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SAT0608 (2018)
How to differentiate adult onset still’s disease from overall other causes of fever of unknown origin: results of a prospective study from a tertiary referral centre
E. Bilgin1, A. Erden2, B. Armagan2, L. Kl2, A. Sar2, G. Yardmc2, M.K. Hayran3, A. Akdoan1, . Karada2, S. Apra Bilgen2, I. Ertenli2, S. Kiraz2, U. Kalyoncu2
1Internal Medicine
2Internal Medicine, Division of Rheumatology
3Prevantive Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey

 

Background: Adult onset Still’s disease (AOSD) is a rare, auto-inflammatory disease that commonly presents as fever of unknown origin (FUO), and most common rheumatologic cause of FUO. Clinical and/or laboratory parameters that can discriminate AOSD from other causes of FUO need to be clarified in current literature.

Objectives: To determine clinical and/or laboratory parameters that help to differentiate AOSD from other causes of FUO and demonstrating a clinician-friendly algorithm for this purpose.

Methods: Data from patients who admitted to Hacettepe University Hospitals, inpatients sections of department of internal medicine with the complaint of FUO, who eventually had a certain diagnosis, collected prospectively during 30 months. AOSD patients followed at Hacettepe University department of rheumatology were included. Clinical and laboratory data were collected at the time of diagnosis of AOSD and time of admission of patients with FUO.

Results: Total 156 patients (n=69, for AOSD; n=87, for FUO) were included. FUO group were also divided into three subgroups: rheumatologic (n=31, 35.6%), infectious (n=28, 32.2%) and malignant (n=28, 32.2%) causes. While 51 (74%) patients were female in AOSD group, 43 (49,4%) patients were female in FUO group (p=0.03). Frequency of rash, arthralgia, arthritis, sore throat, fever at night (p<0.001 for each), history of hemophagocytosis (p=0.037) were significantly higher in AOSD group. Fever peak number equal and/or higher than 3, presence of lymphadenopathy (p=0002 and p=0,001,respectively) were significantly higher in FUO group. While leukocytosis, neutrophilia, thrombocytosis, hyperferritinemia, higher lactate dehydrogenase and complement 3 levels (p<0.001 for each) were significantly more frequent in AOSD group, albumin levels lower than 3 g/dl and positive rheumatoid factor (p=0.009 and p=0.002,respectively) were significantly more frequent in FUO group. Results of univariate and multivariate analysis are given in table 1. Algorithm for discrimination of AOSD and FUO is given atAbstract SAT0608 – figure 1.

Abstract SAT0608 – Table 1 Results of univariate and multivariate analysis

Univariate Analysis

Multivariate Analysis

Variables

Odds Ratio

Confidence Interval

p value

Odds Ratio

Confidence Interval

p value

Favours Still’s

Fever at night

7,66

3,53–16,5

<0001

Rash

10,08

4,80–21,2

<0001

31,3

3,6–271,9

0002

Arthritis

6,58

3,09–14,01

<0001

Arthralgia

36

10,46–123,8

<0001

158,1

4,3–5755,8

0006

Sore throat

27,72

11,58–66,33

<0001

20,8

2,8–154,7

0003

Hemophagocytosis

4,79

0,96–23,89

0079

Neutrophilia

10,87

4,90–24,13

<0001

18,4

2,6–132,3

0004

Ferritin≥5 x UNL

4,88

2,34–10,16

<0001

132,8

7,1–2502,9

0001

LDH

7,12

2,35–21,59

<0001

6,2

0,76–50,9

0087

C3

3,20

1,47–7,00

0003

Female

2,90

1,46–5,73

0002

Favours FUO

Pleuritis

2,04

0,68–6,12

0,19

Fever peak number≥3

3,66

1,16–11,52

0019

69

2,2–2114,4

0015

Lymphadenopathy

3,39

1,72–6,79

<0001

UNL: Upper normal limit (for ferritin: 336 ng/ml)
abs_ZRQQRRTW_F001.jpg

Conclusions: Presence of arthralgia, hyperferritinemia, sore throat and neutrophilia strongly favour AOSD in patients presenting as FUO. This study demonstrates a clinician-friendly algorithm for the first time in current literature.

Disclosure of Interest: None declared

DOI: 10.1136/annrheumdis-2018-eular.3786



Citation: Ann Rheum Dis, volume 77, supplement Suppl, year 2018, page A1156
Session: Other orphan diseases