scholarly journals Highly elevated serum levels of interleukin-18 in systemic juvenile idiopathic arthritis but not in other juvenile idiopathic arthritis subtypes or in Kawasaki disease: Comment on the article by Kawashima et al

2002 ◽  
Vol 46 (9) ◽  
pp. 2539-2541 ◽  
Author(s):  
Nobuaki Maeno ◽  
Syuji Takei ◽  
Yuichi Nomura ◽  
Hiroyuki Imanaka ◽  
Masashi Hokonohara ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Elevated Serum ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Serum Levels ◽  
Interleukin 18

scholarly journals The Role of Interleukin 18/Interleukin 18-Binding Protein in Adult-Onset Still’s Disease and Systemic Juvenile Idiopathic Arthritis

2022 ◽  
Vol 11 (2) ◽  
pp. 430
Author(s):  
Charlotte Girard-Guyonvarc’h ◽  
Mathilde Harel ◽  
Cem Gabay
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Binding Protein ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Free Form ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Interleukin 18 ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Interleukin 18 (IL-18) is a pro-inflammatory cytokine of the IL-1 family, whose activity is tightly controlled at the level of production, as well as signalization. Notably, it is buffered by its natural inhibitor, IL-18 binding protein (IL-18BP), which is massively present in circulation in normal and in most pathological conditions, thus preventing harmful pro-inflammatory systemic effects of IL-18. IL-18 has long been considered to be involved in the pathophysiology of various inflammatory diseases. However, a first clinical trial using recombinant IL-18BP for the treatment of rheumatoid arthritis and psoriasis gave disappointing results. Direct measurements of unbound, bioactive, free form of circulating IL-18 demonstrated that IL-18 was more specifically involved in adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA) but also in their most severe complication, macrophage activation syndrome (MAS). More importantly, administration of recombinant IL-18BP to patients with AOSD, and sJIA with MAS, showed promising results. This review summarizes available data regarding IL-18 and IL-18BP in AOSD and sJIA in mouse models and humans and shows the importance of IL-18/IL-18BP imbalance in these conditions, leading to the conclusion that IL-18, particularly free IL-18, may be a useful biomarker in these diseases and an interesting therapeutic target.

scholarly journals Elevated Serum Levels of IL-21 in Kawasaki Disease

2012 ◽  
Vol 4 (6) ◽  
pp. 351 ◽  
Author(s):  
Yon Jung Bae ◽  
Mi Hyun Kim ◽  
Hae Yong Lee ◽  
Young Uh ◽  
Mee Kyung Namgoong ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Elevated Serum ◽  
Serum Levels

Elevated serum levels of interleukin-18 in patients with overt diabetic nephropathy: effects of miglitol

2010 ◽  
Vol 15 (1) ◽  
pp. 58-63 ◽  
Author(s):  
Takashi Uzu ◽  
Hiroki Yokoyama ◽  
Hirofumi Itoh ◽  
Daisuke Koya ◽  
Atsushi Nakagawa ◽  
...  
Keyword(s):  
Diabetic Nephropathy ◽  
Elevated Serum ◽  
Serum Levels ◽  
Interleukin 18

Interleukin-18 as a mediator of systemic juvenile idiopathic arthritis

2006 ◽  
Vol 26 (8) ◽  
pp. 1332-1334 ◽  
Author(s):  
Marija Jelušić ◽  
Ivan Krešimir Lukić ◽  
Lana Tambić-Bukovac ◽  
Klara Dubravčić ◽  
Ivan Malčić ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Interleukin 18

scholarly journals Prevalence and Significance of Serum 14-3-3η in Juvenile Idiopathic Arthritis

2020 ◽  
Author(s):  
Iris Reyhan ◽  
Olga S. Zhukov ◽  
Robert J. Lagier ◽  
Robert F. Bridgeforth ◽  
Gary J. Williams ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Los Angeles ◽  
Disease Activity ◽  
Elevated Serum ◽  
Serum Levels ◽  
Control Group ◽  
Adult Rheumatoid Arthritis ◽  
Single Positive ◽  
Prompt Diagnosis

Abstract Background: Prompt diagnosis of juvenile idiopathic arthritis (JIA) is important to avoid long term complications. Elevated serum 14-3-3η levels improve the diagnostic sensitivity of rheumatoid factor (RF) and cyclic citrullinated peptide (CCP) antibody in adult rheumatoid arthritis (RA), and have been associated with more severe phenotype. We investigated the prevalence and clinical significance of serum 14-3-3η in different types of JIA.Methods: JIA patients (n=151) followed by the Pediatric Rheumatology Core at Children’s Hospital of Los Angeles were categorized into 5 groups: polyarticular JIA RF+ (PJIA RF+; n=39), PJIA RF- (n=39), psoriatic arthritis (PsA; n=19), enthesitis-related arthritis (ERA; n=18), and oligoarticular JIA (OJIA [control group]; n=36). RF, CCP antibody, and 14-3-3η were measured for all patients. 14-3-3η serum levels >0.2ng/mL were considered positive. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score-71 (JADAS-71). Results: Elevated 14-3-3h levels were detected in 34/151 (23%) patients, and across all groups tested. Most patients with 14-3-3h had titers ≥4 times above the cutoff value. The majority (22, 65%) of 14-3-3h-positive patients were also positive for RF or CCP antibodies, 16 (47%) were positive for all 3, and 12 (35%) were single-positive for 14-3-3η. The highest prevalence of 14-3-3η was in PJIA RF+ patients (49%), followed by OJIA (22%). Positivity for 14-3-3h was not significantly associated with disease activity or age at diagnosis. Conclusion: Serum 14-3-3h can be detected in all forms of JIA tested but appears to be most common in PJIA RF+. 14-3-3h does not appear to correlate with disease activity in JIA.

Abstract O.36: Kawasaki Disease and Systemic Juvenile Idiopathic Arthritis - Two Ends of the Same Spectrum

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Rae S Yeung ◽  
Mira van Veenendaal ◽  
Cedric Manlhiot ◽  
Rayfel Schneider ◽  
Brian W McCrindle
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Presumptive Diagnosis ◽  
Treatment Regimens ◽  
Hepatic Transaminases ◽  
Activation Syndrome

Background: Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. We critically reviewed children with KD and sJIA to identify those with both diagnoses in order to characterize discriminating findings at baseline. Methods: Data from prospectively acquired KD (n=1765) and sJIA (n=112) cohorts were reviewed for common patients (1990-2011). Those with both diagnoses (KD/sJIA n=8) were reviewed for clinical presentation, laboratory investigations, treatment regimens, coronary artery outcome and complications including macrophage activation syndrome, and results were compared to the overall KD cohort. Results: All children with KD/sJIA fulfilled diagnostic criteria for KD and sJIA (ILAR classification). Co-diagnosis was present in 0.45% (8 of 1765) and 7.1% (8 of 112) of those with KD and sJIA, respectively. Time between diagnosis of KD and presumptive diagnosis of sJIA was a median of 24 days (IQR 21-45 days). KD/sJIA patients had bilateral conjunctival injection less frequently, lower hepatic transaminases together with signs of more intense inflammation as expressed a by higher white blood cell count and lower albumin than the KD cohort alone. All KD/sJIA patients had recalcitrant disease consisting of prolonged fever requiring multiple doses of intravenous immunoglobulin and steroids. Coronary artery abnormalities (CAAs) were observed in 5 KD/sJIA patients. Macrophage activation syndrome occurred in one KD/sJIA patient and in 0.9% and 8% of KD patients and sJIA patients respectively. Conclusions: A small portion of our patients with KD developed subsequent sJIA. KD/sJIA patients were characterized by more intense inflammation at initial presentation, a recalcitrant disease course and a high prevalence of CAAs. These patients may provide clues to potentially shared immunopathology. The clinical presentations of MAS, KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 3 entities are syndrome complexes defined by massive immune activation. We propose that the intensity and duration of the immune response may be the key distinguishing features, which dictate which one of these clinical syndromes the affected child presents with.

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