Serum IL-18 as a potential specific marker for differentiating systemic juvenile idiopathic arthritis from incomplete Kawasaki disease

2014 ◽  
Vol 35 (1) ◽  
pp. 81-84 ◽  
Author(s):  
Tadamori Takahara ◽  
Masaki Shimizu ◽  
Yasuo Nakagishi ◽  
Noriko Kinjo ◽  
Akihiro Yachie
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Specific Marker ◽  
Incomplete Kawasaki Disease

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.

scholarly journals AB1002 IS KAWASAKI DISEASE OR SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS IN CHILDREN WITH FEVER WITHOUT A SOURCE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1794.2-1794
Author(s):  
B. Sözeri ◽  
F. Demir ◽  
T. Merter ◽  
M. Karacan
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Clinical Features ◽  
Systemic Vasculitis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Feature ◽  
Unknown Etiology ◽  
Ivig Treatment ◽  
Factors Affecting ◽  
The Mean

Background:Fever without a source (FWS) is caused by various diseases, making differential diagnosis difficult. Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. Kawasaki disease (KD), a self-limiting systemic vasculitis, remains of unknown etiology and can cause irreversible coronary artery aneurysms (CAAs). SoJIA is sometimes confused with incomplete KD because both diseases have overlapping clinical features and can be accompanied with CAAs and/or SJIA with macrophage activation syndrome (MAS).Objectives:In this study, the frequency of both KD and SJIA among the patients evaluated with FWS and the clinical features of patients diagnosed with Kawasaki disease.Methods:Medical records of patients who first visited our department between January 2016 and December 2019 were reviewedResults:A total of 107 patients were enrolled in this study, including 43 patients (40.2%, 23 males) who fulfilled the criteria of Kawasaki disease and 64 patients (59.8%, 39 males) who did not fulfill them. In patients who fulfilled the criteria of classical FWS, 36(33.6%, 20 males) patients were diagnosed with systemic juvenile idiopathic arthritis. The mean age of the patients with Kawasaki disease was 30.0±20,4 months (median 25 months), the mean age of other patients was 52,6±40 months (median 39,5 months). The mean age of the patients with sJIA patients was 87,6±49,8 (median 80months). Kawasaki patients were younger than others (p=0.01). There was no difference in gender between groups.In Kawasaki patients, the most common clinical feature at diagnosis was fever (100%) followed by conjunctival congestion and mucosal changes (69%). The last two findings are more significant in kawasaki patients than others (p<0,00). Twenty-six (59%) patients had completed KD while 25% had incomplete KD. 7 (16%) patients had atypical KD. The mean fever duration was longer in sJIA patients than KD and others (median 14,8 and 7 days, p<0.00). All patients with KD received IVIG (2 g/kg, infusion in 12 h) and aspirin (60 mg/kg/day). 13.6% of the patients also received oral corticosteroids because of IVIG resistance. Thirty-one patients (72.1%) responded to IVIG treatment, whereas 12 (6 female, 6 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 10 (22.7%) (6 female; 4 male) patients. We also detected 4 patients pericarditis with /without CIA.Conclusion:The clinical presentations of KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 2 entities may provide clues to potentially shared immunopathology.References:[1]Arslanoglu Aydin E et al. The factors affecting the disease course in Kawasaki disease. Rheumatol Int. 2019 Aug;39(8):1343-1349[2]Dong S et al. Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease. J Pediatr. 2015 May;166(5):1283-8.Disclosure of Interests:None declared

scholarly journals Kawasaki Disease and Systemic Juvenile Idiopathic Arthritis – Two Ends of the Same Spectrum

2021 ◽  
Vol 9 ◽  
Author(s):  
Ellen Go ◽  
Mira van Veenendaal ◽  
Cedric Manlhiot ◽  
Rayfel Schneider ◽  
Brian W. McCrindle ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Macrophage Activation ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Treatment Regimens ◽  
Coronary Artery Dilatation ◽  
High Prevalence ◽  
Disease Entities

Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (sJIA) are two distinct systemic inflammatory diseases of childhood. Each diagnosis is based on criteria, but numerous clinical features are overlapping. As no specific diagnostic tests are available, differentiation between both disease entities can be challenging. Here, we describe the disease course of patients with co-diagnosis of both KD and sJIA (KD/sJIA). All our KD (n = 1765) and sJIA (n = 112) cases were critically reviewed for co-diagnosis of KD/sJIA. Eight KD/sJIA cases were identified and their clinical presentation, treatment regimens, coronary artery outcome and complications are herein described. Each KD/sJIA patient fulfilled diagnostic criteria for KD and for sJIA. Ongoing fever, rash and arthritis were present in each patient. The KD/sJIA patients had recalcitrant KD requiring multiple doses of intravenous immunoglobulin and steroids. Five patients had coronary artery dilatation at KD diagnosis, which resolved in all by 6 weeks. Pericardial effusion was present in 5 patients. One KD/sJIA patient developed macrophage activation syndrome. In conclusion, a small proportion (0.5%) of our KD patients evolved into sJIA, and 7% of our sJIA population presented initially as KD. KD/sJIA patients were characterized by a recalcitrant KD course and a high prevalence of coronary artery dilatation. Patients with co-diagnoses may provide a clue to potentially shared immunopathology in KD and sJIA, leading us to posit that both entities may be part of the same clinical spectrum.

Incomplete Kawasaki Disease followed by Systemic Onset Juvenile Idiopathic Arthritis- The Diagnostic Dilemma

2012 ◽  
Vol 80 (9) ◽  
pp. 783-785 ◽  
Author(s):  
Shivani Dogra ◽  
Arushi Gehlot ◽  
Deepti Suri ◽  
Amit Rawat ◽  
Rohit Manoj Kumar ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Incomplete Kawasaki Disease ◽  
Diagnostic Dilemma ◽  
Systemic Onset

scholarly journals Performance of Recent PRINTO Criteria Versus Current ILAR Criteria for Systemic Juvenile Idiopathic Arthritis: A Single-Center Experience

2021 ◽  
Author(s):  
Oya KOKER ◽  
Fatma Gul DEMIRKAN ◽  
Figen CAKMAK ◽  
Nuray AKTAY AYAZ
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Pediatric Rheumatology ◽  
Early Recognition ◽  
Periodic Fever ◽  
Tumor Necrosis Factor Receptor ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Autoinflammatory Diseases ◽  
Control Group ◽  
Mevalonate Kinase Deficiency

Abstract Objectives The purpose of this study is to evaluate the performances of recently proposed Pediatric Rheumatology International Trials Organization (PRINTO) criteria versus current International League of Associations for Rheumatology (ILAR) criteria, for systemic juvenile idiopathic arthritis (sJIA). Methods The study was performed at the Department of Pediatric Rheumatology in Istanbul Faculty of Medicine with a retrospective design, covering the date range 2010 to 2021. Patients being followed-up with a diagnosis of sJIA, Kawasaki disease (control group-1), and common autoinflammatory diseases (control group-2) were included in the study. Both the ILAR and PRINTO classification criteria were applied to each patient and compared against expert rheumatologist diagnosis. Results Eighty-two patients with a diagnosis of sJIA compared against 189 [74 Kawasaki disease, 83 familial Mediterranean fever (FMF), 16 mevalonate kinase deficiency (MKD), 10 cryopyrin-associated periodic syndromes (CAPS), 6 tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS)] patients. The PRINTO criteria demonstrated higher sensitivity (62.2% vs 80.5%, p=0.003), but comparable specificity (90.5% vs 91%) as regards the ILAR criteria. Conclusions The revised criteria appear to enhance the ability to provide early recognition and pertinent classification of sJIA. The two criteria were not superior to each other in segregating sJIA from common autoinflammatory diseases and Kawasaki disease, namely in terms of specificity.

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