Clinical Features and Prognosis of Adult-onset Still’s Disease: 61 Cases from China

2009 ◽  
Vol 36 (5) ◽  
pp. 1026-1031 ◽  
Author(s):  
TING ZENG ◽  
YU-QIONG ZOU ◽  
MEI-FANG WU ◽  
CHENG-DE YANG
Keyword(s):  
Prognostic Factors ◽  
Clinical Features ◽  
Chinese Patients ◽  
Adult Onset Still’S Disease ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Laboratory Findings ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Objective.To describe the onset, clinical features, prognostic factors, and treatment of adult-onset Still’s disease (AOSD) in cases from China.Methods.Sixty-one Chinese patients with AOSD were analyzed retrospectively.Results.Common clinical features were fever (100.0%), rash (88.5%), and arthritis (82.0%). The laboratory findings were as follows: leukocytosis (83.6%), increased erythrocyte sedimentation rate (100.0%), elevated transaminase concentrations (23.0%), elevated ferritin levels (79.6%), negative antinuclear antibody (88.5%), and negative rheumatoid factor (88.5%). Of the 61 patients, 44.3% exhibited a monocyclic disease pattern, 29.5% experienced disease relapse at least once, 16.4% exhibited chronic articular course, and 9.8% died; most deaths were due to pulmonary infection and respiratory failure. Based on the disease course, we divided the 61 patients into 2 groups: those with favorable outcome (cyclic disease course, n = 45) and unfavorable outcome (chronic disease course or death, n = 16). We analyzed the prognostic factors for the 2 groups, and found that pleuritis, interstitial pneumonia, elevated ferritin levels, and failure of fever to subside after 3 days of prednisolone at 1 mg/kg/day were unfavorable prognostic factors for patients with AOSD.Conclusion.Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone.

Association of Fcγ Receptor Polymorphisms with Adult Onset Still’s Disease in Korea

2009 ◽  
Vol 36 (2) ◽  
pp. 347-350 ◽  
Author(s):  
JIN-HYUN WOO ◽  
YOON-KYOUNG SUNG ◽  
JIN-SOOK LEE ◽  
WON TAE CHUNG ◽  
JUNG-YOON CHOE ◽  
...  
Keyword(s):  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Chi Square ◽  
Still's Disease ◽  
Specific Pcr ◽  
Chi Square Test ◽  
Adult Onset Still's Disease

Objective.Fcγreceptors (FcγR) have important functions in the regulation of immune response and clearance of immune complex. High levels of immunoglobulins have been observed in patients with the active stage of adult onset Still’s disease (AOSD), and high-dose intravenous immunoglobulin treatment has decreased the disease activity of AOSD. We investigated polymorphisms of FcγR as genetic factors influencing susceptibility or disease course of AOSD in Korea.Methods.We genotyped the FcγRIIA H/R131, IIIA F/V176, and IIIB NA1/NA2 loci in 98 patients with AOSD and 151 healthy controls. Genotyping was performed using sequence-specific PCR. Patients with AOSD were subdivided into groups according to disease course: monocyclic systemic, polycyclic systemic, or chronic articular type. Allelic, genotypic, and haplotypic associations were analyzed by chi-square test.Results.No significant skewing in any of the 3 FcγR polymorphisms was found between Korean AOSD patients and controls. FcγRIIA R/R131 and R/H131 genotype in patients with chronic articular-type disease was more frequent than in controls (p = 0.006 and pcorr = 0.018). No differences of genotypic and allelic frequencies were found between other disease course types and controls. Haplotype IIA R131-IIIA F176-IIIB NA2 was more frequent in AOSD patients than in controls (p = 0.021).Conclusion.Although FcγR polymorphisms are not associated with development of AOSD in Koreans, the haplotype IIA R131-IIIA F176-IIIB NA2 may be associated with AOSD. Also, the FcγRIIA polymorphism may be associated with chronic articular-type AOSD. We need to identify whether these polymorphisms are associated with a response to anti-tumor necrosis factor agents in patients with AOSD.

Adult onset still's disease: Clinical features and course

1992 ◽  
Vol 11 (4) ◽  
pp. 516-520 ◽  
Author(s):  
D. M. Sánchez Loria ◽  
M. J. Moreno Alvarez ◽  
J. A. Maldonado Cocco ◽  
E. J. Scheines ◽  
O. D. Messina
Keyword(s):  
Clinical Features ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Functional Association of Interleukin 18 Gene −607 (C/A) Promoter Polymorphisms with Disease Course in Chinese Patients with Adult-onset Still’s Disease

2009 ◽  
Vol 36 (10) ◽  
pp. 2284-2289 ◽  
Author(s):  
DER-YUAN CHEN ◽  
YI-MING CHEN ◽  
HSIN-HUA CHEN ◽  
CHIA-WEI HSIEH ◽  
CHI-CHEN LIN ◽  
...  
Keyword(s):  
Chinese Patients ◽  
Adult Onset Still’S Disease ◽  
Healthy Controls ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Interleukin 18 ◽  
Promoter Polymorphisms ◽  
Functional Association ◽  
Adult Onset Still's Disease

Objective.Interleukin 18 (IL-18) has a central role in the pathogenesis of adult-onset Still’s disease (AOSD). We investigated the functional association of −607 (C/A) IL-18 promoter polymorphisms with disease course in Chinese patients with AOSD.Methods.Sequence-specific primer polymerase chain reaction and the restriction fragment-length polymorphism method were used to analyze the genotypes of IL-18 promoter polymorphism at position −607 in 96 unrelated patients with AOSD and 164 ethnically-matched healthy controls. Serum IL-18 levels were determined using ELISA in patients with active untreated AOSD.Results.Significantly lower frequencies of single-nucleotide polymorphism −607/AA were observed in patients with AOSD compared to healthy controls (18.8% vs 31.1%, respectively; p < 0.05). Median levels of serum IL-18 were significantly lower in AOSD patients with AA genotype compared to those with CA genotype or CC genotype (147.5 pg/ml vs 410.5 pg/ml or 262.4 pg/ml, respectively; both p < 0.05). Significantly lower IL-18 levels were demonstrated in AOSD patients with a monocyclic systemic course than in those with a polycyclic systemic course or a chronic articular course. The AA genotype was more frequently observed in patients with monocyclic systemic course, which had the best prognosis, than in those with the other 2 disease courses. In contrast, a lower frequency of the AA genotype than the CA or the CC genotype was observed in patients with chronic disabling arthritis (5.5% vs 25.0% or 19.2%, respectively).Conclusion.The SNP −607/AA genotype with lower IL-18 levels might be a genetically protective factor for the occurrence of AOSD in the Chinese population, against progression of chronic disabling arthritis.

The Mid-Range of the Adjusted Level of Ferritin Can Predict the Chronic Course in Patients with Adult Onset Still’s Disease

2009 ◽  
Vol 36 (1) ◽  
pp. 156-162 ◽  
Author(s):  
SANG-WON LEE ◽  
YONG-BEOM PARK ◽  
JUNG-SOO SONG ◽  
SOO-KON LEE
Keyword(s):  
Chronic Disease ◽  
Ferritin Level ◽  
Adult Onset Still’S Disease ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Laboratory Results ◽  
Disease Pattern ◽  
Adult Onset Still's Disease

Objective.To find a measure that can predict the disease course in patients with adult onset Still’s disease (AOSD).Methods.We retrospectively investigated the medical records of 71 hospitalized patients with AOSD. Patients were divided according to chronic and nonchronic disease course. The initial laboratory results were defined as those at the time of admission, the extremely deviated laboratory results as the highest or the lowest results, and the adjusted laboratory results as area under the curve divided by the days of hospitalization. All measures were compared and the odds ratio (OR) for the chronic disease pattern was assessed.Results.The mean age was 39.7 ± 13.5 years and women accounted for 63 of the total 71 (88.7%). Thirty patients (42.3%) had self-limited disease, 9 (12.7%) intermittent disease, and 23 (32.4%) the chronic disease pattern (32.4%). Nine patients (12.7%) died. The initial levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT) and ferritin, the highest levels of lactate dehydrogenase (LDH) and ferritin, and the adjusted level of ferritin in patients with chronic disease were significantly higher than those with nonchronic disease. Among them, only the middle range of the adjusted ferritin level (784.1~4120.0 ng/ml) was found to have a significant predictive value for the chronic disease pattern (OR 81.7, p = 0.007).Conclusion.A novel measure, the adjusted level of ferritin during the first hospitalization, might be useful to predict progression to chronic disease in patients with AOSD.

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