scholarly journals Frequency of juvenile idiopathic arthritis and associated uveitis in pediatric rheumatology clinics in Turkey: A retrospective study, JUPITER

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Sezgin Sahin ◽  
Ceyhun Acari ◽  
Hafize Emine Sonmez ◽  
Fatma Zehra Kilic ◽  
Erdal Sag ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA), is the most common pediatric rheumatologic disorder with unknown etiology. Currently, no population-based data are available regarding the distribution of categories and frequency of uveitis in patients with JIA in Turkey. The purpose of this study was to evaluate the frequency of JIA-associated uveitis (JIAU) and distribution of JIA categories in a Turkish JIA cohort. Methods This was a retrospective study of 500 randomized patients in four pediatric rheumatology clinics in Turkey. Results Oligoarticular JIA (oJIA) was the most common JIA disease category in this study cohort (38.8%). The frequencies of the other categories were as follows: enthesitis-related arthritis (ERA), 23.2%; rheumatoid factor (RF)–negative polyarthritis, 15.6%; systemic arthritis, 12.2%; juvenile psoriatic arthritis, 5.2%; undifferentiated arthritis, 2.8%; and RF-positive polyarthritis, 2.2%. JIA-associated uveitis was observed in 6.8% of patients at a mean (Standard Deviation, SD) age of 9.1 (3.8) years over a mean JIA disease duration of 4 (1.9) years. Uveitis developed after joint disease, with a mean (SD) duration of 1.8 (1.9) years. Patients with oJIA had the highest rate of uveitis (12.9%) followed by patients with ERA (5.2%) and polyarticular RF-negative disease (3.8%). Compared with persistent oJIA, the extended oJIA category had a > 3-fold higher risk of uveitis (11.3% vs 27.7%; odds ratio, 3.38 [95% Confidence Interval, 1.09–10.4]). The most frequently administered drug after development of uveitis was tumor necrosis factor–alpha inhibitors (38.2%). Five patients (14.7%) had uveitis-related complications that required surgical intervention. Conclusions Turkish pediatric patients with JIA experience a lower frequency of oJIA and higher frequency of ERA than their white European counterparts; the occurrence of uveitis is also somewhat lower than expected. Geographic and ethnic factors may affect these differences and need further investigation.

2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Elisabet Berthold ◽  
Bengt Månsson ◽  
Robin Kahn

Abstract Background As the treatment arsenal for children with juvenile idiopathic arthritis (JIA) has expanded during the last decades, follow-up studies are needed on children diagnosed in the era of biological treatment to evaluate if this has improved the outcome. Our aim was to study the epidemiology and outcome of JIA in southern Sweden using a population-based cohort of children with a validated diagnosis of JIA collected over 9 years. Methods Potential cases of JIA between 2002 and 2010 were collected after a database search, using the ICD codes M08-M09. The study area was Skåne, the southernmost county of Sweden (population 1.24 million; 17.6% aged < 16 years). The JIA diagnosis was validated and subcategorized through medical record review based on the criteria defined by the International League of Associations for Rheumatism (ILAR). Parameters on disease activity and pharmacologic treatment were recorded annually until the end of the study period (December 31, 2015). Results In total, 251 cases of JIA were confirmed. The mean annual incidence rate for JIA was estimated to be 12.8/100,000 children < 16 years, with the highest age-specific annual incidence at the age of 2 years (36/100,000). Oligoarthritis was the largest subgroup (44.7%), and systemic JIA was the smallest subgroup (2.8%). Methotrexate was the most common disease-modifying anti-rheumatic drug prescribed (60.6%). Tumor necrosis factor alpha inhibitors were used as treatment for 23.9% of the children. Only 40.0% of the follow-up years, with a median follow-up time of 8 years, were free of arthritis or uveitis. Uveitis occurred in 10.8% of the children (8.0% chronic uveitis), and the need for joint corrective orthopedic surgery was 9.2%. Conclusions The incidence of JIA in this well-defined, population-based cohort is slightly lower than in previously published studies from Scandinavia. The need for orthopedic surgery and the presence of uveitis are diminished compared to studies with patients diagnosed more than 20 years ago. Children with JIA however still experience disease activity more than 50% of the time. In conclusion, we still have long-term challenges in the care for children with JIA, in spite of state-of-the-art treatment.


2019 ◽  
Vol 10 (1) ◽  
pp. 84-88
Author(s):  
Gule Tajkia ◽  
Syed Khairul Amin ◽  
Shamim Rima ◽  
Soma Halder ◽  
Fabia Hannan

Systemic-onset juvenile idiopathic arthritis (sJIA) or Still's disease is a chronic inflammatory disease of unknown etiology belongs to the group of Juvenile Idiopathic Arthritis. In contrast to other JIA patients in whom the joint disease usually overshadows the more general symptomatology, in Systemic-onset juvenile idiopathic arthritis (sJIA) extra-articular features such as spiking fever, hepatosplenomegaly, lymphadenopathy, rash, pleurisy, or pericarditis, and vasculopathy are most prominent. Thus the onset of disease can be vary nonspecific and may suggest bacterial or viral infection, malignancy or other rheumatic disease. As it is highly characterized by its extra-articular systemic illness features, in some ways, it resembles a fever of unknown origin. We present 2 cases of fever of unknown origin, initially presented with fever and other extra-articular features, without any arthritis, but after several months develops arthritis and finally diagnosed as Systemic-onset juvenile idiopathic arthritis (sJIA) or Still's disease . Anwer Khan Modern Medical College Journal Vol. 10, No. 1: Jan 2019, P 84-88


BMJ Open ◽  
2018 ◽  
Vol 8 (8) ◽  
pp. e021516 ◽  
Author(s):  
Yan Zhang ◽  
Yadong Niu ◽  
Liang Zhang

ObjectiveThe lack of coordinated and appropriate healthcare across sectors has produced more patients for county hospitals in China. This study examined differences in patient choice between township and county hospitals for readmission after a first township hospitalisation, and the determinants that influenced this choice.DesignA retrospective study of readmissions across hospitals after a first admission in township hospital. A township–township (TT) inpatient group and a township–county (TC) inpatient group were compared. A two-level logistic regression model was used to examine the determinants of choice for hospital readmission.SettingData were drawn from a population-based health utilisation database for Qianjiang District, China, from 1 January 2008 to 31 December 2013.ParticipantsThis study focused on readmitted patients whose first admission was in a township hospital. Readmission cases were identified as the same diagnosis (International Classification of Diseases, Tenth Revision) in a subsequent hospitalisation within 30 days. In total, 6764 readmissions had first admissions in township hospitals.Primary outcome measuresPatient choice for hospital readmission after a first township hospitalisation.ResultsThe TT group accounted for 62.5% (4225) and the TC group for 37.5% (2539) of readmissions in 6 years, and the proportion of TC readmissions in total inpatients increased from 1.66% to 1.89%. Readmission rates varied among towns (p<0.001). Differences between the TC and TT groups included: length of stay (LOS) of first admission (6.96 days vs 9.23 days), average interval between admissions (6.03 days vs 14.95 days) and disease category. Admission year, age, travel time to county hospital, interval between admissions, first admission LOS and disease category were determinants of choice for hospital readmission.ConclusionsPatients whose first admission was in a township hospital were more likely to be readmitted to a county hospital. A combination of first LOS and interval between admissions may be an effective identification index for TC readmission.Trial registration numberChiCTR-OOR-14005563.


1998 ◽  
Vol 30 (6) ◽  
pp. 573-577 ◽  
Author(s):  
Lauritz B. Dahl ◽  
Anne-Lise Høyland ◽  
Harald Dramsdahl ◽  
Per Ivar Kaaresen

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 240.2-241
Author(s):  
F. Zekre ◽  
R. Cimaz ◽  
M. Paul ◽  
J. L. Stephan ◽  
S. Paul ◽  
...  

Background:Idiopathic juvenile arthritis (JIA) is a heterogeneous group of pathologies whose origin remains unknown at present (1). They are characterised by a systemic inflammatory and joint disease affecting children under 16 years of age. The current classification groups the different forms of JIA into 7 distinct entities (systemic forms, polyarticular forms with or without rheumatoid factors, oligoarticular forms, inflammatory arthritis associated with enthesopathies (ERA), arthritis associated with psoriasis and unclassifiable arthritis). Exact etiology of JIA is still unknown. To date, the various hypotheses put forward on the occurrence of JIAs integrate the genetic and environmental framework.The link between periodontal disease and rheumatoid arthritis (RA) is largely reported. Recently, Porphyromonas gingivalis (P. gingivalis) infection explained the occurrence of arthritis in rodent and in RA (2). Several studies mention the beneficial effect of P. gingivalis treatment on disease activity.Currently, there are very few studies on the prevalence of P. gingivalis in patients with JIA and the possible involvement of the germ in the development of inflammatory joint diseases in the pediatric population(3)(4).Objectives:The objective of our study is to determine presence of high IgG antibodies against P. gingivalis and Prevotella Intermedia in a cohort of patients with JIA compared to a control population and to determine variation of level according to sub-classes of JIA.Methods:Sera were obtained from 101 patients satisfying the ILAR classification criteria for JIA and in 25 patients with two other dysimmune disorders (type 1 diabetes and juvenile inflammatory bowel disease). Level of IgG antibodies against P. gingivalis and Prevotella Intermedia were obtained by homemade ELISA already used previously (5).Results:In the JIA group, major children were oligarthritis (47.5%), polyarthritis represents 31.7% of JIAs, ERA and systemic forms of JIA are respectively 9 and 11%. For the control group, 10 (40%) children had diabetes and 15 (60%) had IBD.Levels of anti-P. gingivalis anti-Prevotella Intermedia antibodies were higher in AJI group compared at control groups (P<0.01, P<0.05). Theses difference are mainly related to oligoarthritis and ERA subsets for both P. gingivalis and Prevotella Intermedia.Figure 1.Relative titer of antibodies to P. gingivalis and anti Prevotella intermedia. *: P<0.05; **: P<0.01; ***: P<0.001. P. gingivalis (control vs oligoarthritis p= 0.0032. control vs ERA p= 0.0092). Prevotella intermedia (control vs oligoarthritis p= 0.0194. control vs ERA p= 0.0039).Conclusion:We confirmed high level of anti-P. gingivalis and anti-Prevotella intermedia antibodies in JIA compared to other inflammatory disorders. For the first time, we observed that this high level was mainly in oligoarthritis and ERA. Further investigations are required to investigate involvement of oral dysbiosis in AJI pathogenesis. As observed in RA, it could be a new way to integrate in JIA therapy management.References:[1]Thatayatikom A, De Leucio A. Juvenile Idiopathic Arthritis (JIA). StatPearls Publishing; 2020[2]Cheng Z, Meade J, Mankia K, Emery P, Devine DA. Periodontal disease and periodontal bacteria as triggers for rheumatoid arthritis. Best Pract Res Clin Rheumatol. 2017;31(1):19–30.[3]Romero-Sánchez C, Malagón C, Vargas C, Fernanda Torres M, Moreno LC, Rodríguez C, et al. Porphyromonas Gingivalis and IgG1 and IgG2 Subclass Antibodies in Patients with Juvenile Idiopathic Arthritis. J Dent Child Chic Ill. 2017 May 15;84(2):72–9.[4]Lange L, Thiele GM, McCracken C, Wang G, Ponder LA, Angeles-Han ST, et al. Symptoms of periodontitis and antibody responses to Porphyromonas gingivalis in juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2016 Feb 9[5]Rinaudo-Gaujous M, Blasco-Baque V, Miossec P, Gaudin P, Farge P, Roblin X, et al. Infliximab Induced a Dissociated Response of Severe Periodontal Biomarkers in Rheumatoid Arthritis Patients. J Clin Med. 2019 May 26;8(5).Disclosure of Interests:None declared.


2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Sabine A. Hartlieb ◽  
Lina Sieverling ◽  
Michal Nadler-Holly ◽  
Matthias Ziehm ◽  
Umut H. Toprak ◽  
...  

AbstractTelomere maintenance by telomerase activation or alternative lengthening of telomeres (ALT) is a major determinant of poor outcome in neuroblastoma. Here, we screen for ALT in primary and relapsed neuroblastomas (n = 760) and characterize its features using multi-omics profiling. ALT-positive tumors are molecularly distinct from other neuroblastoma subtypes and enriched in a population-based clinical sequencing study cohort for relapsed cases. They display reduced ATRX/DAXX complex abundance, due to either ATRX mutations (55%) or low protein expression. The heterochromatic histone mark H3K9me3 recognized by ATRX is enriched at the telomeres of ALT-positive tumors. Notably, we find a high frequency of telomeric repeat loci with a neuroblastoma ALT-specific hotspot on chr1q42.2 and loss of the adjacent chromosomal segment forming a neo-telomere. ALT-positive neuroblastomas proliferate slowly, which is reflected by a protracted clinical course of disease. Nevertheless, children with an ALT-positive neuroblastoma have dismal outcome.


2018 ◽  
Vol 119 (6) ◽  
pp. 720-725 ◽  
Author(s):  
Yair Lahav ◽  
Yoram Epstein ◽  
Ron Kedem ◽  
Haggai Schermann

AbstractAnthropometric measures of body composition are often used for rapid and cost-effective estimation of percentage body fat (%BF) in field research, serial measurements and screening. Our aim was to develop a validated estimate of %BF for the general population, based on simple body circumferences measures. The study cohort consisted of two consecutive samples of health club members, designated as ‘development’ (n 476, 61 % men, 39 % women) and ‘validation’ (n 224, 50 % men, 50 % women) groups. All subjects underwent anthropometric measurements as part of their registration to a health club. Dual-energy X-ray absorptiometry (DEXA) scan was used as the ‘gold standard’ estimate of %BF. Linear regressions where used to construct the predictive equation (%BFcal). Bland–Altman statistics, Lin concordance coefficients and percentage of subjects falling within 5 % of %BF estimate by DEXA were used to evaluate accuracy and precision of the equation. The variance inflation factor was used to check multicollinearity. Two distinct equations were developed for men and women: %BFcal (men)=10·1−0·239H+0·8A−0·5N; %BFcal (women)=19·2−0·239H+0·8A−0·5N (H, height; A, abdomen; N, neck, all in cm). Bland–Altman differences were randomly distributed and showed no fixed bias. Lin concordance coefficients of %BFcal were 0·89 in men and 0·86 in women. About 79·5 % of %BF predictions in both sexes were within ±5 % of the DEXA value. The Durnin–Womersley skinfolds equation was less accurate in our study group for prediction of %BF than %BFcal. We conclude that %BFcal offers the advantage of obtaining a reliable estimate of %BF from simple measurements that require no sophisticated tools and only a minimal prior training and experience.


Cancers ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 2254
Author(s):  
Matteo Franchi ◽  
Roberta Tritto ◽  
Luigi Tarantini ◽  
Alessandro Navazio ◽  
Giovanni Corrao

Background: Whether aromatase inhibitors (AIs) increase the risk of cardiovascular (CV) events, compared to tamoxifen, in women with breast cancer is still debated. We evaluated the association between AI and CV outcomes in a large population-based cohort of breast cancer women. Methods: By using healthcare utilization databases of Lombardy (Italy), we identified women ≥50 years, with new diagnosis of breast cancer between 2009 and 2015, who started adjuvant therapy with either AI or tamoxifen. We estimated the association between exposure to AI and CV outcomes (including myocardial infarction, ischemic stroke, heart failure or any CV event) by a Cox proportional hazard model with inverse probability of treatment and censoring weighting. Results: The study cohort included 26,009 women starting treatment with AI and 7937 with tamoxifen. Over a median follow-up of 5.8 years, a positive association was found between AI and heart failure (Hazard Ratio = 1.20, 95% CI: 1.02 to 1.42) and any CV event (1.14, 1.00 to 1.29). The CV risk increased in women with previous CV risk factors, including hypertension, diabetes and dyslipidemia. Conclusions: Adjuvant therapy with AI in breast cancer women aged more than 50 years is associated with increased risk of heart failure and combined CV events.


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