Juvenīla idiopātiska artrīta klīniskās vadlīnijas

A.Vētra, D.Bērtule, I.Tāse - consultantspublishersversionPeer reviewe

Kawasaki disease in Latvia 2012–2019 : epidemiology and rate of resistance to initial treatment

Introduction: Kawasaki disease (KD) is an acute febrile illness of early childhood, characterised by vasculitis of the coronary and medium-sized arteries. The incidence of the disease differs worldwide, but the incidence of KD has not been established in Latvia. The aim of the study was to describe the epidemiological characteristics and estimate the incidence rate and resistance to initial treatment of KD among hospitalised children in Latvia. Material and methods: The study was a descriptive, population-based study, which used hospital discharge records of patients < 18 years old diagnosed with KD in the years 2012–2019. Incidence rate was calculated using the number of KD patients and corresponding national census data. Results: There were 36 KD patients in Latvia. The median age at admission was 2.8 years, with 67% of cases under 5 years of age, and the male/female ratio was 1.3 : 1. The mean annual incidence rate was 1.3 per 100,000 children 0–18 years old, and 2.9 per 100,000 for children < 5 years old. The mean length of hospital stay was 14 days. Coronary artery dilatation was recorded in 8% of the patients (all of whom were male). Every patient received intravenous immunoglobulin (IVIG), with a median of 7 days from fever onset to IVIG administration. Refractory treatment was reported in 25% of cases. Conclusions: This is the first epidemiological study of KD in Latvia. The incidence rates are lower than those reported for other European countries. The percentage of refractory treatment was higher than in other studies.publishersversionPeer reviewe

Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020 : A Nationwide Retrospective Study

Zane Davidsone is a representative of European Reference Network on Connective Tissue and Musculoskeletal Disease-ERN-ReCONNET.Background and Objectives: Systemic juvenile idiopathic arthritis (sJIA) is a distinctive JIA subtype with mostly nonspecific systemic clinical features, which can be a diagnostic challenge. This study aimed to analyze our experience with sJIA in Latvia for twelve years: assessing clinical and epidemiological characteristics, the efficacy of therapy, and disease outcomes, including the development of macrophage activation syndrome (MAS). Materials and methods: This is a descriptive study in which we conducted a retrospective case review of all patients with sJIA diagnosis admitted to the only pediatric tertiary centre in Latvia during the period 2009-2020. Results: sJIA was diagnosed in 35 patients with a mean annual incidence rate of 0.85 patients per 100,000 children. Major clinical signs at the first visit were: fever, rash, arthritis, and lymphadenopathy. Almost half of the patients, 48.5%, had a monocyclic disease course, and only 20% of patients had persistent disease. MAS developed in 28.6% of patients. Biological therapy was administered to 48.6% of patients, mostly by tocilizumab, which induced remission in 75% after one year, and in 81.2% after two years without any serious therapy-related complications. In our study, none of the patients had interstitial lung disease, drug reaction with eosinophilia and systemic symptoms (DRESS)-like syndrome, or fatal disease. Conclusions: The incidence and clinical characteristics of sJIA correlate with the literature findings, although MAS was more common than described in other studies. There is a tendency for the persistent disease to decrease with the use of biological therapy. Tocilizumab is an efficient choice of treatment with a good safety profile.publishersversionPeer reviewe

HLA II class alleles in juvenile idiopathic arthritis patients with and without temporomandibular joint arthritis

Publisher Copyright: © 2016 Davidsone et al. Copyright: Copyright 2017 Elsevier B.V., All rights reserved.Background: Temporomandibular joint (TMJ) arthritis is seen very often (38-87 %) in children with juvenile idiopathic arthritis (JIA). With contrast enhanced magnetic resonance imaging (MRI) we can detect more cases of TMJ arthritis than ever before. Previous studies show that HLA II class alleles may have protective or risk importance in JIA subtypes. Our objective is to identify HLA II class alleles of risk and protection in JIA patients with TMJ arthritis. Methods: During the period from 2010 to 2015 MRI for TMJ was performed in 85 JIA patients who were genotyped for HLA- DRB1; DQB1 and DQA1 using RT-PCR with sequence-specific primers. As a control group, data of 100 individuals were taken from the genetic bank of RSU Joint Laboratory of Clinical Immunology and Immunogenetics. Associations of DRB1; DQB1; DQA1 alleles in patients were examined individually using the χ 2 test. P-value (<0.05) and odds ratio were calculated using EPI INFO 6.0 software. Results: Out of 85 JIA patients with mean age of 13.7 ± 3.0 years (range 6.9-17.9 years), 59 (69 %) were girls and 26 (31 %) were boys. The mean duration of the disease was 3.07 ± 2.35 years (range 0.2-11.0 year). JIA subtypes were as follows: seronegative polyarthritis 51 (60 %), seropositive polyarthritis 6(7 %), oligoarthritis extended 7(8 %), oligoarthritis persistent 2 (2 %) arthritis with enthesitis 14 (17 %), undifferentiated 3 (4 %) and 2 (2 %) systemic arthritis. Two groups where separated after TMJ MRI exam: first with at least two signs of active inflammation and/or any structural damage (n = 62); second with no pathologic signs or with slight contrast enhancement (n = 23). We discovered that there are risk alleles that are found in all JIA patient's groups (MRI positive and negative groups) versus controls such as DRB1*07:01, DQB1*03:03; DQB1*05:01. Also some protective alleles as DRB1*18:01, DQB1*06:02-8 were found in overall JIA group. Alleles DRB1*12:01, DQB1*03:01; DQA1*05:01 were found to be protective for TMJ arthrits. Conclusion: In our study there were no convincing risk alleles, but there are alleles that probably are protective for TMJ arthritis like DRB1*12:01, DQB1*03:01; DQA1*05:01.publishersversionPeer reviewe

Proceedings Of The 23Rd Paediatric Rheumatology European Society Congress: Part Two

PubMe