scholarly journals Juvenile Idiopathic Arthritis in Adults: Long-Term Observation of Ukrainian Patients

2017 ◽  
Vol 23 (1) ◽  
Author(s):  
Marta Dzhus

The assessment of long-term outcome of functional disability and disease activeness in adult patients with juvenile idiopathic arthritis appears to be complicated due to the absence of a unified approach to the classification and estimation of disease activeness, as well as the loss of supervision over a patient because of remission or his/her transition from pediatric to adult rheumatic service. The objective of the research was to determine how adults with the history of juvenile idiopathic arthritis fulfill the classification criteria for adult rheumatic diseases, as well as to assess activeness of these diseases, the degree of functional disorders, and social activeness of patients in Ukraine. Materials and methods. Patients with juvenile idiopathic arthritis older than 18 years and with more than 3 years of disease duration living in different parts of Ukraine were included into the study. Data regarding sociodemographic features, fulfillment of adult classification criteria, Health Assessment Questionnaire, articular and extra-articular Juvenile Arthritis Damage Index and disease activity were analyzed.Results. We observed 122 adult patients with the history of juvenile idiopathic arthritis irrespective of the presence of active inflammation at the moment of the examination. This group included patients from different regions of Ukraine diagnosed with juvenile idiopathic arthritis during 1984-2013. An adult rheumatologist examined all patients and the diagnosis was revised according to the adult classification of rheumatic diseases. Typical diagnostic criteria for rheumatoid arthritis were estimated in 32.8% of patients, ankylosing spondylitis – in 31.1% of patients, undifferentiated arthritis – in 13.9% of patients, Still’s disease – in 4.9% of patients, psoriatic arthritis – in 0.8% of patients, steady clinical laboratory remission – in 16.5% of patients. Most patients (81.8%) with rheumatoid factor positive polyarticular juvenile idiopathic arthritis fell under rheumatoid arthritis criteria in adulthood, and in 85% of patients with enthesitis-related arthritis as well as 53.8% of patients with extended oligoarthritis ankylosing spondylitis developed in adulthood. 68.8% of patients with systemic juvenile idiopathic arthritis, 68% of patients with rheumatoid factor negative polyarthritic subtype and 55% of patients with enthesitis-related arthritis had disability and incapacitation. Minimal disorders of the patients’ general condition according to the Health Assessment Questionnaire in adult age were found in most subtypes of juvenile idiopathic arthritis classified according to the International League of Associations for Rheumatology (extended and persistent oligoarthritis, rheumatoid factor positive polyarthritis, systemic subtype); moderate disorders of the general condition were found in enthesitis-related arthritis and rheumatoid factor negative polyarthritis. Side effects of juvenile idiopathic arthritis according to the articular Juvenile Arthritis Damage Index included severe articular damage being most frequently found in systemic and rheumatoid factor positive polyarthritis subtypes of juvenile idiopathic arthritis, while side effects of juvenile idiopathic arthritis according to the extra-articular Juvenile Arthritis Damage Index included extra-articular damage being found in systemic and rheumatoid factor negative polyarthritis subtypes of juvenile idiopathic arthritis, that was confirmed by the assessment of physical health according to the Short Form Health Survey-36, which was the worst in patients with systemic (40.3±12.6) and rheumatoid factor negative polyarthritis (38.9±9.4) subtypes of juvenile idiopathic arthritis.Conclusions. Further research of remote consequences of juvenile idiopathic arthritis in adult age and long-term observation of such patients require a detailed study to improve diagnostics and provide adequate treatment of rheumatic diseases with juvenile onset in adult age.

2017 ◽  
Vol 24 (3) ◽  
Author(s):  
M. Dzhus ◽  
H. Mostbauer ◽  
T Karasevska ◽  
O. Ivashkivsky

To assess the long-term effects of juvenile idiopathic arthritis in adulthood, unified diagnostic methods for articular and extra-articular lesions should be used which depend on the juvenile idiopathic arthritis variants, the disease activity and treatment. The objective of the research was to compare the clinical manifestations in adult patients with different juvenile idiopathic arthritis-specific immunogenic markers and to evaluate their impact on the long-term articular and extra-articular damage.Materials and methods. We observed 132 young patients with different juvenile idiopathic arthritis variants. According to genetic/immunological markers the following groups were formed:  Group I - 38 positive human leukocyte antigen B27 patients; Group II - 13 positive antinuclear antibody patients; Group III - 26 positive rheumatoid factor/anti-cyclic citrullinated peptide patients and Group IV - 55 patients with all negative markers. Long-term effects of juvenile idiopathic arthritis were estimated by the articular juvenile arthritis damage index (JADI-A) and the extra-articular juvenile arthritis damage index (JADI-E). Descriptive statistics, the Student’s T-test, the Fisher’s exact test and Mann-Whitney U-test were performed.Results. 70 women and 62 men with the disease duration of 13.6±9.3 years at the age of 24.3±8.3 years were included into the study: 12 (9.1%) patients with positive rheumatoid factor polyarthritis, 30 (22.7%) patients - with negative rheumatoid factor polyarthritis, 32 (24.2%) patients with persistent oligoarthritis, 19 (14.4%) patients with extendent oligoarthritis, 20 (15.2%) patients with entesitis-related arthritis and 19 (14.4%) patients with systemic arthritis; there were no patients with psoriatic arthritis. There were no differences between groups in age, disease-modifying antirheumatic drug cumulative dose, mean dose of prednisolone and quality of life according to the SF-36. In Group I, the delay in the diagnosis was more than one year (18.6±24.2 months). In this group, less painful (p<0.005) and deformed (p<0.01) joints as compared to Group ІІІ, and higher levels of the ESR and C-reactive protein as compared to Group ІV were found, although the Juvenile Arthritis Disease Activity Score index in childhood was lower (p<0.005) as compared to Group ІІ. They received a lower cumulative dose of the glucocorticoids as compared to Group II (p<0.01), respectively. They had lower (p<0.01) JADI-E as compared to Group II (1.31 ± 1.49) and lower (p <0.01) JADI-A as compared to Group III. In Group III, the diagnosis was made the fastest in comparison with other groups (6.4±8.4 months, p<0.05); more painful joints (p <0.05) and ankylosis (p<0.05) were observed as compared to Group I, JADI-A was significantly higher (p<0.05) in Group III as compared to Group I. The most pronounced JADI-A was found in Group III, while in Group I and Group II, this index was the lowest. JADI-E was the most pronounced in Group II, and the most favorable course was found in Group І and Group ІІІ (p<0.05).Conclusions. Presence of anti-cyclic citrullinated peptide/rheumatoid factor in adults with juvenile idiopathic arthritis has negative impact on joint damage (JADI-A) indicating the need for aggressive therapy in both childhood and adulthood. Presences of antinuclear antibodies are associated with more often extra-articular damages in adulthood as compared to other groups.


2009 ◽  
Vol 36 (8) ◽  
pp. 1730-1736 ◽  
Author(s):  
ANKIT MAHENDRA ◽  
RAMNATH MISRA ◽  
AMITA AGGARWAL

Objective.A Th1 biased immune response in synovial fluid has been reported in children with polyarticular and extended oligoarticular-type juvenile idiopathic arthritis (JIA). We investigated T cell phenotypes including Th1, Th2, Th17, and Treg with emphasis on Th17 and Treg, in order to differentiate cytokines in the enthesitis-related arthritis (ERA) form of JIA.Methods.The frequencies of Th1, Th2, Th17, and Treg cells were determined by flow cytometry in peripheral blood (PB) and synovial fluid from patients with ERA and healthy subjects. Levels of interleukin 1ß (IL-1ß), IL-6, IL-21, IL-23, and transforming growth factor ß (TGF-ß), cytokines that influence Th17 lineage cells, were measured in paired plasma and synovial fluid (SF) samples by ELISA. Frequencies are expressed as percentages and cytokine levels as pg/ml.Results.There were no differences in blood samples in the frequency of Th1, Th2, Th17, and Treg cells between patients and controls. In paired samples, the median frequency of CD4+IFN-γ+ (20.49 vs 4.03; p < 0.005) and CD4+IL-17+ (2.27 vs 0.57; p < 0.01) cells was significantly higher in SF compared to PB, respectively; whereas the frequency of CD4+IL-4+ (1.79 vs 2.29; p < 0.04) cells was significantly reduced in the SF compared to PB. There was no difference in the frequency of regulatory T cells. Patients receiving methotrexate had fewer Th2 cells, whereas the Childhood Health Assessment Questionnaire score had a negative association with the frequency of Treg. Median levels of IL-1ß (p < 0.008), IL-6 (p < 0.0001), and IL-17 (p < 0.0001) were higher in SF than in plasma and levels of TGF-ß were lower (p < 0.001). Levels of IL-21 were similar in SF and plasma, whereas IL-23 was undetectable.Conclusion.In patients with ERA, peripheral blood Th1, Th2, Th17, and Treg cells were unchanged, but Th1 and Th17 cells were increased and Th2 cells were reduced in the SF compared to blood. Elevated IL-1ß and IL-6 in SF may be responsible for increased Th17 cells.


2020 ◽  
Vol 15 (4) ◽  
pp. 86-90
Author(s):  
V.K. Sevostyanov ◽  
◽  
A.O. Davydov ◽  
A.S. Novikov ◽  
A.I. Polukhina ◽  
...  

Objective. To evaluate the frequency, nature, and structure of therapy for uveitis associated with juvenile idiopathic arthritis (JIA) using the data from the Moscow city register of children with rheumatic diseases. Patients and methods. We analyzed the data of 117 patients with JIA and uveitis aged between 3 and 17 years residing in Moscow. Results. JIA-associated uveitis was diagnosed in 11% of children with JIA. It was more common in females than in males (13.1% vs 7.3% respectively). More than half of patients with uveitis (53.8%) had oligoarticular JIA, whereas 34.2% of patients had polyarticular RF-seronegative JIA. The majority of children (85.5%) received basic therapy, primarily with methotrexate (97% of them). Biologicals were administered to 64.1% of patients; of them, 68% received adalimumab. Sixty-one percent of children had bilateral eye lesions. In 41.5% of patients, the disease started with joint syndrome, while 46.3% of patients had both joint syndrome and eye lesions at onset and 12.2% of children initially had eye lesions. Remission of uveitis was registered in 75.6% of patients. Conclusion. Our results are consistent with both Russian and foreign data. Early diagnosis of uveitis and its timely treatment will decrease the incidence of complications in patients with JIA (including complete vision loss) and reduce the burden of disability for patients, their families, and the state. Key words: biological therapy, rheumatoid uveitis, rheumatic diseases in children, juvenile arthritis


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).


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
A Fazaa ◽  
F Ben Messaoud ◽  
S Miladi ◽  
L Souabni ◽  
K Ouenniche ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children and is one of the major causes of morbidity and physical disability. Due to frequent absences, children with chronic health impairments are also often confronted with educational difficulties. The aims of this study were to assess the impact of JIA on children’s schooling and to determine the factors that influence their school level. Methods This is a cross-sectional study including patients with JIA (ILAR criteria). A detailed questionnaire was completed for each participant by interviewing them or their parents as well as by information obtained from their medical records. Collected data included age, sex, subtype of JIA, disease duration, level of disability according to the Childhood Heath Assessment Questionnaire (CHAQ), visual analogue scale for patient’s overall assessment of disease activity (VASOA), duration of morning stiffness, tender joint counts (TJCs), swollen joint counts (SJCs), erythrocyte sedimentation rate (ESR), C-Reactive Protein (CRP), Disease Activity Score (DAS28) for polyarticular and oligoarticular JIA, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) for Enthesitis-related arthritis. Medications used for JIA treatment were also documented. Data on the school performance of patients and their siblings were obtained using telephone interviews (educational level, absenteeism, school delay by repetition, drop-out). Results A total of 43 patients with JIA were included, 25 female and 18 male, with a mean age of 26 years [12–51] and a mean disease duration of 237 months (5–496). The average age of the onset of the disease was 7.4 years [1.5–16]. The most common subtype was rheumatoid factor-positive polyarthritis (n = 18) followed by systematic (n = 8), oligoarticular (n = 8), rheumatoid factor-negative polyarthritis (n = 5) and Enthesitis-related arthritis (n = 4). The mean DAS28 was 3.02 [0.76 – 5.55] and the median CHAQ was 0.66 [0–3]. Twenty-nine of the children were receiving corticosteroid. Disease-modifying anti-rheumatic drugs were used by 38 of the 43 patients: methotrexate (n = 27), sulfasalazine (n = 8), leflunomide (n = 7), biotherapies (n = 16). Twenty patients had complications: Hip arthritis (n = 18), growth stunting (n = 14), uveitis (n = 5). Joint replacement was required in 11 cases. Four patients were illiterate, 14 had dropped out of school, 24 reported repeated absences due to illness. A year of schooling was repeated by 50.85% of patients. Eleven out of 32 patients over the age of 20 had an university level. Almost 80% of patients were exempted of physical education. There were no significant associations between the school-related problems, the socio-demographic characteristics and the various parameters of clinical and biological activity studied. Conclusion Our study suggested that JIA negatively affects schooling of children. More studies, with a larger sample of children, are needed to identify the variables associated with school failure in order to ensure the proper management of these patients and to increase their academic performance.


2018 ◽  
Vol 77 (7) ◽  
pp. 996-1002 ◽  
Author(s):  
Jens Klotsche ◽  
Kirsten Minden ◽  
Martina Niewerth ◽  
Gerd Horneff

ObjectivesTo determine the reasons of methotrexate (MTX) discontinuation, frequency of adverse events (AE) and whether the time in inactive disease before MTX withdrawal disease is associated with the risk of disease flare.MethodsPatients with juvenile idiopathic arthritis (JIA) beginning treatment with MTX were prospectively observed in the national JIA biologic register Biologika in der Kinderrheumatologie/Biologics in Paediatric Rheumatology and its follow-up register Juvenile arthritis Methotrexate/Biologics long-term Observation. Inactive disease was defined by a clinical Juvenile Arthritis Disease Activity Score ≤1, flare after MTX discontinuation by reoccurrence of at least moderate disease activity or restart of treatment with a disease-modifying antirheumatic drug .ResultsMTX treatment was initiated in 1514 patients after a mean disease duration of 2.1 years (SD=2.8). 40% of the patients experienced oligoarticular onset of JIA. MTX was discontinued in 982 (64.9%) patients. Ineffectiveness (36.9%) and achieving inactive disease (32.1%) were the most common reasons. Among the latter (n=316), 184 (58.2%) patients experienced a flare on follow-up. The likelihood of a flare was a function of time in inactive disease prior to MTX discontinuation (HR 0.95; 95% CI 0.92 to 0.97). Patients with inactive disease for longer than 12 months had a significantly lower flare rate (58 of 119, 48.7%; HR 0.48; 95% CI 0.34 to 0.69). The most frequently reported AE was MTX intolerance, including nausea, aversion and vomiting, accounting for 441 events (13.0 events/100 exposure years) in 307 (20.3%) patients.ConclusionsPatients who spent at least 12 months in inactive disease before MTX discontinuation had a significantly lower flare rate.


2009 ◽  
Vol 36 (3) ◽  
pp. 628-634 ◽  
Author(s):  
ALESSANDRA MAGNANI ◽  
ANGELA PISTORIO ◽  
SILVIA MAGNI-MANZONI ◽  
ALESSANDRA FALCONE ◽  
GIUSEPPINA LOMBARDINI ◽  
...  

Objective.To investigate whether the achievement of inactive disease in the first 5 years predicts a more favorable outcome of children with juvenile idiopathic arthritis (JIA).Methods.We reviewed clinical charts of 123 patients who started taking methotrexate, were followed for at least 5 years, and received a yearly assessment in the first 5 years. At each yearly visit, the presence of inactive disease was assessed. Patients were divided into 3 groups: (1) patients who never reached inactive disease; (2) patients who reached inactive disease in only 1 visit; and (3) patients who reached inactive disease in ≥ 2 visits. Outcome was evaluated after 6 to 18 years (median 7.1 yrs) by assessing the following clinical measures: restricted joint count, Childhood Health Assessment Questionnaire (CHAQ), Juvenile Arthritis Damage Index (JADI), and Poznanski score of radiographic damage.Results.In the first 5 years, 62 patients (50.4%) were noted to have active disease at their yearly visit, 40 patients (32.5%) were noted to have inactive disease only once, and 21 patients (17.1%) were noted to have inactive disease in ≥ 2 visits. Patients who achieved inactive disease 1 or more times had lower restricted joint count (p = 0.007) and JADI-Articular score (p = 0.004) at last followup visit than those who never reached such a state. A similar trend, although not significant, was observed for CHAQ and Poznanski score of radiographic damage.Conclusion.Attainment of the state of inactive disease at least once in the first 5 years was found to be associated with less longterm joint damage and with a trend toward less functional impairment.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1395.2-1395
Author(s):  
A. Migowa ◽  
R. Odhiambo ◽  
J. Orwa ◽  
J. Shah

Background:Pediatric rheumatic diseases are chronic illnesses that impart a significant disease burden upon societies (1-3). Determination of the burden and clinical characteristics of these diseases is a critical first step to improving access to care and optimizing use of existing health systems for the well-being of these patients (4-6). A pediatric rheumatology registry is critical in defining the spectrum, clinical characteristics, outcomes and responses of various interventions for pediatric rheumatic diseases. Given that none exists in Kenya, the Kenya Pediatric Rheumatology Registry (KAPRI) registry offers a platform to generate this much needed data in sub-Sahara Africa.Objectives:Our objective was to determine the baseline patient characteristics, clinical features and outcomes of the Juvenile Idiopathic Arthritis (JIA) patients assessed at the Aga Khan University Medical College East Africa who were enrolled into the KAPRI registry from inception in March 2019 to December 2020.Methods:All patients with an ICD 10 M code diagnosis of Juvenile Arthritis were selected from the KAPRI registry database. Age, gender, laboratory and clinical features at diagnosis and treatment options offered were extracted from the database. A further detailed chart review was undertaken to determine the proportion of patients who achieved remission or minimally active diseases.Results:Among the 207 patients enrolled thus far, 16 (7.7%) were diagnosed to have JIA. Majority of the patients were females (75%; n=12) with a mean age of 7 years and 3 months (Range:1 year – 13 years 7 months).All patients had joint pain and swelling as the initial presenting complaints. Majority of the patients had polyarticular JIA (75%, n=12). The other 4 patients were oligoarticular (n=2) and systemic JIA (n=2). Among the polyarticular JIA patients (n=12), only 3 (25%) were rheumatoid factor (RF) positive and 1 was antinuclear antibody (ANA) positive. The oligoarticular and systemic JIA patients were all negative for antinuclear antibody, rheumatoid factor and cyclic citrullinated peptide antibodies (anti-ccp). Seven patients (43.8%) required biological therapies; tocilizumab (n=2: systemic JIA), adalimumab (n=2: polyarticular JIA), etanercept (n=2: polyarticular JIA) and tofacitnib (n=1: polyarticular JIA). One patient with systemic JIA on tocilizumab developed herpes simplex which was successfully managed with oral acyclovir. All the other patients did not develop any infections, allergic reactions or any other untoward events as adverse outcomes following the use of biological therapies. Five patients have attained remission as illustrated in the Table 1 below. Two patients have been lost to follow up.Conclusion:Seronegative polyarticular JIA was the predominant form of JIA observed with a predilection to affect more girls and boys. Over a period of 2 years, remission has been attained among 31.25% of the patients (5 of 16) with use of synthetic disease modifying anti-rheumatic drugs and biological therapies.References:[1]Moorthy LN, Peterson MG, Hassett AL, Lehman TJ. Burden of childhood onset arthritis. Pediatr Rheumatol Online J. 2010;8:20.[2]Minden K, Niewerth M, Listing J, et al. The economic burden of juvenile idiopathic arthritis-results from the German paediatric rheumatologic database. Clin Exp Rheumatol. 2009;27(5):863–9.[3]Bernatsky S, Duffy C, Malleson P, Feldman DE, St Pierre Y, Clarke AE. Economic impact of juvenile idiopathic arthritis. Arthritis Rheum. 2007;57(1):44–8.[4]Migowa A, Colmegna I, Hitchon C, Were E, Ng’ang’a E, Ngwiri T, et al. The spectrum of rheumatic in-patient diagnoses at a pediatric hospital in Kenya. Pediatric Rheumatology (2017)[5]Woolf AD. The bone and joint decade 2000–2010. Ann Rheum Dis. 2000; 59(2):81–2.[6]Scott C, Webb K. Pediatric rheumatology in sub-Saharan Africa. Rheumatology (Oxford). 2014;53(8):1357–8.Disclosure of Interests:None declared


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