Miglior Poster

A 49- year-old woman had a clinical history that began at age 35, characterized by the presence of anemia, increased C-reactive protein (CRP) and Erythrocyte sedimentation rate (ESR), hypergammaglobulinemia, symmetric polyarthritis involving the small joints in the hands and wrists, treated which resulted with improvement of symptoms. She was hospitalized in March because of fever and dyspnea. The chest CT scanning revealed interstitial pneumonia and pleural effusion, she was treated with antibiotic therapy for about 10 days. A medical examination showed fever, chest pain and cardipalmo and she was again hospitalized. At the clinical examination, she appeared unwell, febrile at 39 ºC, with a widespread vasculitic rash was present and most prominent on the legs. At the physical exam of the chest: dullness to percussion at the right base, with increased tactile fremitus and bilateral basal crackles. No evidence of lymphadenopathy was observed and the rest of the examination was unremarkable. During the hospitalization she presented pancitopenica, serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody, anti-SS-A, anti-SS-B, anti-Sm and anti-RNP antibody were positive. Futhermore she showed hyperferritinemia, hypofibrinogenemia, liver dysfunction with hypertriglyceridemia and raised liver enzymes, splenomegaly and bone marrow aspirate showed hemophagocytosis. Repeated urine and blood cultures were negative. Sputum was negative for bacterial and fungal cultures. A thorough infection screen, including a viral panel for Herpes Simplex, Herpes Zoster, Epstein-Barr, Cytomegalovirus, Hepatitis B, Hepatitis C, HIV, Echo and Coxsackie viruses was negative. She was diagnosed having Systemic lupus erythematosus (SLE) associated with Macrophage activation syndrome (MAS). She was treated only with corticosteroids (metilprednisolone) with improvement of symptoms.