Adult-onset Still's disease

Abstract

Adult onset Still's disease seems to be the adult form of Still's disease in children. The key symptoms of the disease are high spiking fever, arthritis and a macular or maculopapular, salmon-pink evanescent rash, almost always accompanied by neutrophilic leukocytosis and frequently by sore throat, intense myalgias, lymphadenopathy, splenomegaly and signs of serositis. Tests for IgM rheumatoid factor and antinuclear antibody are characteristically negative. With respect to haematologic abnormalities, the disease may give rise to several problems. First, there is a neutrophilic leukocytosis, which currently is unexplained, and often a normocytic normochromic anaemia, that may be profound. The anaemia has the characteristics of anaemia of chronic inflammatory disease. Both abnormalities disappear after effective treatment of the disease or at spontaneous remission. Secondly, there might be a problem to differentiate AOSD from malignant haematological disorders, including malignant lymphoma and leukaemia, especially when the picture is dominated by lymphadenopathy, splenomegaly, fever and leukocytosis. Although in rare cases the differential diagnosis is extremely difficult, diagnosis can mostly be made or excluded by peripheral blood smear staining, bone marrow biopsies and occasionally lymph node biopsy. Finally, like the juvenile counterpart, AOSD is occasionally complicated by sometimes life-threatening diffuse intravascular coagulation. Factors that might be important in the development of this complication include severe disease activity, liver abnormalities and particular drugs including salicylates, other NSAIDs and some slow-acting antirheumatic drugs. Prompt therapy, including withdrawal of the drug, corticosteroids and sometimes anticoagulant therapy have been successfully applied to most patients.