Abstract Twenty Six

Abstract Twenty Six

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

DISCLAIMER:

The materials and information on this server are intended for educational and informational purposes only. The materials and information are not intended to replace the services of a trained health professional or to be a substitute for medical advice of physicians and/or other health care professionals. The International Still's Disease Foundation is not engaged in rendering medical or professional medical services. You should consult your physician on specific medical questions, particularly in matters requiring diagnosis or medical attention. The International Still's Disease Foundation makes no representations or warranties with respect to any treatment, action, application medication or preparation by any person following the information offered or provided within this website. Any information used from other websites was done so with permission from each site, with an exception to those of "public domain", whereas we believe any site without a cited reference was a "public domain site" and for our use. The International Still's Disease Foundation is a non-profit organization. This page was last updated on January 17, 2001