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Baillieres Clin Rheumatol 1991 Aug;5(2):263-75

Adult-onset Still's disease.

van de Putte LB, Wouters JM

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.

Publication Types:
Review, tutorial
PMID: 1756584, UI: 92097216


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