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Abstract Number: 269

Items of Yamaguchi Criteria Might be Associated with Disease Severity and Prognosis in Adult-Onset Still’s Disease

Umut Kalyoncu1, Dilek Solmaz2, Hakan Emmungil3, Ayten Yazici4, Timucin Kasifoglu5, Gezmis Kimyon6, Ayse Balkarli7, Cemal Bes8, Mustafa Ozmen9, Fatma Alibaz-Oner10, Sukran Erten11, Yonca Cagatay12, Gozde Cetin13, Sedat Yılmaz14, Fatih Yildiz15, Omer Nuri Pamuk16, Orhan Kucuksahin17, Levent Kilic1, Abdulsamet Erden1, Veli Yazisiz18, Omer Karadag1, Suleyman Serdar Koca19, Mutlu Hayran20, Servet Akar2, Kenan Aksu21, Nurullah Akkoc22, Gokhan Keser23, Emel Gonullu24, Bunyamin Kisacik25, Ahmet Mesut Onat25, Mehmet Soy26, Nevsun Inanc27, Haner Direskeneli28, Mehmet Sayarlioglu29, Eren Erken30, Murat Turgay31, Ayse Cefle32, Ihsan Ertenli1 and Salih Pay33, 1Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 2Rheumatology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey, 3Rheumatology, Ege University Faculty of Medicine, Izmir, Turkey, 4Rheumatology, Kocaeli University, Faculty of Medicine, Kocaeli, Turkey, 5Rheumatology, Eskisehir Osmangazi University School of Medicine, Eskisehir, Turkey, 6Department of Rheumatology, Gaziantep University School of Medicine, Gaziantep, Turkey, 7Department of Rheumatology, Pamukkale University School of Medicine, Denizli, Turkey, 8Rheumatology, Bolu Izzet Baysal School of Medicine, Bolu, Turkey, 9Rheumatology, Izmir Ataturk Research and Training Hospital, Izmir, Turkey, 10Department Rheumatology, Marmara University Faculty of Medicine, Istanbul, Turkey, 11Rheumatology, Ataturk Training and Research Hospital, Ankara, Turkey, 12Department of Rheumatology, Bilim University Faculty of Medicine, Istanbul, Turkey, 13Rheumatology, Sutcu Imam University Faculty of Medicine, Kahramanmaras, Turkey, 14PsART study group, Ankara, Turkey, 15Rheumatology, Cukurova University, School of Medicine, Adana, Turkey, 16Department of Rheumatology, Trakya University Medical Faculty, Edirne, Turkey, 17Rheumatology, Yildirim Beyazit University School of Medicine, Ankara, Turkey, 18Rheumatology, Akdeniz University School of Medicine, Antalya, Turkey, 19İnternal Medicine Division of Rheumatology, Firat University, Faculty of Medicine, Elazig, Turkey, 20Preventive Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 21Rheumatology, Ege University School of Medicine, Izmir, Turkey, 22Department of Rheumatology, Dokuz Eylul University, Faculty of Medicine, Izmir, Turkey, 23Rheumatology, Ege University Medical Faculty, Izmir, Turkey, 24Rheumatology, Osmangazi University, Faculty of Medicine, Eskisehir, Turkey, 25Rheumatology, Gaziantep University School of Medicine, Gaziantep, Turkey, 26Rheumatology, Abant Izzet Baysal University Faculty of Medicine, Bolu, Turkey, 27Marmara University Faculty of Medicine, Rheumatology, Istanbul, Turkey, 28Rheumatology, Marmara University, Faculty of Medicine, Istanbul, Turkey, 29Department of Internal Medicine Division of Rheumatology, Ondokuz Mayıs University, Faculty of Medicine, Samsun, Turkey, 30Rheumatology, Cukurova University Faculty of Medicine, Adana, Turkey, 31Internal Medicine-Rheumatology, Ankara University School of Medicine, Ankara, Turkey, 32Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey, 33Rheumatology, Gulhane School of Medicine, Ankara, Turkey

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Still's disease and prognostic factors

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Session Information

Date: Sunday, November 8, 2015

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:
Widely
accepted outcome measures for disease activity and severity at Adult Onset
Still’s Disease (AOSD) is not developed until now. Predictive factors for worse
prognosis, such as response to initial treatment or life-threatening
complications  are also not clearly defined  Yamaguchi criteria is routinely
used  for AOSD diagnosis which include 4 major and 4 minor criteria, reflecting
almost whole essential AOSD features. These essential features might also be
associated with disease severity and the objective of this study was to assess the
relationship of arithmetic count of Yamaguchi criteria with clinical,
laboratory features and treatment choice.  

Methods: At
2012, 19 rheumatology centers collected data on clinical features
(demographics, fever, rash, arthralgia, arthritis, myalgia, sore-throat,
lymphadenopathy,  hepatomegaly,  splenomegaly, pleuritis,  pericarditis and
rare findings including macrophage activation syndrome) laboratory features
(complete blood count, neutrophil percentage, ferritin, erythrocyte
sedimentation rate, C-reactive protein (CRP), AST, ALT, ALP, GGT, LDH,
bilirubin, albumin, triglyceride, total cholesterol, HDL-cholesterol,
LDL-cholesterol), treatment choices and disease course, retrospectively.
Overall 356 patients were enrolled and 269 of 356 patients were available for
further analysis. Yamaguchi items were added arithmetically. Total Yamaguchi
scores were between 5 to 8. At the end of statistical analysis, a cut-off of 7
or more score is chosen.  

Results: Overall,
269 (56.1% female) patients were assessed. Mean age was 38.5 (14.5) years and
mean diagnosis age was 34.9 (14.5) years. Total Yamaguchi score ≥ 7 was present
in 83 (30.9%) patients. Hepatomegaly and serositis were found more frequently
at Yamaguchi score ≥ 7. Essential laboratory activity parameters, such as
ferritin and CRP were also related with high Yamaguchi scores. There was a
difference at treatment choices that physicians started higher dosage of corticosteroids
with more frequent methotrexate use (Table).

Conclusion:
Higher Yamaguchi scores may be one of the criteria for higher disease activity
and severity in AOSD. We suggest that clinical features, level of acute phase
reactant/ferritin and treatment choices are affected from disease load.     

Table: Clinical, laboratory and treatment differences
according toYamaguchi Score in AOSD

 

Yamaguchi < 7

n=186

Yamaguchi ≥ 7

n=83

p

Clinical Differences

 

 

 

Hepatomegaly (%)

20

46

<0.001

Pleuritis (%)

5

17

0.002

Pericarditis (%)

4

13

0.008

Laboratory Differences

 

 

 

Ferritin mean (SD)

3882 (8608)

7966 (10193)

0.001

Ferritin > 1500 (%)

54.6

88.9

<0.001

Ferritin > 10.000 (%)

10.3

30.9

<0.001

CRP mg/L mean (SD)

117 (78)

158 (90)

<0.001

ALP mean (SD)

154 (147)

203 (160)

0.028

ALP > UL (%)

35

47

0.002

GGT mean (SD)

78 (91)

146 (142)

<0.001

GGT > UL (%)

45

73

<0.001

LDH mean (SD)

576 (698)

953 (1341)

0.008

LDH > UL (%)

43

72

<0.001

Albumin mean (SD)

3.49 (0.56)

3.13 (0.55)

<0.001

Albumin ≤ 3.0 mg/dl (%)

19

37

0.005

Thrombocytosis

41

27

0.023

Treatment Differences

 

 

 

Initial treatment with methotrexate (%)

53

68

0.021

Baseline corticosteroid dosage (mg) mean (SD)

43.2 (17.9)

51.6 (16.7)

0.001

 


Disclosure: U. Kalyoncu, None; D. Solmaz, None; H. Emmungil, None; A. Yazici, None; T. Kasifoglu, None; G. Kimyon, None; A. Balkarli, None; C. Bes, None; M. Ozmen, None; F. Alibaz-Oner, None; S. Erten, None; Y. Cagatay, None; G. Cetin, None; S. Yılmaz, None; F. Yildiz, None; O. N. Pamuk, None; O. Kucuksahin, None; L. Kilic, None; A. Erden, None; V. Yazisiz, None; O. Karadag, None; S. S. Koca, None; M. Hayran, None; S. Akar, None; K. Aksu, None; N. Akkoc, None; G. Keser, None; E. Gonullu, None; B. Kisacik, None; A. M. Onat, None; M. Soy, None; N. Inanc, None; H. Direskeneli, None; M. Sayarlioglu, None; E. Erken, None; M. Turgay, None; A. Cefle, None; I. Ertenli, None; S. Pay, None.

To cite this abstract in AMA style:

Kalyoncu U, Solmaz D, Emmungil H, Yazici A, Kasifoglu T, Kimyon G, Balkarli A, Bes C, Ozmen M, Alibaz-Oner F, Erten S, Cagatay Y, Cetin G, Yılmaz S, Yildiz F, Pamuk ON, Kucuksahin O, Kilic L, Erden A, Yazisiz V, Karadag O, Koca SS, Hayran M, Akar S, Aksu K, Akkoc N, Keser G, Gonullu E, Kisacik B, Onat AM, Soy M, Inanc N, Direskeneli H, Sayarlioglu M, Erken E, Turgay M, Cefle A, Ertenli I, Pay S. Items of Yamaguchi Criteria Might be Associated with Disease Severity and Prognosis in Adult-Onset Still’s Disease [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/items-of-yamaguchi-criteria-might-be-associated-with-disease-severity-and-prognosis-in-adult-onset-stills-disease/. Accessed .
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