Adult-onset Still Disease: Diagnosis And Evolution [doença De Still Do Adulto: Diagnóstico E Evoluçã O]

Objective: To determine the clinical manifestations at disease onset and during follow-up of patients with Adult-Onset Still Disease (AOSD) in a tertiary university hospital in Brazil. Methods: Thirteen cases of AOSD were identified in the Arthritis Outpatients Unit -Unicamp. Their clinical records were reviewed retrospectively in order to determine clinical manifestations regarding disease onset, follow-up and prescription. Results: The prevalence of AOSD was 4.3%. The mean age of disease onset was 30.8 years old with a slight prevalence of men (54.2%). Constitutional symptoms, fever and cutaneous rash were observed in all cases. Hepatic involvement was observed in 11 patients, splenomegaly in 4, hematuria in 4, cardiac involvement in 2 and pleuritis in 2. Poliarticular involvement was observed more frequently, and 50% of them presented carpal ankylosis after 3 years of follow-up. Ferritine was elevated in 9 of 13 patients during active disease. All patients used oral non-steroidal anti-inflammatory drugs, steroids and 7 patients needed methotrexate. Conclusions: Although AOSD is a rare multisystemic rheumatic disorder, it should always be considered in febrile cases with poliarthritis. Neoplasia and infection should always be excluded. Normally it is characterized by a chronic course and carpal ankylosis as the main disabling feature.436352357Al-Arfaj, A.S., Al-Saleh, S., Adult-Onset Still's disease in Saudi Arabia (2001) Clin Rheumatol, 20 (3), pp. 197-200Cush, J.J., Adult-onset Still's disease (2000) Bull Rheum Dis, 49 (6), pp. 1-4Garcia-Porrua, C., Gonzalez-Gay, M.A., Crespo, F., Gonzalez-Juanatey, C., Adult onset Still's disease in Catalonia, Spain (2000) J Rheumatol, 27 (1), pp. 280-281Cousin, F., Grezard, P., Roth, B., Balme, B., Gregoire-Bardel, M., Perrot, H., Kikuchi's disease associated with Still's disease (1999) Int J Dermatol, 38 (6), pp. 464-467Schachna, L., Littlejohn, G.O., Adult-onset Still's disease: A concise review of clues to an important rare syndrome (1999) Aust N Z J Med, 29 (1), pp. 98-99Jaime, M.A.I., Baptista, R., Azevedo, M., Doença de Still do adulto: Estudo de 25 casos. (1998) Rev Bras Reumatol, 38, pp. 285-290Rêgo, J., Ximenes, A.C., Silva, N.A., Doença de Still no adulto. (1991) Rev Bras Reumatol, 31 (1), pp. 1-4Cheema, G.S., Quismorio Jr., F.P., Pulmonary involvement in adult-onset Still's disease (1999) Curr Opin Pulm Med, 5 (5), pp. 305-309Nguyen, K.H., Weisman, M.H., Severe sore throat as a presenting symptom of adult onset Still's disease: A case series and review of the literature J Rheumatol, 24 (3), pp. 592-597Moder, K.G., Miller, T.D., Allen, G.L., Cardiac tamponade: An unusual feature of adult onset Still's disease J Rheumatol, 22 (1), pp. 180-182Marie, I., Levesque, H., Perraudin, N., Cailleux, N., Lecomte, F., Courtois, H., Aseptic meningitis and cranial nerve palsy revealing adult-onset Still's disease (1999) Clin Infect Dis, 29 (1), pp. 220-221Schiller, D., Mittermayer, H., Hyperferritinemia as a marker of Still's disease Clin Infect Dis1998, 26 (2), pp. 534-535Akritidis, N., Giannakakis, Y., Sakkas, L., Very high serum ferritin levels in adult-onset Still's disease (1997) Br J Rheumatol, 36 (5), pp. 608-609Fujii, T., Akizuki, M., Kameda, H., Matsumura, M., Hirakata, M., Yoshida, T., Shinozawa, T., Mimori, T., Methotrexate treatment in patients with adult onset Still's disease-retrospective study of 13 Japanese cases (1997) Ann Rheum Dis, 56 (2), pp. 144-148Aydinting, A.O., D'Cruz, D., Cevera, R., Low dose methotrexate in treatment of AOSD (1992) J Rheumatol, 19, pp. 431-435Faufie, B., Borget, C., Rozenberg, S., Corticosteroid sparing effect of low dose MTX treatment in AOSD (1999) J Rheumatol, 26, pp. 373-378Sampalis, J.S., Esdaile, J.M., Medsger Jr, T.A., A controlled study of the long-term prognosis of adult Still's disease (1995) Am J Med, 98 (4), pp. 384-38