scholarly journals Outcome in juvenile idiopathic arthritis: a population-based study from Sweden

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 17 (1) ◽  
Author(s):  
Mia Glerup ◽  
◽  
Steffen Thiel ◽  
Veronika Rypdal ◽  
Ellen Dalen Arnstad ◽  
...  

Abstract Background To determine the serum levels of the lectin pathway proteins early in the disease course and 17 years after disease onset and to correlate the protein levels to markers of disease activity in participants from a population-based Nordic juvenile idiopathic arthritis (JIA) cohort. Additionally, to assess the predictive value of lectin pathway proteins with respect to remission status. Methods A population-based cohort study of consecutive cases of JIA with a disease onset from 1997 to 2000 from defined geographical areas of Finland, Sweden, Norway and Denmark with 17 years of follow-up was performed. Clinical characteristics were registered and H-ficolin, M-ficolin, MASP-1, MASP-3, MBL and CL-K1 levels in serum were analyzed. Results In total, 293 patients with JIA were included (mean age 23.7 ± 4.4 years; mean follow-up 17.2 ± 1.7 years). Concentrations of the lectin protein levels in serum were higher at baseline compared to the levels 17 years after disease onset (p ≤ 0.006, n = 164). At baseline, the highest level of M-ficolin was observed in systemic JIA. Further, high M-ficolin levels at baseline and at 17-year follow-up were correlated to high levels of ESR. In contrast, high MASP-1 and MASP-3 tended to correlate to low ESR. CL-K1 showed a negative correlation to JADAS71 at baseline. None of the protein levels had prognostic abilities for remission status 17 years after disease onset. Conclusion We hypothesize that increased serum M-ficolin levels are associated with higher disease activity in JIA and further, the results indicate that MASP-1, MASP-3 and CL-K1 are markers of inflammation.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1207.2-1207
Author(s):  
A. García Fernández ◽  
A. Briones-Figueroa ◽  
L. Calvo Sanz ◽  
Á. Andreu-Suárez ◽  
J. Bachiller-Corral ◽  
...  

Background:Biological therapy (BT) has changed the treatment and perspectives of JIA patients but little is known about when is the best moment to start BT and the impact of this prompt iniciation.Objectives:To analyze the response to BT of Juvenile Idiophatic Arthritis (JIA) patients according to the time when the BT was started.Methods:A retrospective, descriptive study was conducted on JIA patients followed up in a referal hospital that started BT up to 24 months after diagnosis from 2000 to 2018. Disease activity was measured, at 2 years after diagnosis, according to Wallace criteria for remission (absence of: active arthritis, active uveitis, fever, rash or any other manifestation attributable to JIA, normal CRP and ESR, PGA indicating no active disease) for at least 6 months.Results:55 JIA patients that started BT up to 24 months from diagnosis were analyzed. 69,1% were girls with a median age at diagnosis of 8 years old IQR(3-13), median age at the start of BT of 9 years old IQR(3-13). Regarding JIA categories: 25,5% were Oligoarticular Persistent (OligP), 18,2% Systemic JIA (sJIA), 16,4% Entesitis related Arthritis (ERA), 12,7% Psoriatic Arthritis (APso) and Polyarticular RF- (PolyRF-), 5,5% Oligoarticular Extended (OligE) and Polyarticular RF+ (PolyRF+), 3,6% Undifferentiated (Und). 20% of patients had uveitis during followup. Conventional DMARD (cDMARD) was indicated in 83,6% of patients (95,7% Methotrexate) at diagnosis [median 0 months IQR(0-2,3)]. At the end of followup (2 years) only 30,9% of patients continued with cDMARDs. The main causes of discontinuation were: adverse events (46,7%), remission (36,7%). TNF inhibitors were precribed in 81,8% of patients and 18,2% of patients recieved two BT during the first 2 years from diagnosis. 54,5% of BT were indicated during the first 6 months from diagnosis, 27,3% from 7 to 12 months, 12,7% from 13 to 18 months, 5,5% from 19 to 24 months.After 2 years from diagnosis, 78,2% of patients were on remission and 21,8% active. Among patients with active disease: 75% had arthritis, 16,7% had uveitis and 8,3% had both. There were no differences regarding disease activity among patients with uveitis and neither taking cDMARDs. Regarding JIA categories: 66,7% of OligE, 57,1% of PolyRF- and 57,1% of APso patients were active at 2 years from diagnosis when compared to the other categories (p=0.004).Patients on remission at 24 months from diagnosis started sooner the BT than active patients [CI 95% (0,46-8,29) p=0,029]. The time when the BT was started was correlated to the activity at 2 years (K= 0,294 p=0,029). When the BT was prescribed after 7,5months from diagnosis it was correlated, in a COR curve, with a higher probability of active disease at 2 years (S= 0,67 E= 0,63). There was a correlation, among patients on remission at 2 years, between prompt start of BT and less time to reach remission (K= -0,345 p=0,024). Patients with active disease at 2 years, regardless of moment of BT iniciation, required more BT during follow-up (p=0,002).Conclusion:Prompt iniciation of BT was correlated with a better outcome. JIA patients that started BT early after diagnosis had a higher probability of remission after 2 years. Starting BT after 7,5 months was correlated with a higher probability of active disease at 2 years. Active disease at 24 months was correlated with persistent active disease during follow-up.Disclosure of Interests:None declared


Rheumatology ◽  
2019 ◽  
Vol 58 (Supplement_4) ◽  
Author(s):  
Ashley Jones ◽  
Dannii Clayton ◽  
Gloria Nkhoma ◽  
Frances Sherratt ◽  
Matthew Peak ◽  
...  

Abstract Background Background Corticosteroids (CS) are widely used for rapid-action or induction treatment in children and young people (CYP) with juvenile idiopathic arthritis (JIA). Given a lack of evidence base on CS induction regimen for CYP with JIA, and since criteria for choosing CS are based on healthcare professional (HCP) preference, further research is needed (1). Methods A national electronic survey was undertaken among HCPs across the UK as part of the Steroid Induction Regimen for Juvenile Idiopathic Arthritis (SIRJIA) study. We aimed to establish the opinions of HCPs current practice regarding the clinical criteria for commencing CS treatment Results A total of 39 (24%) responses were received from 162 HCPs. These included 22 (56%) NHS consultants, five (13%) grid trainees, eight (21%) clinical nurse specialists and four other HCPs (10%). The most common treatments newly diagnosed JIA or a disease flare were intra-articular IACS or a combination of DMARDs and IAS (except for systemic JIA and oligoarticular JIA). The majority of HCPs 17 would treat new and flaring CYP the same with 53% choosing a different regime or not answering. The key criteria HCPs used for commencing CS and choosing route of administration were rapid induction of remission (31 (89%)), high disease activity (31 (89%)), severity of systemic JIA (30 (86%)) and level of inflammation (28 (80%)), see Table 1. The main determinants of route of administration was disease severity disease subtype. The majority of HCPs (52-72%) would consider entering CYP with JIA into a trial randomising to modes of administration. P14 Table 1 Reasons of CS Choice Number N = 39 Percentage % High Disease Activity 35 89.7 Rapid induction of remission 34 87.18 Severity of Systemic JIA 34 87.18 Level of inflammation 32 82.5 Severe Uveitis 30 76.92 JIA subtype 27 68.21 Targeting Specific Joints 26 66.67 Level of Disability 18 46.15 Level of pain 16 41.03 Long-standing Disease 11 28.1 Patient reluctance to take DMARDS 8 20.5 Conclusion The results from this national survey of clinical practice showed varying practices in the management of new CYP with JIA and those that are flaring. The majority of HCPs who completed this survey, indicated that they would be prepared to consider entering CYP into a trial that randomised to the four CS delivery methods. Conflicts of Interest The authors declare no conflicts of interest.


2009 ◽  
Vol 69 (01) ◽  
pp. 126-131 ◽  
Author(s):  
J Augustsson ◽  
M Neovius ◽  
C Cullinane-Carli ◽  
S Eksborg ◽  
R F van Vollenhoven

Objective:To investigate the effect of tumour necrosis factor (TNF) antagonist treatment on workforce participation in patients with rheumatoid arthritis (RA).Methods:Data from the Stockholm anti-TNFα follow-up registry (STURE) were used in this observational study. Patients with RA (n = 594) aged 18–55 years, (mean (SD) 40 (9) years) followed for up to 5 years were included with hours worked/week as the main outcome measure. Analyses were performed unadjusted and adjusted for baseline age, disease duration, Health Assessment Questionnaire (HAQ), 28-joint Disease Activity Score (DAS28) and pain score.Results:At baseline patients worked a mean 20 h/week (SD 18). In unadjusted analyses, significant improvements in hours worked/week could already be observed in patients at 6 months (mean, 95% CI) +2.4 h (1.3 to 3.5), with further increases compared to baseline at 1-year (+4.0 h, 2.4 to 5.6) and 2-year follow-up (+6.3 h, 4.2 to 8.4). The trajectory appeared to stabilise at the 3-year (+6.3 h, 3.6 to 8.9), 4-year (+5.3 h, 2.3 to 8.4) and 5-year follow-up (+6.6 h, 3.3 to 10.0). In a mixed piecewise linear regression model, adjusted for age, sex, baseline disease activity, function and pain, an improvement of +4.2 h/week was estimated for the first year followed by an added improvement of +0.5 h/week annually during the years thereafter. Over 5 years of treatment, the expected indirect cost gain corresponded to 40% of the annual anti-TNF drug cost in patients continuing treatment.Conclusion:Data from this population-based registry indicate that biological therapy is associated with increases in workforce participation in a group typically expected to experience progressively deteriorating ability to work. This could result in significant indirect cost benefits to society.


2008 ◽  
Vol 68 (5) ◽  
pp. 635-641 ◽  
Author(s):  
F H M Prince ◽  
M Twilt ◽  
R ten Cate ◽  
M A J van Rossum ◽  
W Armbrust ◽  
...  

Objective:We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes.Methods:At baseline we collected patient and disease characteristics of all Dutch patients with JIA who started treatment with etanercept. Disease activity was evaluated (at start of the study, after 3 months and then yearly) according to the JIA core set of the American College of Rheumatology paediatric definition for 30, 50 and 70% improvement (ACR Pedi 30, 50 and 70). Use of etanercept and concomitant drugs was monitored. Adverse events were recorded.Results:We included 146 patients with JIA with a median follow-up of 2.5 years per patient (range 0.3–7.3). JIA subtypes represented: 27% systemic, 8% polyarticular rheumatoid factor positive, 38% polyarticular rheumatoid factor negative, 19% oligoarticular extended, 3% enthesitis-related and 5% psoriatica. Most patients (77%) met the criteria of the ACR Pedi 30 in the first 3 months of treatment. For the majority of patients this improvement was sustained; 53 (36%) of all patients met the remission criteria. No other second-line agents were needed in 43 patients. Although patients with systemic JIA responded initially less to etanercept therapy than patients from other subtypes, those who did respond showed equal effectiveness in the long term. Serious adverse events rate was low (0.029 per patient year).Conclusions:Etanercept is effective and safe in JIA, even for a large proportion of the patients with systemic JIA. The greatest improvement occurred in the first 3 months of treatment, and was sustained for a long time in most patients (up to 75 months).


2017 ◽  
Vol 42 (1) ◽  
pp. 49-52
Author(s):  
Mohammed Mahbubul Islam ◽  
Mohammad Imnul Islam ◽  
Manik Kumar Talukdar ◽  
Mujammel Haque ◽  
Shahana A Rahman

About 50% of systemic onset juvenile idiopathic arthritis (sJIA) patients run a recalcitrant disease course and resistant to the conventional disease modifying anti inflammatoy drugs (DMARDs), ultimately resulting in permanent disability from joint destructions. Thalidomide has been reported as an effective and safe drug in the management of systemic JIA due to its immunomodulatory properties. This was an interventional study, aimed to evaluate the efficacy of thalidomide in refractory JIA patients. Twenty five systemic JIA patients who were refractory to conventional DMARDs were included in this study. These patients were prescribed thalidomide at a dose of 2-3mg/kg/day for 12 months. Efficacy of thalidomide was assessed by using Wallace criteria at 6th month and 12th month of thalidomide treatment. Active arthritis was improved in 55% and 73% of the patients at 6th and 12th month of thalidomide treatment respectively. Fever and rash subsided in 72.8% and 81.2% of patients respectively at 12th month of follow up. Hepatosplenomegaly and lymphadenopathy regressed in 100% of patients at 12th month follow up. ESR was also improved in 50% and 68.2% cases at 6th and 12th months respectively. Few minor side effects were observed like transient elevation of liver enzyme and somnolence in this study. It may be concluded that thalidomide is safe and effective in refractory JIA patients.


2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Mohammad H. Al-Hemairi ◽  
Shatha M. Albokhari ◽  
Mohammed A. Muzaffer

Introduction.Juvenile Idiopathic Arthritis (JIA) is the most common chronic arthritis in children. Our aim is to describe demographic, clinical, and laboratory characteristics and treatment of JIA patients followed up in Pediatric Rheumatology clinic in a tertiary center in Saudi Arabia.Methods. Medical records of all patients who are followed up between January 2007 and January 2015 were retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment.Results.Total patients were 82, males were 31 (37.8%), and mean age of JIA onset was 7.1 ± 3.6 yr. Mean follow-up duration was 2.67±1.6 yr. Systemic onset JIA (SoJIA) was the commonest (36.5%), followed by polyarticular in 29.2% and oligoarticular in 28%. Large and small joints are involved in 76 (92%) and 30 (36.6%), respectively. Main extra-articular feature was fever in 34 (41.4%). Uveitis was diagnosed in 7 (8.5%) and in 5 (21.7%) of oligoarticular JIA. Anemia was found in 49 (59.7%), high ESR in 45 (54.8%), and leukocytosis and thrombocytosis in 33 (40.2%). Positive ANA was found in 30 (36.5%) mainly in oligoarticular subtype as 12 (52%) patients (out of 23) had this positive test. 9 patients (10.9%) required NSAIDs only, 6 patients (7.3%) required NSAIDs and intra-articular steroids only, and 19 (23%) required NSAIDs, methotrexate, steroids, and biologics.Conclusion.SoJIA is the most common JIA subtype in our study. A population based rather than a single center study will give more details about JIA characteristics in Saudi Arabia


2021 ◽  
Author(s):  
Céline La ◽  
Phu Quoc Lê ◽  
Alina Ferster ◽  
Laurence Goffin ◽  
Delphine Spruyt ◽  
...  

Abstract IntroductionIn the management of juvenile idiopathic arthritis (JIA), there is a lack of diagnostic and prognostic biomarkers. This study assesses the use of serum calprotectin (sCal) as a marker to monitor disease activity, and as a classification and prognosis tool of response to treatment or risk of flares in patients with JIA. MethodsEighty-one patients with JIA from the CAP48 multicentric cohort were included in this study, as well as 11 non-pediatric healthy controls. An enzyme-linked immunosorbent assay (ELISA) method was used to quantify sCal with a commercial kit.ResultsPatients with an active disease compared to healthy controls and to patients with inactive disease showed an 8-fold and a 2-fold increased level of sCal respectively. sCal was found to be correlated with the CRP and even more strongly with the ESR. Evolution of DAS28 scores correlated well with evolution of sCal, as opposed to evolution of CRP. With regard to CRP, sCal could differentiate forms with active oligoarthritis from polyarthritis and systemic forms. However, sCal brought an added value compared to the CRP as a prognosis marker. Indeed, patients with active disease and reaching minimal disease activity (according to JADAS) at 6 months following the test had higher sCal levels, while patients with inactive disease had higher sCal levels if a flare was observed up to 3 to 9 months following the test.ConclusionsThis study confirms the potential uses of serum calprotectin as a biomarker in the diagnosis and follow-up of JIA.


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.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 935.2-936
Author(s):  
S. Lanni ◽  
O. De Lucia ◽  
S. Orsi ◽  
S. Costi ◽  
G. Beretta ◽  
...  

Background:The ankle is one of the most commonly affected sites in juvenile idiopathic arthritis (JIA). This region has a complex anatomical structure owing to the presence of multiple joint recesses and surrounding tendons. While the prognostic value of ultrasound (US)-detected arthritis has been investigated in recent studies, the role of tenosynovitis in JIA remains still unexplored.Objectives:To investigate: 1) US features of ankle involvement in JIA at disease onset; 2) the predictive value of US-detected tenosynovitis in ankles with clinically active disease of children with new-onset JIA.Methods:The clinical charts of all consecutive patients with new-onset JIA between May 2018 and January 2020 at study centres (Policlinico and G.Pini Hospitals of Milan) and with clinically active ankle disease among the joints affected were reviewed retrospectively. Data on ankle US assessment were retrieved and patients were then stratified as follows: 1) patients with detection on US of isolated arthritis in at least one of the joint recesses of the ankle region; 2) patients with detection on US of tenosynovitis in at least one of the tendon compartments of the ankle irrespective of the presence of concomitant arthritis. For each of these two categories, estimation of patients who were able to achieve clinical disease remission at 12 months since disease onset was evaluated.Results:Twenty-seven new-onset JIA patients were found to have clinical involvement of the ankle among the joints affected. Nine of them (33.3%) showed on US isolated arthritis of the ankle, whereas US-detected tenosynovitis was found in 18 (66.7%) patients. The amount of patients who were able to achieve disease remission at 12-months was the same (66.7%) for both patients with and without US-detected tenosynovitis in the ankle (12/18 and 6/9 patients, respectively). In patients with US-detected tenosynovitis and clinical disease remission at 12 months, the lateral tendon compartment (LTC) was the tendon site more frequently affected by pathology (75.0%). Patients with US-detected tenosynovitis that did not achieve clinical disease remission at follow-up had the highest frequency of tendon pathology on US in the medial tendon compartment (MTC) (83.3%). The anterior tendon compartment was the less frequently affected tendon compartment of the ankle in all patients (33.3% in both patients with and without clinical remission of disease at the 12-months follow-up visit).Conclusion:US-detected tenosynovitis of the ankle is a common finding in patients with new-onset JIA with clinically ankle disease activity and is more frequent than the detection on US of isolated arthritis. The MTC and LTC are the tendon compartments more commonly affected on US. The detection on US of tenosynovitis at disease onset in ankles with clinical disease activity did not seem to affect the change to achieve the overall clinical disease remission compared to patients without tendon pathology but with joint disease in the ankle region.Disclosure of Interests:None declared


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