eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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4/2007
vol. 45
 
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abstract:

Original paper
Difficulties of diagnosis in adult-onset Still\'s disease in our material

Bożena Kowalewska
,
Eliza Roszkowska

Reumatologia 2007; 45, 4: 177–185
Online publish date: 2007/08/30
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Adult-onset Still’s disease (AOSD) is a rare, systemic inflammatory disorder of unknown cause characterised by quotidian fevers, evanescent rash, arthritis and multiorgan involvement: lymphadenopathy, splenomegaly, hepatomegaly, pleuritis or pericarditis.
Laboratory abnormalities include elevated ESR, a neutrophilic leukocytosis, elevated C-reactive protein (CRP), elevated levels of hepatic enzymes, hypoalbuminaemia with hypergammaglobulinaemia and particularly extreme elevations of serum ferritin.
AOSD patients are seronegative for rheumatoid factor (RF) and antinuclear antibody (ANA). There is no diagnostic specific test or pathognomonic histopathology, so the diagnosis of AOSD is often a diagnosis of exclusion.
The aim of the study was to present the clinical course of 19 AOSD patients treated in our centre. All patients presented symptoms of fever and arthritis, 74% with skin lesions; among some of them lymphadenopathy, splenomegaly, hepatomegaly, cardiac lesions occurred. The time from the first clinical activity to diagnosis was 6.6 months, the average age of patients 39.4 years.This group consisted of 10 women and 9 men. Among most patients typical laboratory abnormalities were observed. The disease’s clinical course of a few patients was monitored by ferritin level and indicated correlation of ferritin level with activity of disease. All patients were treated with non-steroidal antiinflammatory drugs, oral (prednisone) or intravenous (methylprednisolone) corticosteroids. Modifying antirheumatic drugs such as methotrexate were used.
keywords:

adult-onset Still’s disease, diagnostic criteria, clinical symptoms, laboratory findings, treatment





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