Manifestation Of Juvenile Idiopathic Arthritis Research Articles

環軸関節回旋位固定で発症した 若年性特発性関節炎の2 例

環軸関節回旋位固定で発症した 若年性特発性関節炎の2 例

Current management of juvenile idiopathic arthritis affecting the craniovertebral junction.

Craniovertebral instability is a rare and serious problem. While previously treated surgically, better understanding of disease processes has permitted the field to move towards conservative management. Juvenile idiopathic arthritis (JIA) is one cause of pediatric craniovertebral instability. Early recognition and institution of appropriate medical therapy and bracing in a multidisciplinary fashion is critical to avoid long-term instability, joint abnormalities, or morbid surgical procedures. We seek to highlight cases of this rare problem and provide a principled approach to management decisions. We review 6 cases that have presented over the last 6years and highlight 3 cases in particular regarding craniovertebral instability as a presentation of JIA. We reviewed the clinical records and radiographic features with particular emphasis of the stability of the craniovertebral junction. Age range of the subjects was from 5 to 12. All patients presented with neck pain and abnormal head rotation. Four of the patients responded to medical management and/or cervical bracing with no long-term sequelae or instability. Two patients had refractory rotary subluxation, one that responded to manual reduction under pharmacological paralysis and bracing; the other had an incompetent transverse ligament requiring surgical reduction and fixation. Neck pain and abnormal head rotation in an older child is rare finding but should prompt suspicion as a manifestation of JIA to the general pediatrician or initial provider. Appropriate serologic studies and MRI studies with contrast at the craniovertebral junction is necessary for evaluation. Early institution of medical management and cervical bracing under a multidisciplinary team of pediatric rheumatology and neurosurgery is key to avoiding surgical intervention and long-term abnormalities at the craniovertebral junction.

Treating juvenile idiopathic arthritis (JIA)-related uveitis beyond TNF-α inhibition: a narrative review.

Chronic anterior uveitis is the most frequent among extra-articular manifestations of juvenile idiopathic arthritis (JIA) and a relevant cause of ocular morbidity in children. Asymmetric arthritis, early onset disease, female sex, and anti-nuclear antibody (ANA) positivity are counted among risk factors for developing this complication. It usually has insidious onset and asymptomatic chronic-relapsing course, but the persistence of low-grade chronic inflammation can lead to irreversible structural ocular damage and to vision-threatening complications. For such reasons, achieving a complete absence of inflammation through early targeted and aggressive treatments is a primary therapeutic goal in these patients. This review is aimed at summarizing scientific evidence about biologic rescue therapy of JIA-related uveitis in patients who fail to achieve clinical remission, in spite of being treated with conventional disease-modifying anti-rheumatic drugs (cDMARDs) and at least one biologic tumor necrosis factor (TNF)-α inhibitor. Interleukin (IL)-6 inhibition appears a promising and safe option for refractory JIA-related uveitis. Abatacept and rituximab proved to be beneficial as well, but their efficacy together with some safety concerns needs to be more extensively evaluated.

Standardizing the Clinical Orofacial Examination in Juvenile Idiopathic Arthritis: An Interdisciplinary, Consensus-based, Short Screening Protocol.

To develop a consensus-based, standardized, short (< 3 min) clinical examination protocol to assess the multidimensional, orofacial manifestations of juvenile idiopathic arthritis (JIA). The study was conducted by a multidisciplinary task force from the Temporomandibular Joint Juvenile Arthritis Working Group (TMJaw). The study used an acknowledged sequential approach involving (1) a global multidisciplinary online questionnaire study, (2) a systematic literature review and consensus meetings to identify items for inclusion, (3) pilot testing of included items, (4) test of reliability in 22 subjects with JIA by 4 examiners, (5) test of construct validity in a case-control study involving 167 subjects, and (6) establishment of final recommendations. Six items were recommended for the final examination protocol: (1) clinician-assessed pain location, (2) temporomandibular (TMJ) joint pain on palpation (open and closed mouth), (3) mandibular deviation at maximal mouth opening (≥ 3 mm), (4) maximal unassisted mouth opening capacity, (5) frontal facial symmetry, and (6) facial profile. All recommended items showed acceptable reliability and construct validity. The average mean examination time was 2 min and 42 s (SD ± 38.5 s). A consensus-based, short clinical examination protocol was developed. The protocol takes less than 3 min to complete and provides information about orofacial symptoms, TMJ dysfunction, and dentofacial deformity. The standardized examination protocol is applicable to routine clinical care, as well as future research studies.

Temporomandibular joint disorders as the only manifestation of juvenile idiopathic arthritis: a case report

ABSTRACTJuvenile idiopathic arthritis is a term used to include all chronic childhood arthritis of unknown etiology. It is characterized by chronic inflammation persisting for at least 6 weeks, beginning before 16 years of age. The characteristics present are chronic synovitis, arthralgia, impaired joint mobility in at least one joint, and erosion with destruction of cartilage and subchondral bone, that could be associated or not with systemic involvement, according to each subtype of the disease. During the pathologic process, the temporomandibular joint can be involved by the juvenile idiopathic arthritis, resulting in severe mandibular dysfunction, with higher frequency in female patients. Initially, these lesions can show minor alterations like flattening of the condyle, erosions, and evolve to severe lesions, like destruction of the head of the condyle. We report a case of male patient who had destruction of both condyles, as a result from juvenile idiopathic arthritis. Proposed mechanisms to explain the juvenile idiopathic arthritis was reviewed. In this report the patient did not have pain or inflammatory process, and the temporomandibular diseases was the only manifestation.

The first manifestation of juvenile idiopathic arthritis in orthopaedic practise

The first manifestation of juvenile idiopathic arthritis in orthopaedic practise

Update on the Treatment of Uveitis in Patients with Juvenile Idiopathic Arthritis: A Review.

Chronic uveitis is a common extra-articular manifestation of juvenile idiopathic arthritis. The classic clinical picture is one of chronic anterior uveitis, which usually remains asymptomatic until ocular complications arise. The risk of uveitis is increased in girls with an early onset of oligoarthritis and positive antinuclear antibodies. Even though the inflammation in patients with juvenile idiopathic arthritis is initially limited in the anterior part of the eye, chronic active inflammation may eventually cause significant damage to the posterior pole. Complications may include band keratopathy, cataract, secondary glaucoma, posterior synechiae, cystoid macular edema, and hypotony. The cooperation of ophthalmologists with rheumatologists may help define the best treatment plan. The ophthalmic therapeutic regimen includes topical corticosteroids and mydriatics, while in severe cases immunosuppressive and biological agents are introduced. Surgical management of complications might be needed.

Isolated Arthritis of the Temporomandibular Joint as the Initial Manifestation of Juvenile Idiopathic Arthritis.

To describe characteristics of patients with juvenile idiopathic arthritis (JIA) presenting with isolated arthritis of the temporomandibular joints (TMJ). Patients with JIA with isolated TMJ arthritis from 4 large tertiary pediatric rheumatology centers were included. Demographic and clinical data were analyzed using descriptive statistics. Fifty-five patients were identified (65% bilateral presentation). Six patients developed arthritis in other joints (median time 6 mos); 4 patients developed uveitis, all prior to arthritis. At last followup, 9% were still taking antirheumatic medications. JIA TMJ arthritis can occur in isolation, and is probably underdiagnosed. Care providers including dentists and orthodontists should be aware of this presentation.

Uveitis Associated with Juvenile Idiopathic Arthritis, our Observations.

Introduction:Uveitis as extra-articular manifestation of juvenile idiopathic arthritis (JIA) is present in 20% of children with the persistent form, in 30% with the extended oligoarticular form, while it is present in psoriatic and polyarticular rheumatoid factor (RF) negative arthritis in 10% and 14%, respectively.Aim:The aim of the study was to evaluate the frequency of uveitis and its complications in children with JIA.Material and Methods:A retrospective study was conducted with an analysis of the medical records of children with JIA who were treated for the last 5 years. The analysis included the following: the child’s age and sex, age at onset of arthritis, of uveitis, complications, RF values and antinuclear antibodies (ANA).Results:The study included 97 children with JIA: in 14 (14.4%) uveitis was observed; the most common form of JIA was the oligoarticular extended form (6/14), oligoarticular persistent form was observed in 5 children, while 3 children with uveitis had polyarticular RF negative JIA. The age of arthritis onset was lower in children with uveitis (4.7 vs 8.2 years); ANA positivity was more common in children with JIA and uveitis (64% vs 41%). Uveitis was the first manifestation of the disease for 2 children; 28.6% of children had clinically asymptomatic uveitis, while 42.4% of children developed uveitis within 4 years from the JIA onset. 8/14 children developed uveitis complications: 3 cases of synechia, 2 band keratopathy, 2 cataracta, 1 glaucoma.Conclusion:Uveitis as significantly present manifestation of JIA requires to timely recognize, treat, monitor children in order to prevent complications.

Психоемоційний стан у молодих дорослих з ювенільним ідіопатичним артритом

Aim.&nbsp;To determine emotional state in adults with juvenile idiopathic arthritis (JIA) using questionnaires/ Material and Methods.&nbsp;Patients diagnosed with JIA (N=106) aged over 18 years were examined on the basis of the Alexander Municipal Clinical Hospital, Kyiv, Ukraine. The diagnosis was verified according to the classification criteria of the International League of the Association of Rheumatologists. The study did not include patients with organic pathology of the brain, encephalopathy associated with hypertension, diabetes mellitus, and chronic renal failure. Clinical examination included count of swollen and painful joints; count of joints requiring prosthetics in consequence of destructive changes; assessment of disease activity with regard to administered anti-rheumatic drugs; assessment of quality of life and health with the questionnaires SF-36, HAQ, PHQ-9; evaluation of anxiety by Beck and Hamilton (HAM-A) questionnaires; evaluation of depression by Beck questionnaire surveys and Chaban questionnaire on self-evaluation of anxiety and depression; and determining the social factors that appear to affect health. Results and Discussion.&nbsp;All patients were divided into two groups, depending on the need for prosthetic joints regardless of JIA clinical variants. Group I included 20 patients aged 24,20±8,7 years, with the need of one or more prosthetics joints as a result of JIA. The second group included 86 patients who did not require prosthetic joints, with mean age 23,18±6,18 years. In group I, a longer disease duration (p=0.025), more deformed joints (p&lt;0.001), more frequent cervical spine damage (p=0.015), arthritis of hands joints (p=0.008), lesions of tendons (p=0.037), more systemic manifestations of JIA (p=0.003), higher ESR (p=0.001) and DAS 28 (p&lt;0,0001) were found; &nbsp;they also received more aggressive therapy, including longer course of glucocorticoids (GC) (81.74 months) than the patients in group II (15.34 months) (p&lt;0.0001) and a higher cumulative dose of GC (p = 0.001). The patients' physical condition according to the SF-36 was 33,92±14,0 in &nbsp;group I vs 43,74±13,62 in group II, indicating a lower level of physical health (p&lt;0.001) and poorer functional status by HAQ (p=0.034). However, the patients' psychological health by the SF-36 did not differ in two groups (p=0.832), although the PHQ-9 questionnaire showed that the patients in group I revealed moderate severity depression (11,17±6,19) vs light ("subclinical") depression (6,4±6,02) in group II (p=0.019), which is also confirmed by the self-evaluation scale for depression, where a tendency towards depression was found in group I (9,0±6,39) compared to no signs of depression (4,5±3,98) in group II (p=0.018). According to the questionnaire on psychosomatic disorders, higher need for medical psychologist's consultation (8,5±6,35) was found in &nbsp;group I (p=0.013) than in group II (4,08±3,56). However, in anxiety assessment, the difference was not detected by any questionnaires in both groups of patients, although the anxiety level was higher in group I. Conclusions.&nbsp;Patients with JIA with destructive changes in the joints have higher tendency towards psycho-emotional disorders that require medical consultations of a psychologist or a &nbsp;psychosomatic medicine professional. Active and early treatment of JIA using modern methods, including biological therapy, allow to prevent destructive changes in the joints, that in its turn reduces psycho-emotional disorders in adult patients with JIA. Patients with aggressive destructive course of JIA need higher social adjustment, which causes a need to include medical psychologists in the team of doctors (along with rheumatologists, podiatrists, rehabilitation professionals, and ophthalmologists) who treat adults with JIA.&nbsp

若年性特発性関節炎（JIA）の関節外症状とその対策

若年性特発性関節炎（JIA）の関節外症状とその対策

Uveitis Events During Adalimumab, Etanercept, and Methotrexate Therapy in Juvenile Idiopathic Arthritis: Data From the Biologics in Pediatric Rheumatology Registry

Uveitis is a major extraarticular quality of life-restricting manifestation of juvenile idiopathic arthritis (JIA). The aim of the study is to describe the occurrence of uveitis in JIA patients receiving tumor necrosis factor inhibitors or methotrexate (MTX). Patients' characteristics, treatment, and the reported first occurrence of uveitis as an adverse event were searched in the Biologics in Pediatric Rheumatology Registry. The rates per exposed patients, exposure time, and time until event were calculated. Uveitis was reported as an adverse event in 75 of 3,467 patients; 51 of 2,844 patients were receiving MTX, 37 of 1,700 patients were receiving etanercept, and 13 of 364 patients were receiving adalimumab. Patients with uveitis were younger (mean ± SD age 4.6 ± 4.2 versus 7.4 ± 4.5 years; P < 0.0001), more likely to be antinuclear antibody positive (69% versus 43%; odds ratio [OR] 2.7, P < 0.0001), and had extended oligoarticular JIA (OR 2.2, P = 0.0005). Patients with a uveitis diagnosis before starting treatment more often had a uveitis event (n = 28, 8.4%; OR 8.5, P < 0.0001), and more often received adalimumab (OR 2.15 [95% confidence interval 1.58-2.94], P < 0.0001). In 16 patients, a new uveitis event occurred: 11 while taking MTX (3.2 per 1,000 patient-years), 2 while taking etanercept monotherapy (1.9 per 1,000 patient-years), and 3 while taking etanercept and MTX combination (0.9 per 1,000 patient-years). A new uveitis event occurred early in the disease course after a median disease duration of 1.5 years (interquartile range [IQR] 1.3-3.8) while taking etanercept and 1.8 years (IQR 1.8-2.1) for the MTX cohort. A recurrent uveitis event was reported after a disease duration of 7.6 years (IQR 4.3-10.0) in the etanercept cohort and 4.8 years (IQR 1.0-5.8) in the MTX cohort. Univariate analysis showed that MTX, but not etanercept or adalimumab, led to a lower rate of uveitis. Patients with a history of uveitis had higher risks for uveitis events while taking both etanercept and adalimumab. Methotrexate turned out to be protective. Few patients developed a first uveitis event while taking etanercept, while the rate is comparable to that with MTX. Uveitis may not be attributed to be an adverse drug reaction to etanercept.

Can the Methotrexate Therapy Prevent the Development of Uveitis in Patients with Juvenile Idiopathic Arthritis: Results of a Retrospective Study

Background: Uveitis is one of the most common extra-articular manifestations of juvenile idiopathic arthritis (JIA). Currently, the possibility of reducing the risk of uveitis in children with JIA by using methotrexate has been studied. Objective: Our aim was to analyze the results of treatment of children with JIA by studying the relation between the use of methotrexate and the risk of uveitis. Methods: A retrospective uncontrolled study. The case histories of patients with JIA who were treated for at least 2 years after the onset of the disease were studied. The results of treatment of patients who received and who did not receive methotrexate were studied (standard therapy — non-steroidal anti-inflammatory drugs and intra-articular injections of glucocorticoids). The established cases of uveitis were taken into account. Results: The study analyzed the results of observation of 281 children with JIA. In the methotrexate group, uveitis was detected in 22/191 (11.5%), and in the control group — in 42/90 (46.7%) of patients (OR 6.7; 95% CI 3.7–12.3). The time period between the onset of JIA and development of uveitis in two groups under study was the same and equal to 24 (12; 67) and 17 months (7; 35), respectively (p = 0.232). Multivariate regression analysis showed that the main predictors of uveitis were oligoarticular course of JIA (HR = 1.89), positive antinuclear antibody test (HR = 2.14), onset of JIA under the age of 5 (HR = 2.56), female gender (HR = 1.82), and the absence of methotrexate in the therapy (HR = 0.24). Conclusion : The treatment with methotrexate may reduce the risk of uveitis in patients with JIA. To confirm this hypothesis, randomized studies are needed.

Managing monoarthritis in children

Monoarthritis, defined as inflammation of a single joint, requires a thorough physical examination in children, as pain may be lacking in 10% to 30% of cases and joint stiffness may be the only symptom. Joint aspiration is a crucial diagnostic tool that remains markedly underused. Joint aspiration may be unnecessary, however, when the family history or other investigations provide the diagnosis. Radiographs of the involved joint may supply information on the severity of the lesions. In doubtful cases and in patients with arthralgia, B-mode and Doppler ultrasound or magnetic resonance imaging (MRI) may confirm the presence of synovitis. Although suspected septic arthritis is an emergency and occurs frequently, particularly before 2 years of age, acute monoarthritis is often a post-infectious manifestation and therefore associated with a good outcome. Lyme disease should be sought, particularly when a lower limb joint is involved, as tick bites often go unnoticed. Chronic monoarthritis is very often a manifestation of juvenile idiopathic arthritis (JIA), which exists as several variants. Oligoarthritis is a specifically pediatric joint disease, whereas the other patterns of JIA have corresponding forms in adults, despite a number of specific features due to their onset during childhood. Tests for antinuclear antibodies should be performed routinely in children with monoarthritis persisting longer than 3 weeks. A decline in general health or a fever should suggest arthritis revealing a malignancy, which is a hematological disease in most cases. Finally, suggestive symptoms are often present in patients with less common causes such as auto-inflammatory diseases and connective tissue diseases.

Clinical Possibilities of Manifestation of Juvenile Idiopathic Arthritis

Juvenile Idiopathic Arthritis is the most common rheumatologic disease in children and one of the most common chronic diseases of childhood. The term JIA encompasses a heterogeneous group of disorders that all share the clinical manifestation of chronic joint inflammation. The etiology is unknown, but both genetic and environmental factors are believed to be involved. Management of the disease has improved in recent years due to advances of pharmacologic treatment options. The prognosis of patients with juvenile idiopathic arthritis is better.

Histone antibodies as a biomarker of uveitis in JIA

Uveitis is the most common extraarticular manifestation of juvenile idiopathic arthritis (JIA). The nature of JIA-associated uveitis is often insidious and asymptomatic, and baseline predictors can aid early diagnosis of eye disease for prompt and adequate treatment. Antihistone antibodies (AHA) are among subtypes of antinuclear antibodies (ANA) identified in children with JIA. An association between early-onset JIA, oligoarthritis and uveitis is shown in some studies, and we have previously shown that AHA is a significant predictor of chronic uveitis in a Norwegian JIA cohort. New interest for histones has emerged because epigenetic alterations of these DNA-binding molecules may be involved in the pathological processes of autoimmunity.

Pathophysiology of JIA-associated Uveitis

Juvenile idiopathic arthritis (JIA)-associated uveitis is an intriguing manifestation of JIA and an important contributor of long-term damage. Its pathophysiology is still poorly understood. This review summarizes current concepts. JIA is both a multifactorial and polygenetic disease. In the past 2 decades, multiple studies have indicated that the genetic contribution to both JIA and JIA-associated uveitis is modest. From an ophthalmological point of view, much of the pathophysiological data is derived from studies in experimental uveitis animal models. The pathophysiology of the arthritic manifestations of JIA has been studied extensively in humans. These studies have focused on the principal cells of the adaptive immune system, in particular different subsets of regulatory and effector T cells, as well as on antigen presenting cells or dendritic cells. With advancing technology and advancing knowledge of the underlying mechanisms of JIA-associated uveitis, new targets for therapy might evolve in the coming years.

Bilateral Panuveitis at Etanercept Initiation for Juvenile Idiopathic Arthritis

Introduction: Uveitis is a well-known extra-rheumatological manifestation of juvenile idiopathic arthritis (JIA). Tumor necrosis factor has been used to treat uveitis associated with inflammatory diseases. A new onset uveitis under anti-TNF therapy is uncommon. Case presentation : A 12-years-old male, affected since the age of six years, by a severe form of polyarticular JIA. When etanercept was started, he presented panuveitis bilaterally, so we switched to infliximab with good response. Conclusions : The TNF soluble receptor could be considered as a possible promoter in inducing endogenous new onset uveitis in JIA.

Neural mechanisms underlying the pain of juvenile idiopathic arthritis

Pain is the most common symptom of juvenile idiopathic arthritis (JIA) and is arguably a more important factor in disability than the progression of the disease itself. Studies have highlighted the extent of this pain and its persistence in some young patients despite effective disease control. Understanding and effective management of pain in JIA is limited, and improved diagnosis and treatment would benefit from increased knowledge of the mechanisms underlying pain in childhood. This Review focuses upon the developmental neurobiology of pain, reviewing studies in animal models that increase clinical understanding and inform treatment of the painful manifestations of JIA. Pain processing in the juvenile nervous system differs from that in adults: nociceptive thresholds are lower and endogenous pain control systems are slow to mature. Furthermore, increasing evidence points to tissue injury in childhood having prolonged effects upon the developing pain system. Injury, inflammation and stress in early life can 'prime' peripheral nociceptors and central pain circuits, such that the pain associated with tissue inflammation is exacerbated in later life. A developmental, mechanism-based approach towards developing novel targets for the treatment of pain in JIA might therefore benefit the patient both as a child and as an adult if the disease recurs or persists.

Ocular Manifestation of Juvenile Idiopathic Arthritis and its Relation to Disease Activity

Purpose:To determine the ocular manifestations of juvenile idiopathic arthritis (JIA) and its relation to its disease activity. To analyze the prevalence and complications of uveitis and their predictors in JIA. Methods: Fifty eight JIA patients and 20 age and sex matched healthy controls were included in this study; all JIA patients were recruited from Rheumatology and paediatric department. All patients were undergone complete rheumatological and ophthalmic examination including visual acuity, slit lamp examination of the anterior segment, dry eye evaluation using Rose Bengal stain and Schirmer test. Fundus examination and fundus photography were done to the suspected cases Results: Sixteen patients (27.6%) had ocular manifestations. Most of JIA were asymptomatic 49 (84.48%) for ocular manifestation. Nine patients (15.5%) complained of ocular manifestations in the form of blurring of vision, burning sensation and eye dryness. Patients with ocular abnormalities had an earlier disease onset and longer disease duration than those without any lesions (p<0.001). There was a highly statistically significant difference between patients and controls as regarding all ocular lesions, which was significantly higher in the patients group. Meanwhile, the most common ocular manifestation of JIA is uveitis (17.2%), five patients had keratoconjunctivitis Sicca, 8 patients had visual field defects, also four had macular edema, two had cataract and two had increase intraocular pressure. Conclusion: Ocular manifestations including sight threatening complications are not rare in patients with JIA. Uveitis is the most common ocular lesion. Therefore, continuous ophthalmologic examinations are needed in young people with JIA.