Manifestation Of Juvenile Idiopathic Arthritis Research Articles

Extraarticular manifestations of juvenile idiopathic arthritis and their impact on health-related quality of life.

The objective of this study is to investigate extraarticular manifestations (EAMs) in patients with juvenile idiopathic arthritis (JIA) and assess their impact on health-related quality of life (HRQoL) among these patients. This cross-sectional analytic study was carried out on 117 patients with JIA. EAMs were identified clinically by history and examination. Sicca symptoms, peripheral neuropathy, enthesitis, and skin lesions were picked up during clinical examination. Pulmonary involvement was evaluated by high-resolution CT chest. Patients were assessed by abdominal ultrasonography to assess the size of liver and spleen. Atlantoaxial subluxation was evaluated by cervical spine x-rays. Patients were evaluated by Pediatric Quality of Life Inventory-4 (PedsQL-4) and PedsQL-3 arthritis module. The median age of patients was 14years with a median disease duration 4years, 82.9% were females. Of the studied 117 JIA patients, 85 patients (72.6%) had at least one EAM. Persistent fatigue (51.3%) was the most prevalent EAM, followed by recurrent skin rash (16.2%), enthesitis (15.4%), recurrent fever (13.7%), and uveitis (12%). Patients with EAMs scored significantly lower in physical functioning (p = 0.001), emotional functioning (p < 0.001), social functioning (p = 0.005), and school functioning (p = 0.001). Regarding PedsQL arthritis module, patients with EAM had also significantly lower scores than did patients without EAM on the domains of pain and hurt (p < 0.001), daily activities (p = 0.008), and worry (p = 0.001). EAMs are prevalent among JIA patients and have a negative impact on their HRQoL. So, early identification and treatment are highly recommended. Key Points • A large percentage of JIA patients experienced at least one extraarticular manifestation (EAM). • Persistent fatigue and recurrent skin rash are the most prevalent EAMs in JIA patients. • JIA patients with EAMs have worse scores in almost all domains of HRQoL.

P010 To establish compliance with British Society for paediatric and adolescent rheumatology (BSPAR) and the Royal College of Ophthalmology (RCOphth) guidelines for screening for uveitis in juvenile idiopathic arthritis (JIA) at a District General Hospital: a retrospective audit

Abstract Background/Aims JIA associated Uveitis (JIA-U) is most common extra-articular manifestation of JIA. The prevalence of uveitis in JIA is approximately 8-30%, but may be as high as 45-57% in young patients with oligoarticular disease. Uveitis is predominantly asymptomatic and requires slit-lamp examination for diagnosis. Up to 67% of JIA-U cases develop ocular complications such as cataracts (20%), glaucoma (19%), band keratopathy (16%), macular oedema and irreversible permanent visual impairment. The primary objective of our audit was to establish compliance of our service with BSPAR/RCOphth 2006 uveitis screening guidelines and therefore reduce the risk of visual complications in children with JIA. Methods A retrospective review of clinical records of children with rheumatological conditions attending our clinic was performed over five-year period. A total of 42 patients with JIA were identified. Data was collated using excel sheet audit tool. Results Results were analysed in accordance with the four major guideline domains as below. 1. Initial Screening exam within 6 weeks of referral: 40 out of 42 children screened within six weeks with 95% compliance rate. 2. Symptomatic patients seen within one week of referral: 4 out of 13 children seen in time with 30% compliance. 70% non-compliance was due to non-availability of ophthalmology appointment. 3. Uveitis may flare on stopping Methotrexate/DMARD’s. Patients should get screened two monthly for six months: 9 out of 13 children were screened with 70% compliance. 15% non-compliance was due to non-availability of ophthalmology appointment. 4. Ongoing screening at two-monthly intervals from onset of arthritis for six months, then 3-4 monthly: 15 out of 20 children screened with 75% compliance. 20% non-compliance was due to non-availability of ophthalmology appointment. Conclusion Practice at our centre as judged against the BSPAR/RCOphth guidelines was suboptimal. The main cause of non-compliance was due to staffing issue, rather than lack of healthcare professional training, delay in onward referral or non-attendance of patients. Non-availability of regular ophthalmology cover with cross-site commitment stretched the uveitis screening service at our centre. We presented this audit in our trust paediatric governance meeting. As a result of this audit, the frequency of paediatric ophthalmology clinics was increased to one a week. The paediatric nurse specialist facilitated liaison with the ophthalmology department and improved compliance with attendance. Patient database on shared drive was regularly updated with separate column for ophthalmology follow-up. The ophthalmologists were granted access to this database detailing clinic attendance and diagnosis. Parent education, reinforced with appropriate literature and a child friendly clinic environment improved awareness and reduces non-attendance. Combined clinics with ophthalmology are being explored. We plan to re-audit the service in near future to assess the impact after above remedial measures. A similar multi-site nation-wide audit may help identify and address local and national concerns. Disclosure K.K. Garg: None. T. Walton: None. A. Holliday: None.

Perinatal events and development of juvenile idiopathic arthritis-associated uveitis

Uveitis is one of the most common manifestations of juvenile idiopathic arthritis (JIA). Currently, JIA is associated with decreased gut microbiota diversity. Studies confirm that perinatal events can cause aberrant microbial colonization. The objective of this study is to determine if JIA is associated with perinatal events with a secondary focus on these variables to the development of JIA-uveitis. 369 patients with strabismus (n = 200) or JIA (n = 196) were included in the study. Completed surveys (JIA 37; strabismus 18) collected data about birth route, pregnancy and labor complications, JIA medications, and the presence of eye disorders. Analysis indicates that there is no relationship between JIA development and the perinatal events investigated. Similarly, no significance was found between JIA-uveitis and birth route or labor complications. Pregnancy complications, namely gestational diabetes (GD), were statistically higher in the JIA group with uveitis compared to JIA without uveitis. The data from this survey study showed that JIA-uveitis was highly associated with pregnancy complications, particularly with GD. However, no statistically significant association was found between JIA and route of delivery, labor complications, or pregnancy complications. Further studies are needed to understand the ways that GD interrelates with the development of uveitis in JIA patients.

Impact of physiotherapy on orofacial manifestations of juvenile idiopathic arthritis

BackgroundPhysiotherapy appears as a promising therapy option for patients with Juvenile Idiopathic Arthritis (JIA) [1, 2], but the effects of physiotherapy and jaw exercises on JIA-related orofacial symptoms remain unknown [3]. The aim of this proof-of-concept study was to assess the impact of orofacial physiotherapy and home-exercise programs in patients with JIA and temporomandibular joint (TMJ) involvement.MethodsTwelve patients with JIA and TMJ involvement received a treatment of physiotherapy, complemented by prescribed home exercises spanning over eight weeks. Orofacial symptoms and dysfunction were monitored pre-treatment, during treatment, after treatment, and at a three-months follow-up.ResultsOrofacial pain frequency and intensity significantly decreased during the course of the treatment (p = 0.009 and p = 0.006), with further reductions observed at the three-month follow-up (p = 0.007 and p = 0.002). During treatment, the mandibular function improved significantly in terms of maximal mouth opening capacity, laterotrusion, and protrusion.ConclusionsThis proof-of-concept study shows favourable effects of physiotherapy and home excercises in the management of JIA-related orofacial symptoms and dysfunctions.

Presence of uveitis as indicator of juvenile idiopathic arthritis severity: results of a retrospective cohort study

BACKGROUND: Juvenile idiopathic arthritis is the most common rheumatic disease in children. A frequent extra-articular manifestation of juvenile idiopathic arthritis is uveitis, which is a serious clinical diagnostic problem in routine pediatric practice. Among the known risk factors for uveitis are the early age of the juvenile idiopathic arthritis onset, oligoarticular subtype, seropositivity by antinuclear factor.&#x0D; AIM: to evaluate the influence of presence of uveitis on the course of juvenile idiopathic arthritis.&#x0D; MATERIALS AND METHODS: A single-center retrospective study included 520 patients with uveitis. The analysis was carried out among patients who developed (n = 116) and did not develop (n = 404) uveitis. The minimum follow-up period was 2 years, for patients who did not develop uveitis.&#x0D; RESULTS: Uveitis was diagnosed in 116 (22.3%) children with juvenile idiopathic arthritis. Most often, uveitis occurred in patients with oligoarthritis and psoriatic arthritis. When comparing the features of the articular status of patients with juvenile idiopathic arthritis who developed and did not develop uveitis, a lower frequency of involvement of the cervical spine, temporomandibular, shoulder, elbow, wrist, proximal and distal interphalangeal joints, hip, talus-heel joint, as well as a smaller number of active joints in children with uveitis was found. Patients with uveitis received methotrexate therapy more often, cumulative doses of corticosteroids were lower, the frequency of prescribing genetically engineered biological drugs was approximately the same in both groups. Remission of arthritis was achieved more often, but the proportion of children who developed an exacerbation was higher. When calculating the risk factors of uveitis by binary logistic regression, it was found that the main predictors of uveitis were oligoarthritis, the number of active joints 8, seropositivity by antinuclear factor and recurrent course of arthritis. The difference in the frequency of achieving remission may be due to more aggressive systemic therapy in the presence of uveitis.&#x0D; CONCLUSIONS: Children with uveitis have a more severe course of juvenile idiopathic arthritis and may require more aggressive immuno-suppressive therapy. Further studies are required to determine the prognostic role of uveitis in the course and outcomes of juvenile idiopathic arthritis.

Incidence of Orofacial Manifestations of Juvenile Idiopathic Arthritis From Diagnosis to Adult Care Transition: A Population-Based Cohort Study.

To estimate the cumulative incidences of orofacial conditions related to temporomandibular joint (TMJ) juvenile idiopathic arthritis (JIA) between diagnosis in childhood to transition into adult care, and to identify features in JIA associated with TMJ involvement. A population-based cohort analysis was conducted of patients with JIA involving longitudinal data on orofacial health from 2000 to 2018. Regardless of TMJ status, the patients were referred to the Regional Specialist Craniofacial Clinic of Western Denmark for routine orofacial examinations. Data collection included information about disease-specific background characteristics, TMJ involvement, JIA-induced dentofacial deformity, and orofacial symptoms and dysfunction. A total of 613 patients were followed up with a mean clinical TMJ observation time of 4.0 years. From JIA onset to transition into adult care, the cumulative incidence of patients with JIA involvement of the TMJ was 30.1%. Furthermore, 20.6% of the cohort had developed arthritis-induced dentofacial deformity. A substantial proportion of the cohort experienced several events with orofacial symptoms (23.5%) and dentofacial dysfunction (52%). Young age at diagnosis (<9 years), female gender, and antinuclear antibody positivity were significantly associated with TMJ involvement. Orofacial signs and symptoms were frequent findings in children and adolescents with JIA. TMJ involvement was seen in 30.1% of the cohort; and 20.6% of the total cohort developed JIA-related dentofacial deformity before transition into adult care. This is the first population-based study in the era of available biologic treatments to document these frequent orofacial complications in children with JIA.

Management of temporomandibular joint arthritis in children and adolescents: An introduction for orthodontists.

Juvenile idiopathic arthritis (JIA) is the most common inflammatory rheumatic disease of childhood. JIA can affect any joint and the temporomandibular joint (TMJ) is one of the joints most frequently involved. TMJ arthritis impacts mandibular growth and development and can result in skeletal deformity (convex profile and facial asymmetry), and malocclusion. Furthermore, when TMJs are affected, patients may present with pain at joint and masticatory muscles and dysfunction with crepitus and limited jaw movement. This review aims to describe the role of orthodontists in the management of patients with JIA and TMJ involvement. This article is an overview of evidence for the diagnosis and treatment of patients with JIA and TMJ involvement. Screening for the orofacial manifestation of JIA is important for orthodontists to identify TMJ involvement and related dentofacial deformity. The treatment protocol of JIA with TMJ involvement requires an interdisciplinary collaboration including orthopaedic/orthodontic treatment and surgical interventions for the management of growth disturbances. Orthodontists are also involved in the management of orofacial signs and symptoms; behavioural therapy, physiotherapy and occlusal splints are the suggested treatments. Patients with TMJ arthritis require specific expertise from an interdisciplinary team with members knowledgeable in JIA care. Since disorders of mandibular growth often appear during childhood, the orthodontist could be the first clinician to see the patient and can play a crucial role in the diagnosis and management of JIA patients with TMJ involvement.

Juvenile idiopathic arthritis presenting as diffuse alveolar hemorrhage at onset: A case series.

The etiology of diffuse alveolar hemorrhage (DAH) in childhood is often unknown, and it may be an early manifestation of rheumatic disease. Juvenile idiopathic arthritis (JIA) is one of the most common rheumatic diseases in children, but DAH as an onset manifestation of JIA is rare. This study summarizes the clinical characteristics of patients with JIA presenting as DAH. We retrospectively analyzed the age of onset, clinical manifestations, imaging features, treatments, and prognosis of five cases of JIA presenting as DAH. Themedian age at DAH onset was 6 months (range, 2 months-3 years). Pallor was the most common manifestation of onset (5/5). Other symptoms included cough (2/5), tachypnea (2/5), hemoptysis (1/5), cyanosis (1/5), and fatigue (1/5). Imaging showed ground-glass opacity (GGO) (5/5), subpleural or intrapulmonary honeycombing (4/5), consolidation (3/5), interlobular septal thickening (2/5), and nodules (1/5). Anticitrullinated protein antibodies (ACPA) and rheumatoid factor (RF) were positive in five children (5/5), and antinuclear antibody (ANA) was positive in four children (4/5). ANA in three children and ACPA/RF in one child were positive before the onset of joint symptoms. The median age at the onset of joint symptoms was 3 years and 9 months (2 years and 6 months-8 years). Joint symptoms were mainly characterized by joint swelling, pain, and difficulty walking, and the most commonly affected joints were the knees, ankles, and wrists. After the diagnosis of DAH, the five patients were treated with glucocorticoids. Alveolar hemorrhage was effectively controlled in three cases, but the other two patients still had anemia and poor improvements in chest imaging. After joint symptoms, the patients were treated with glucocorticoids combined with diclofenac, disease-modifying antirheumatic drugs, and biological agents. Alveolar hemorrhage was in remission, and joint symptoms were relieved in the five cases. DAH can be the first clinical manifestation of JIA, and joint involvement occurs 1-5 years later. Children with DAH who are positive for RF, ACPA, and/or ANA and have GGO accompanied by honeycombing on imaging should be concerned about their joint involvement in future.

Tocilizumab in Juvenile Idiopathic Arthritis Associated Uveitis, a Narrative Review

Juvenile idiopathic arthritis (JIA) associated uveitis (JIA-U) is the most common extra-articular manifestation of JIA, affecting 10-15% of patients, especially in oligoarticular JIA where its course may be faint. Therefore, JIA-U is one of the most challenging pediatric uveitis, associated with major ocular morbidity and possibly leading to irreversible structural ocular damage and to vision-threatening complications. Adequate management is crucial for avoiding visual impairment complications. Since the introduction of biologic disease modifying anti-rheumatic drugs (bDMARDS), the visual prognosis of JIA-U has dramatically improved over the decades. Tumor necrosis factor-α (TNF-α) blockers are the most used bDMARDs in treating JIA-U with large evidence of efficacy. However, inadequate response to these agents, either due to intolerance or inefficacy, may be observed, requiring a swap to other classes of immunosuppressive agents, including anti-IL-6, anti-CD20, and, more recently, JAK inhibitors. Tocilizumab is a humanized monoclonal antibody to the interelukin-6 receptor preventing IL-6 from binding to its soluble and membrane-bound receptors. A growing body of literature provides promising results about the efficacy of intravenous and subcutaneous tocilizumab in the treatment of JIA-U. A narrative review of the literature on this topic will improve our knowledge on the potential use of tocilizumab in JIA-U.

Ассоциации гена VDR с клиническими проявлениями, осложнениями и обеспеченностью витамином D на фоне ювенильного идиопатического артрита у детей

Objective. To conduct an association search between genetic variants (c.1206T&gt;C, c.152T&gt;C, c.1174+283G&gt;A) of the VDR gene and clinical manifestations of juvenile idiopathic arthritis (JIA), need for genetically engineered biological drugs (GEBDs) and vitamin D status in children. Patients and methods. This study included 150 children (mean age: 9.11 ± 2.21 years) with JIA and 333 healthy controls. The determination of serum calcidiol concentrations was performed in all patients. Polymorphic variants of the VDR gene (c.1206T&gt;C, c.1175-9G&gt;T, c.152T&gt;C, c.1174+283G&gt;A) were tested by polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) technique. Results. Carriers of the minor TT genotype of the genetic variant c.1206T&gt;C(A&gt;G) TaqI are 4 time more likely to develop systemic JIA, 5 time more likely to have high disease activity and uveitis, as well as 9.9 times more often require prescription of GEBDs. Carriers of the minor genotype TT of the genetic variant c.152T&gt;C FokI are 7.7 times more likely to develop systemic JIA and 4.3 times – polyarticular JIA, 6.2 times more likely to have high disease activity and 5.6 times – uveitis, 8 times more often have a severe calcidiol deficiency and 4 times more often require prescription of GEBDs. Carrying the minor AA genotype of the BsmlI polymorphism (c.1174+283G&gt;A) increases the risk of systemic-onset JIA by 17 times, high disease activity and uveitis – by 18 times, and need for GEBDs – by 15 times; carrying the AA and GA genotypes increases the risk of calcidiol deficiency by 5 times and severe calcidiol deficiency by 9 times. Conclusion. All studied genetic variants of the VDR gene verifiably affect the development of clinical manifestations, complications, calcidiol levels and response to therapy in JIA. Key words: VDR gene, juvenile idiopathic arthritis, children, vitamin D, autoimmune disease

Tumor necrosis factor (TNF) inhibitors for juvenile idiopathic arthritis-associated uveitis.

Uveitisis the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA) and a potentially sight-threatening condition characterized by intraocular inflammation. Current treatment for JIA-associated uveitis (JIA-U) is largely based on physician experience, observational evidence and consensus guidelines, resulting in considerable variations in practice. OBJECTIVES: To evaluate the effectiveness and safety of tumor necrosis factor (TNF)inhibitors used for treatment of JIA-U. We searched the Cochrane Central Register of Controlled Trials (CENTRAL); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature Database (LILACS); ClinicalTrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). We last searched the electronic databases on 3 February 2022. We included randomized controlled trials (RCTs) comparing TNF inhibitors with placebo in participantswith a diagnosis of JIA and uveitis who were aged 2 to 18 years old. We used standard Cochrane methodology and graded the certainty of the body of evidence for seven outcomes using the GRADE classification. We included three RCTs with 134 participants. One study conducted in the USA randomized participants to etanercept or placebo (N = 12). Two studies, one conducted in the UK (N = 90) and one in France (N = 32), randomized participants to adalimumab or placebo. All studies were at low risk of bias.Initial pooled estimates suggested that TNF-inhibitors may result in little to no difference on treatment success defined as 0 to trace cells on Standardization of Uveitis Nomenclature (SUN)-grading; or two-step decrease in activity based on SUN grading (estimated risk ratio (RR) 0.66; 95% confidence interval (CI) 0.21 to 2.10; 2 studies; 43 participants;low-certainty evidence) or treatment failure defined as a two-step increase in activity based on SUN grading (RR 0.31; 95% CI 0.01 to 7.15; 1 study; 31 participants;low-certainty evidence). Further analysis using the individual trial definitions of treatment response and failure suggested a positive treatment effect of TNF inhibitors; a RR of treatment success of 2.60 (95% CI 1.30 to 5.20; 3 studies; 124 participants; low-certainty evidence), and RR of treatment failure of 0.23 (95% CI 0.11 to 0.50; 3 studies; 133 participants). Almost all the evidence was on adalimumab and the evidence on etanercept was very limited. For secondary outcomes, one study suggests that adalimumab may have little to no effect on risk of recurrence after induction of remission at three months (RR 2.50, 95% CI 0.31 to 20.45; 90 participants; very low-certainty evidence) and visual acuity, but the evidence is very uncertain; mean difference in longitudinal logMAR score change over six months was -0.01 (95% CI -0.06 to 0.03) and -0.02 (95% CI -0.07 to 0.03) using the best and worst logMAR measurement, respectively (low-certainty evidence). Low-certainty evidence from one study suggested that adalimumab treatment results in reduction of topical steroid doses at six months (hazard ratio 3.58; 95% CI 1.24 to 10.32; 74 participants who took one or more topical steroid per day at baseline). Adverse events, including injection site reactions and infections, were more common in the TNF inhibitor group. Serious adverse events were uncommon. Adalimumab appears to increase the likelihood of treatment success and decrease the likelihood of treatment failure when compared with placebo. The evidence was less conclusive about a positive treatment effect with etanercept. Adverse events from JIA-U trials are in keeping with the known side effect profile of TNF inhibitors. Standard validated JIA-U outcome measures are required to homogenize assessment and to allow for comparison and analysis of multiple datasets.

Uveitis Is a Risk Factor for Juvenile Idiopathic Arthritis' Significant Flare in Patients Treated With Biologics

ObjectivesUveitis is the most frequent extra-articular manifestation of juvenile idiopathic arthritis (JIA). Our study is aimed to evaluate the possible difference in arthritis course depending on uveitis presence in patients with JIA, treated with biologics.MethodsFrom our database of patients with JIA treated with biologics, we extracted patients to whom the first agent was administrated with or without MTX. The exclusion criteria included treatment with current systemic corticosteroids, infliximab, rituximab, observation period <3 years, and no missing data. After selection, 175 patients were eligible for analysis. We evaluated clinically significant flare with joint involvement (which required change of biologic or non-biologic DMARD) and time to flare. We compared two groups: (i) patients with uveitis (n = 32) and (ii) patients without uveitis (n = 143). For statistical analysis, we used Cox's regression models, the log-Rank test, x2 test, and the Mann–Whitney test.ResultsThere was no difference in gender distribution and achievement of arthritis remission between groups. Patients in the non-uveitis group predominantly received etanercept (64.3%). In the uveitis group, the most prescribed biologic agent was adalimumab (71.9%). The presence of uveitis increased the risk of JIA flare, OR = 3.8 (95% CI: 1.7; 8.7), and the cumulative probability of joint flare, RR = 4.5 (95% CI: 1.7; 12.1), p =.003, after adjustment on methotrexate, RR = 3.1 (1.6; 6.), p =.0008. In the subgroup of patients treated with adalimumab, the absence of methotrexate increased the cumulative probability of flare [RR = 6.5 (95% CI: 1.4; 31.1), p = 0.02].ConclusionThe presence of uveitis proved to be a risk factor in JIA flare. Methotrexate can decrease the cumulative flare probability. Further trials are required.

Erythrocyte blood indices and state of iron metabolism in children with juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is a systemic chronic disease. One of the extra-articular manifestations of JIA are hematological changes. The most common is anemia. The aim of the study was to explore the indicators of the red blood cells and the state of iron metabolism in children with JIA, depending on the form and duration of the disease. A retrospective analysis of the medical history of patients was carried out. The study of the indicators of the red blood cells consisted in determining the number of erythrocytes, the amount of hemoglobin, the ratio of the number of erythrocytes to blood volume, the average volume of erythrocytes, the average hemoglobin content in the erythrocyte, the average concentration of hemoglobin in the erythrocyte, as well as the distribution of red blood cells along the length in the form of the difference between the largest and smallest size of red blood cells and the deviation of the volume of red blood cells from the average value. The state of iron metabolism was represented by such indicators as the content of serum iron, ferritin, transferrin, latent iron-binding capacity of blood serum, total iron-binding capacity of serum, transferrin saturation coefficient with iron. In 18.7 % of children with JIA, a decrease in hemoglobin of less than 120 g/l was noted. There was a significant (p &lt; 0.001) decrease in the average hemoglobin content in the erythrocyte in 37.9 % of patients. As a result of the study, it was found that the decrease in the iron content in the blood serum is due not only to the true iron deficiency state, but also to the redistribution of iron reserves against the background of chronic autoimmune inflammation. In the systemic form of the disease, changes in the latent iron deficiency state are more pronounced. With a long course of the disease, normalization of red blood cell parameters occurs. The duration of the disease has no effect on ferrokinetics, which is due to the development of iron deficiency compensation against the background of ongoing treatment.

Features of anemic syndrome in juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is a systemic chronic disease. One of the extra-articular manifestations of JIA are hematological changes. The most common is anemia. The aim of the study was to explore the indicators of the red blood cells and the state of iron metabolism in children with JIA, depending on the form and duration of the disease. As a result of the study, it was found that the decrease in the iron content in the blood serum is due not only to the true iron deficiency state, but also to the redistribution of iron reserves against the background of chronic autoimmune inflammation.

Evaluation of Patients with Juvenile Idiopathic Arthritis- Associated Uveitis from Rheumatology Perspective

Objective: Uveitis, the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA), is most commonly found in children with oligoarticular type and polyarticular rheumatoid factor (-) JIA. Close follow-up of these children by pediatric rheumatologists and ophthalmologists is important because of the risk of blindness if these cases are untreated. This study aims to evaluate the frequency of uveitis, demographic characteristics, and complications in children with JIA. Material and Methods: Among the patients with JIA who were followed up at the Pediatric Rheumatology Clinic of Dr Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between January 2017 and July 2021, those with uveitis were included in the study. Location of uveitis, laterality, age at onset of uveitis, complications of uveitis, duration of follow up, laboratory findings, medications used, and status of uveitis at the time of data collection were obtained from the patients’ files. Results : Uveitis was observed in 34 (7.1%) of the 473 children with JIA included in the present study. Twenty three patients were female (67.6%). The age at diagnosis of JIA was 5.9±5.1 years, and the age at diagnosis of uveitis was 7.5±4.2 years. The most common form of JIA was the persistent oligoarticular form. Compared with the age of onset of arthritis in all JIA patients, the age of onset of arthritis was lower in patients with JIA-associated uveitis (7.8±4.6 years vs. 5.9±5.1 years). Anatomically, all patients had anterior uveitis. Antinuclear antibody positivity was more common in children with JIA-associated uveitis (47.1%) than all of our patients with JIA (19%). In one of the patients, arthritis and uveitis were diagnosed simultaneously. Posterior synechia was found in three patients (8.8%). Arthritis was the first symptom in 27 patients (79.4%) and uveitis in six patients (17.6%), cataract in five patients (14.7%), glaucoma in two patients (5.9%), and blindness in one eye (2.9%). Conclusion: Uveitis is the most common extra-articular complication of JIA and has sight-threatening complications which may lead to irreversible visual loss. The findings of this study suggest that the joint effort of pediatric rheumatologists and ophthalmologists is needed to diagnose these children promptly and treat them appropriately. Key Words: Eye diseases, Juvenile Idiopathic Arthritis, Uveitis

ЮВЕНИЛЬНЫЙ ИДИОПАТИЧЕСКИЙ АРТРИТ И СИСТЕМНАЯ КРАСНАЯ ВОЛЧАНКА В ДЕТСКОМ ВОЗРАСТЕ: ЧАСТОТА, ПРИЧИНЫ, КЛАССИФИКАЦИЯ, КЛИНИЧЕСКИЕ ПРОЯВЛЕНИЯ

Juvenile idiopathic arthritis (JIA) is one of the most common and most disabling rheumatic diseases in children. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with unknown etiology, which is based on a genetically determined dysregulation of immunity. In recent years, the view of JIA and SLE as potentially incurable and prognostically unfavorable diseases has been revised. This is largely due to the expansion of the possibilities of early diagnosis of JIA, SLE, which allows starting active therapy at the onset of the disease, the development of a new class of basic anti-inflammatory drugs, and scientific research in the genetics of both diseases. A large number of genes associated with immune responses can, induce an autoimmune response - the leading pathogenetic link in both diseases. The article presents modern data on the possible pathogenesis, spectrum of clinical manifestations of juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE) in children. A brief description of the pathological changes in JIA and SLE is given. Possible factors are considered, incl. genetic, affecting the predisposition to the disease, the process of the formation of organ damage. The issues of assessing the activity of diseases, assessing the quality of life of patients are discussed in detail.

Clinical manifestations of gastrointestinal tract changes in children with juvenile idiopathic arthritis

Summary. Juvenile idiopathic arthritis remains one of the most common chronic inflammatory rheumatic diseases of childhood. A significant proportion of patients experience nausea, vomiting, abdominal pain and loss of appetite with methotrexate therapy, which can significantly complicate the course of the disease. The aim was to study the clinical and anamnestic signs of liver disorders in children, depending on the manifestations of juvenile idiopathic arthritis. Materials and methods of research. The presence of gastrointestinal complaints, namely abdominal pain, loss of appetite, nausea and vomiting in 104 children with juvenile idiopathic arthritis who were treated at the State Institution "Institute of Child and Adolescent Health of the National Academy of Medical Sciences of Ukraine" was analyzed. Results. According to the results of the study, children with juvenile idiopathic arthritis had gastrointestinal complaints in 47.12%. We find out that the majority of children had gastrointestinal complains at the age of 10–13 years (55.36%, p ˂ 0.001), and at the onset of the disease after 15 years (100%, p ˂ 0.01). Young children often complained of abdominal pain and vomiting, older children complained of loss of appetite and persistent nausea, regardless of the variant, activity, duration of arthritis and the presence of methotrexate in complex therapy. It was also found that appearance of gastrointestinal complaints were observed more often at a dose of methotrexate less than 10 mg / m2 / body surface (p ˂ 0.05). Conclusions. 1. We find out that in 47.12% children with juvenile idiopathic arthritis had gastrointestinal complaints, aged 10–13 years (55.36%; p ˂ 0.001). The most amounts of complaints were common for patients older than 15 years old (p ˂ 0.01). 2. The nature of the complaints varied and depended mainly on the age of the patients. Younger children had abdominal pain and vomiting simultaneously older children had decreased appetite and nausea. The presence of complaints did not depend on the variant, activity and duration of the juvenile idiopathic arthritis. 3. According to our study complaints were not due to the presence of methotrexate in combination therapy. Children complained much more often if methotrexate dose was less than 10 mg / m2 than in the case of higher doses (p ˂ 0.05).

Radiographs and MRI of the Cervical Spine in Juvenile Idiopathic Arthritis: A Cross-Sectional Retrospective Study.

Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease among children. In some patients, cervical spine arthritis remains a serious and chronic manifestation of JIA. The aim of this study was to assess the frequency of cervical spine lesions on radiographs and MRI in JIA patients with clinical signs of cervical spine involvement and to verify if with the addition of MRI, the use of radiographs could be abandoned. Methods: This retrospective study evaluated consecutive 34 children (25 girls; aged 6–18 years, median 15.5 years) with JIA and with clinical involvement of cervical spine. In each patient, both radiographs and MRI of the cervical spine were performed. Imaging findings were correlated with clinical and laboratory data. Results: The cervical spine was affected in 35% of patients. The most frequent lesions were subaxial subluxations (SAS; 24%), apophyseal joint ankylosis (9%), and C1/C2 joint lesions (9%). Anterior atlanto-axial subluxation (AAS) was diagnosed only by radiography, and most of the SAS were seen on radiography, whereas only a few on MRI. Reversely, C1/C2 soft tissue involvement were seen on MRI only. Cervical spine involvement was associated with raised ESR (p = 0.012) and CRP (p = 0.014). Conclusions: The cervical spine lesions are still frequent complication of JIA affecting up to 35% of JIA patients. Most of them develop serious complications, such as AAS and ankylosis. Despite advantages of MRI in terms of the imaging of the atlanto-axial region radiography shows superiority in diagnosis of AAS and SAS.

\u201cEnvironmental risk factors associated with juvenile idiopathic arthritis associated uveitis: a systematic review of the literature\u201d

BackgroundJuvenile idiopathic arthritis associated uveitis (JIA-U) is the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA) and carries considerable risk to vision. The aim of this systematic review was to synthesise evidence of environmental risk factors for JIA-U and identify risk factors which may be modifiable or used to stratify JIA patients.MethodsThis systematic review was carried out in accordance with PRISMA guidelines. Four online databases - Cumulative Index of Nursing and Allied Health Literature, Web of Science, MEDLINE and Embase - were searched from database inception to 12th August 2020. Identified studies were screened by two independent reviewers against pre-defined inclusion and exclusion criteria. Data was extracted from all primary studies meeting inclusion criteria and independently checked.ResultsWe identified three studies from 895 unique records which met the inclusion criteria, each examining a different environmental risk factor. This systematic review includes 973, predominantly female, participants with JIA across these three studies. The use of allergy medication or documentation of “allergy”/“allergic” in the medical records was associated with an increased risk of JIA-U in all models presented. Vitamin D sufficiency was associated with reduced risk of JIA-U. There was insufficient evidence to support an association between seasonality and JIA-U.ConclusionsThis review identifies a potential role for allergy and vitamin D in JIA-U. It also illustrates the paucity of data regarding environmental risk factors for JIA-U and highlights the need for further research to both identify additional risk factors and replicate existing findings.

Опыт успешного применения адалимумаба у детей с ювенильным идиопатическим артритом, ассоциированным с увеитом

Objective of the Paper: To describe clinical cases of successful use of adalimumab in children with uveitis-associated juvenile idiopathic arthritis (JIA). Key Points. One of the extraarticular manifestations of JIA is uveitis, i. e. inflammation of the vascular tract of the eye. JIA-associated uveitis is diagnosed primarily in small girls with oligoarthritis, early seronegative polyarthritis; in patients with arthritis associated with enthesitis and psoriatic arthritis. The article describes clinical cases of the use of adalimumab in JIA and uveitis where the therapy with methotrexate and topical glucocorticosteroids fails. Conclusion. Uveitis-associated JIA is one of the most disabling forms of JIA, since, despite timely and adequate management, very often it results in severe ocular complications: cataract, glaucoma and even blindness. Adalimumab, a human anti-TNFα monoclonal antibody, is available and it helps in achieving uveitis remission where methotrexate and topical glucocorticosteroids fail, as well as in delaying ocular complications. Keywords: juvenile idiopathic arthritis, uveitis, adalimumab, children.