Serum β2-microglobulin level is a useful indicator of disease activity and hemophagocytic syndrome complication in systemic lupus erythematosus and adult-onset Still’s disease

2013 ◽  
Vol 32 (7) ◽  
pp. 999-1005 ◽  
Author(s):  
Kuninobu Wakabayashi ◽  
Shigeko Inokuma ◽  
Erika Matsubara ◽  
Kae Onishi ◽  
Hiromitsu Asashima ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Hemophagocytic Syndrome ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Systemic Lupus ◽  
Β2 Microglobulin ◽  
Adult Onset Still's Disease

scholarly journals Systemic Lupus Erythematosus (SLE) in a male patient previously diagnosed as Adult Onset Still’s Disease: A Case Report

2019 ◽  
Vol 30 (2) ◽  
pp. 100-103
Author(s):  
Md Anwar Sayed ◽  
Suman Chowdhury
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Male Patient ◽  
Lupus Erythematosus ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Systemic Lupus ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare clinical entity with unknown etiology, characterized by evanescent rash, arthritis, fever, and other systemic presentation. In this case report, we describe a male patient of 50 years, previously diagnosed as a case of Adult onset still’s disease based on Yamaguchi criteria after the exclusion of other potential diagnoses. Later he was admitted into the Medicine department of Chittagong Medical College Hospital where he was found to have serological features of Systemic lupus erythematosus, another very much uncommon autoimmune disorder in male. On several occasion of his past admissions, SLE and RA were excluded meticulously. He initially responded to oral steroids only, recurrence of symptoms led us to work on the underlying etiology further. Coexistence of SLE in a patient with AOSD is not so commonly found. In our case, we notice this interesting phenomenon which was crucial for his management. Bangladesh J Medicine July 2019; 30(2) : 100-103

scholarly journals IL-18 Serum Level in Adult Onset Still’s Disease: A Marker of Disease Activity

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Serena Colafrancesco ◽  
Roberta Priori ◽  
Cristiano Alessandri ◽  
Carlo Perricone ◽  
Monica Pendolino ◽  
...  
Keyword(s):  
Disease Activity ◽  
Lupus Erythematosus ◽  
Therapeutic Option ◽  
Serum Levels ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Human Sera ◽  
Adult Onset Still's Disease

Introduction. Immunological factors seem to play a pivotal role in Adult Onset Still's Disease (AOSD). Among all, IL-18 cytokine is overexpressed and drives the inflammatory process.Objective. We aimed to investigate the levels of IL-18 in sera of Italian patients with AOSD and to assess its possible role as a marker of disease activity.Methods. IL-18 serum levels were determined by ELISA in 26 Italian patients with AOSD. Disease activity was assessed using Pouchot’s criteria. As controls, 21 patients with Rheumatoid Arthritis (RA), 21 patients with Sjogren's Syndrome (SS), 20 patients with Systemic Lupus Erythematosus (SLE), and 21 healthy subjects (normal human sera, NHS) were evaluated.Results. IL-18 serum levels were significantly higher in patients with active AOSD than in non-active (P=0.001) and control groups (RAP=0.0070, SSP=0.0029, SLEP=0.0032, NHSP=0.0004). A significant correlation between IL-18 serum levels and disease activity (P<0.0001), and laboratory parameters as ferritin (P=0.0127) and C-reactive protein (P=0.0032) was demonstrated.Conclusions. Higher levels of IL-18 are detected in active AODS patients and correlate with disease activity and inflammatory laboratory features. ROC-AUC analysis of the serum concentration of IL-18 suggests that it can be considered a diagnostic marker of AOSD. This paper supports the targeting of this cytokine as a possible therapeutic option in AOSD.

scholarly journals Evaluation of the serum β2 Microglobulin level in patients with systemic lupus erythematosus and its correlation with disease activity

BioMedicine ◽  
2019 ◽  
Vol 9 (3) ◽  
pp. 16 ◽  
Author(s):  
Miramir Aghdashi ◽  
Simak Salami ◽  
Ahmad Nezhadisalami
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Statistical Tests ◽  
Systemic Lupus ◽  
Β2 Microglobulin ◽  
Reactive Protein ◽  
Significant Difference ◽  
Control Study

Background: Designation of disease activity is serious for the management of systemic lupus erythematosus (SLE). Serum level of β2 microglobulin (β2M) may be associated with illness activity in SLE disease. Since the role of β2M for assessing of illness activity in SLE is not completely clear, the current study aimed to discern evaluation of β2M in patients with SLE and its correlation with sickness activity. Materials and Methods: In this case-control study, 50 patients with SLE disease and 25 healthy individuals were selected in Imam Khomeini Hospital in central of Urmia. Blood samples were collected safely from patients, serum was removed, and β2M measured using an ELISA method. The results for other parameters including C reactive protein, C3, C4, anti dsDNA and erythrocyte sedimentation rate were obtained from patients’ medical record. Data analyzed using appropriate statistical tests including Mann-Whitney U test, Independent f-test, Kruskal-Wallis, and Spearman used for analysis of data. Results: In the current study, a significant difference was seen between two groups in terms of β2M (p < 0.001). Remarkable correlation was seen between the level of β2M with disease activity (p < 0.001). Furthermore, there are significant relevancy between the level of β2M with 24-hour urine protein, ESR, disease activity score, and CRP (p < 0.05). Conclusion: The results revealed that serum amount of β2M in SLE patients is higher compared to healthy ones, which is significantly correlated to score of illness activity, CRP, and ESR in patients with SLE disease. Hence β2M might be an excellent serological marker helping the prediction of sickness activity and inflammation in SLE patients.

scholarly journals Adult-onset Still’s disease, the most internistic of the rheumatic diseases: current concepts and a report of six cases

2013 ◽  
pp. 259-264
Author(s):  
Roberto Boni ◽  
Pier G. Rabitti
Keyword(s):  
Serum Ferritin ◽  
Hemophagocytic Syndrome ◽  
Unknown Origin ◽  
Essential Elements ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown origin. It is characterized by spiking fever, evanescent rash, arthralgia/arthritis, and leukocytosis. The differential diagnosis includes a number of other conditions, and management is complicated by the lack of course predictors and the risks associated with complications and treatments. This report examines recent advances in our understanding of adult-onset Still’s disease (pathogenesis, diagnosis, complications, treatment). Current research in this field is focused on the significance of serum ferritin in AOSD, mechanisms underlying the hemophagocytic syndrome, and use of biologic therapies in patients who are refractory to conventional treatment. Six cases of AOSD diagnosed by our staff between 2002 and 2009 are also analyzed and compared with other cases reported in the literature. This analysis showed that Still’s rash and serum ferritin levels were not essential elements for diagnosis. In addition, the course of the disease showed little relation to the severity / characteristics of the presenting picture, but the evolution worsened with the age of the patient at diagnosis.

scholarly journals Serum sTREM-1 in adult-onset Still’s disease: a novel biomarker of disease activity and a potential predictor of the chronic course

Rheumatology ◽  
2020 ◽  
Vol 59 (11) ◽  
pp. 3293-3302 ◽  
Author(s):  
Zhihong Wang ◽  
Huihui Chi ◽  
Yue Sun ◽  
Jialin Teng ◽  
Tienan Feng ◽  
...  
Keyword(s):  
Disease Activity ◽  
Multivariate Logistic Regression Analysis ◽  
Soluble Form ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Potential Predictor ◽  
Still's Disease ◽  
Chronic Course ◽  
Adult Onset Still's Disease

Abstract Objectives Triggering receptor expressed on myeloid cells-1 (TREM-1) is an amplifier of inflammatory signals. Recently, a soluble form of TREM-1 (sTREM-1) was described. This study aimed to investigate the role of serum sTREM-1 in patients with adult-onset Still’s disease (AOSD). Methods Serum sTREM-1 levels were detected in 108 AOSD patients, 88 RA patients and 112 healthy controls (HC). The correlations of sTREM-1 with disease activity, clinical characteristics and laboratory parameters in AOSD patients were analysed by the Spearman correlation test. Risk factors for the chronic course of AOSD were evaluated by multivariate logistic regression analysis. Results AOSD patients had significantly higher serum sTREM-1 levels than RA patients and HC, and serum sTREM-1 levels were correlated with the systemic score, ferritin, leucocyte count, CRP, IL-1β and IL-6. The elevation in the initial sTREM-1 level by itself could discriminate patients developing the chronic course from patients developing the nonchronic course. Moreover, an elevated sTREM-1 level (&gt; 526.4475 pg/ml) was an independent risk factor for the chronic course in active AOSD patients. Furthermore, interfering with TREM-1 engagement led to reductions in the secretion of pro-inflammatory cytokines, such as IL-1β, IL-6 and TNF-α, in neutrophils and monocytes from active AOSD patients. Conclusion Serum sTREM-1 levels are correlated with disease activity, and an elevation in the initial serum sTREM-1 level is a potential predictor of the chronic course in AOSD patients, which currently provides the best predictive model for identifying patients prone to developing the chronic course of AOSD.

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