Adult onset Still's disease: review of 41 cases

CER3847
2011 Vol.29, N°2
PI 0331, PF 0336
Review

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PMID: 21385548 [PubMed]

Received: 11/05/2010
Accepted : 02/12/2010
In Press: 19/04/2011
Published: 19/04/2011

Abstract

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%).