diseasemodifying agent, usually methotrexate. Finally, in seven of them (17%) a biological treatment with TNF-α or specially anti-IL-1 had to be added to control the disease.
Conclusions: The clinical and laboratory findings were similar to previous studies. Anti-CCP antibodies were almost always negative. A monocyclic course was associated with a good prognosis. Most of the patients were in ACR functional class I and II. Biological agents were required in 7 patients (17%).