2003 abstract from ACR meeting in Nov

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Adult Onset Still's Disease in the Setting of Fever of Unknown Origin

Category: 31 Miscellaneous rheumatic diseases

C. Crispin, Deborah ez-Baños, Alcocer-Varela.

Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico

City, Mexico

Presentation Number: 1598

Poster Board Number: 441

When patients with adult onset Still's disease (AOSD) present as

fever of unknown origin (FUO), diagnosis is complex due to its low

prevalence, the ambiguity of its clinical manifestations, and the absence of

specific serological markers. Hence, in clinical practice AOSD is considered

an exclusion diagnosis. The aim of this work was to evaluate the cases of

FUO admitted in our institution during the last 10 years and compare the

patients diagnosed as AOSD with the patients with other diagnosis.

We performed a case control study and analyzed 27 patients with

AOSD and 105 patients with FUO due to other diseases. We divided the control

group into three categories: a) infectious diseases (n=30); malignant

diseases (n=34); c) autoimmune diseases (n=41). In a secondary analysis we

considered only the patients with the most prevalent conditions, namely

tuberculosis (n=13), Hodgkin's disease (n=16), non-Hodgkin lymphoma (n=15),

and systemic lupus erythematosus (SLE, n=24).

The mean age of patients with AOSD was lower when compared to

the control patients (P<0.01). A higher percentage of patients with AOSD had

sore throat (P<0.01), evanescent rash (P<0.01), splenomegaly (P<0.05), and

elevations on leukocyte and granulocyte counts (P<0.001) when compared to

patients with other diseases. Additionally, arthralgia and arthritis were

more frequently observed in patients with AOSD than in patients with all

other diseases (P<0.01), except SLE. Patients with AOSD segregated clearly

from other diseases, except SLE. We compared these two clinical groups and

found that the parameters more strongly associated with AOSD were elevated

leukocyte count (OR [95%CI] 48.4 [8.4-276.5]), elevated granulocyte count

(27.8 [5.8-132.2]), evanescent rash (24.7 [2.9-210]), and sore throat (5.1

[1.5-17.1]). Parameters negatively associated with AOSD, were leukopenia

(0.45 [0.005-0.3]), positive serology (0.1 [0.05-0.6]), and

hypocomplementemia (0.1 [0.2-0.9]). When we compared the control patients

with AOSD patients, the negative predictive value of arthralgia was 96%, of

arthritis 95%, of elevated leukocyte count 94%, and of elevated granulocyte

count 94%.

Our findings suggest that although AOSD is an heterogeneous

condition with ambiguous clinical manifestations, it may be safely

distinguished from other diseases, including SLE, based on its pivotal

characteristics: arthralgia/arthritis, sore throat, evanescent rash, and

elevated leukocyte/granulocyte counts. Furthermore, these results suggest

that in a patient who is undergoing a diagnostic evaluation for FUO, the

absence of leukocytosis and arthritis make AOSD an unlikely diagnosis.

GO DAWGS !!!!!