Adult Still's Disease: Cohort Study

Purpose describe the clinical and laboratory finding, as well as the treatment, of adult still's disease (ASD). Methods retrospective analysis of a 24 patients cohort. Results clinical features were: fever (100%), rash (79.1%) and arthritis (66.6%). Most frequent laboratory alterations were: leukocytosis (75%), followed by increased PCR, VSG and ferritin, 70.8%, 66.65% and 62.5% respectively. 50% of the patients showed a monocyclic course, while 33% showed polycyclic systemic and 17% joint polycyclic. No joint monocyclic cases were found. Besides, some unusual manifestations such urticaria in 7 patients (29%); interstitial lung disease in 6 patients (25%); aseptic meningitis in 4 patients (16.6%) and SDRA in 4 patients (16.6%) were found. All the patients received steroid and 9 of them (37.5%) required therapy with IV methilprednisolone. Methotrexate was the most used medication between all; DMARD in 15 cases (62.5%), followed by chloroquine, alone or in combination, in 11 patients (45.8%). Besides, 3 patients (12.5%) required treatment with anti TNF due to poor response to conventional therapy. 2 patients died (8.4%) due to adult respiratory distress syndrome. Conclusions ASD has a broad and difficult differential diagnosis despite classical features. The presence of unusual manifestations may delay the diagnosis and treatment of this disease. ASD in not a benign disease and may appear with high mortality when it is associated with severe lung involvement.