ADULT ONSET STILL'S DISEASE: A REVIEW OF 41 CASES

E. Riera, A. Olivé, J. Narváez, S. Holgado, M. Bianchi, L. Mateo, P. Santo, X. Tena, J. NollaRheumatology, Hospital Mutua de Terrassa, Terrassa Rheumatology, Hospital Universitari Germans Trias i Pujol de Badalona Rheumatology, Hospital Universitari de Bellvitge, Barcelona, SpainObjectives: To describe the clinical manifestations, treatment, evolution, prognosis and functional class of 41 patients with Adult Onset Still's Disease (AOSD). Methods: Retrospective study (1984-2006). Multicenter study. Setting: Hospital Universitari Germans Trias i Pujol and Hospital Universitari de Bellvitge. All patients fulfilled Yamaguchi et al Classification criteria. (J Rheumatol 1992; 19: 424-430). Results: Forty one patients were included (15 men and 25 women). Mean age at diagnosis: 38.19 years (SD 15,59). All patients had spiking fever. Typical exanthema was present in 38 patients (92,6%). Symptoms were preceded by sore throat in 37 (90,2%). Forty patients (97,5%) had polyarthralgia and 36 (88%) had arthritis; polyarticular pattern in 29 (80,5%) and oligoarticular in 7 (19,5%). Knees, wrists and ankles were the joints more often involved. Other clinical manifestations were lymphadenopathy in 17 patients (41%), hepatomegaly/splenomegaly in 16 (39%), abdominal pain in 5 (12%), pleuritis in 6 (14,6%), pericarditis in 5 (12%) and interstitial pneumopathy in 2 (4,8%). Most of the patients had leucocytosis and neutrophilia and a remarkable elevation of ESR and CRP. Liver function abnormalities were detected in 50% of the patients. Hyperferritinemia was preset in (83%.) The course of the disease was monocyclic in 44% of the patients and polycyclic/chronic in 56%. ASA or NSAID controlled the disease in seven patients (17,5%) and prednisone in eleven (27,5%). Immunosupressors were required in 55% of the patients and in seven of them (17,5%) a biological treatment with TNF-α blockers or anakinra had to be added in order to control the disease. Currently 17 patients (42,4%) are free of disease without treatment (mean duration of disease 114.74 moths. SD: 105,65) and totally asymptomatic (72.55 moths; SD 30,75). Fifteen patients had a monocyclic patterns and a polycyclic-chronic in two. Twenty-three patients (57,5%) are still on treatment (duration of disease: 110.96 months. SD: 70,22). Three patients treated with ASA, 1 with NSAID and prednisone, 8 with NSAID, prednisone and methotrexate, 4 with methotrexate alone and 7 with NSAID, prednisone, methotrexate and a biological treatment. Four patients had a monocyclic course and 19 a polycyclic-chronic one. Erosions were present in nine patients (22%). ACR class were as follows: 29 (72,5%) class I, 7 (17,5%) class II, 2 (5%) class III and 2 (5%) class IV. Conclusion: The clinical manifestations were similar to other series. A fevered polyarthritis was the most frequent clinical presentation. Patients with monocyclic course tend to have a better prognosis. Fifty six % of the patients had a polycyclic-chronic course and required an aggressive treatment. Radiological bony erosions were present in 22% of patients. Most patients are in functional class I. Disclosure of Interest: None declaredCitation: Annals of the Rheumatic Diseases, volume 68, supplement 3, year 2009, page 316Session: Miscellaneous rheumatic diseases (Poster Presentations )