Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort

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.

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

The Journal of Rheumatology ◽

10.3899/jrheum.071254 ◽

2009 ◽

Vol 36 (2) ◽

pp. 347-350 ◽

Cited By ~ 5

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.

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

The Journal of Rheumatology ◽

10.3899/jrheum.080537 ◽

2009 ◽

Vol 36 (1) ◽

pp. 156-162 ◽

Cited By ~ 20

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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Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

Journal of Clinical Medicine ◽

10.3390/jcm10040733 ◽

2021 ◽

Vol 10 (4) ◽

pp. 733

Author(s):

Stylianos Tomaras ◽

Carl Christoph Goetzke ◽

Tilmann Kallinich ◽

Eugen Feist

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Large Body ◽

Adult Onset Still’S Disease ◽

Clinical Aspects ◽

Disease Course ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Systemic Onset ◽

Adult Onset Still's Disease

Adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. The reason behind the nomenclature of this condition is that AoSD shares certain symptoms with Still’s disease in children, currently named systemic-onset juvenile idiopathic arthritis. Immune dysregulation plays a central role in AoSD and is characterized by pathogenic involvement of both arms of the immune system. Furthermore, the past two decades have seen a large body of immunological research on cytokines, which has attributed to both a better understanding of AoSD and revolutionary advances in treatment. Additionally, recent studies have introduced a new approach by grouping patients with AoSD into only two phenotypes: one with predominantly systemic features and one with a chronic articular disease course. Diagnosis presupposes an extensive diagnostic workup to rule out infections and malignancies. The severe end of the spectrum of this disease is secondary haemophagocytic lymphohistiocytosis, better known as macrophage activation syndrome. In this review, we discuss current research conducted on the pathogenesis, diagnosis, classification, biomarkers and complications of AoSD, as well as the treatment strategy at each stage of the disease course. We also highlight the similarities and differences between AoSD and systemic-onset juvenile idiopathic arthritis. There is a considerable need for large multicentric prospective trials.

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Adult-onset still's disease. Disease course and HLA associations

Arthritis & Rheumatism ◽

10.1002/art.1780290316 ◽

1986 ◽

Vol 29 (3) ◽

pp. 415-418 ◽

Cited By ~ 39

Author(s):

Jacques M. G. W. Wouters ◽

Paul Reekers ◽

Levinus B. A. van de Putte

Keyword(s):

Adult Onset Still’S Disease ◽

Disease Course ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Adult Onset Still's Disease

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Elevated Expression of C-Type Lectin Domain Family 5-Member A (CLEC5A) and Its Relation to Inflammatory Parameters and Disease Course in Adult-Onset Still’s Disease

Journal of Immunology Research ◽

10.1155/2020/9473497 ◽

2020 ◽

Vol 2020 ◽

pp. 1-11

Author(s):

Po-Ku Chen ◽

Shie-Liang Hsieh ◽

Joung-Liang Lan ◽

Chi-Chen Lin ◽

Shih-Hsin Chang ◽

...

Keyword(s):

Mrna Expression ◽

Adult Onset Still’S Disease ◽

Disease Course ◽

Adult Onset ◽

Still’S Disease ◽

Domain Family ◽

Lectin Domain ◽

Receptor Complexes ◽

Still's Disease ◽

Adult Onset Still's Disease

C-type lectin domain family 5-member A (CLEC5A) associates with adaptor DAP12 (DNAX activation protein 12) to form receptor complexes involved in inflammatory responses. We postulated a potential role of CLEC5A in the pathogenesis of adult-onset Still’s disease (AOSD) and aimed to investigate CLEC5A expression and its association with activity parameters and disease course. In 34 AOSD patients and 12 healthy controls (HC), circulating levels of CLEC5A-expressing monocytes or granulocytes were determined by flow cytometry analysis, the mRNA expression of CLEC5A and DAP12 on PBMCs by quantitative PCR, and plasma levels of proinflammatory cytokines by ELISA. AOSD patients had significantly higher percentages and mean fluorescence intensity (MFI) of CLEC5A-expressing monocytes (median 62.1% and 3.20, respectively) or granulocytes (72.6% and 3.22, respectively) compared with HC (in monocytes: 17.0% and 0.65, both p<0.001; in granulocytes: 67.3%, p<0.05 and 0.90, p<0.001; respectively). Patients also had significantly higher levels of CLEC5A mRNA expression on PBMCs compared with HC (median 1.77 vs. 0.68, p<0.05). The levels of CLEC5A-expressing monocytes or granulocytes were positively associated with activity scores and levels of IL-1β and IL-18 in AOSD patients. The patients with a systemic pattern had significantly higher levels of CLEC5A-expressing granulocytes and IL-18 compared to those with a chronic articular pattern of disease course. After 6 months of therapy, levels of CLEC5A-expressing monocytes and granulocytes significantly declined, paralleling the decrease of AOSD activity. Elevated CLEC5A levels and their positive association with activity parameters suggest that CLEC5A is involved in the pathogenesis and may serve as an activity indicator of AOSD.

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