Adult Juvenile Idiopathic Arthritis Patients Research Articles

JAK inhibitors in the treatment of adult patients with juvenile idiopathic arthritis: a retrospective monocentric experience.

This study aims to evaluate the efficacy and safety of JAK inhibitors (JAKi) in a monocentric cohort of adult patients with juvenile idiopathic arthritis (JIA). Patients attending a rheumatology transition clinic were retrospectively included in case of: i) JIA diagnosis according to current classification criteria (1); ii) age ≥18 years and iii) treatment with JAKi for at least 3 months. Seventeen adult patients with JIA were treated with JAKi (as first JAKi, 9 patients (52.9%) received tofacitinib and 8 (47.1%) baricitinib). At 3 months after JAKi initiation, 8 patients (47%) achieved a response and 4 patients (23.5%) achieved disease remission (3 patients with baricitinib and 1 with tofacitinib, 37.5% vs. 16.7%, p=0.294). None of those with systemic JIA and enthesitis-related arthritis obtained remission; the remission rate at 3 months was higher, although not significantly, in the oligoarticular subset compared to the polyarticular subset (37.5% vs. 20%). Patients with ≤1 active joint involvement at JAKi start had a higher remission rate (50% vs. 22.2%). Subjects who achieved remission on JAKi had a significantly lower pre-treatment DAS28-CRP compared to those with still active disease (p=0.010, Mann-Whitney U=4). A pre-treatment DAS28-CRP <3.76 predicted response to JAKi with 100% sensitivity and 84.6% specificity (p=0.023). The remission rate was lower among patients who had been treated with ≥2 biological drugs before JAKi start (9% vs. 66.7%; p=0.05). One patient in concomitant treatment with leflunomide developed severe arterial hypertension. JAKi may represent an effective and safe treatment option for adult JIA patients with low/moderate disease activity, particularly in case of oligoarticular involvement.

Revising diagnosis of juvenile idiopathic arthritis in adults: a single-center retrospective study

The study aimed to assess how many adult patients with juvenile idiopathic arthritis (JIA) treated with biologics fulfill classification criteria for adult rheumatic diseases and to evaluate the course of JIA in adulthood. 138 patients with JIA over 18 years old treated with biologics were included in a cross-sectional observative study. Among 138 adult patients with JIA treated with biologics, 81 patients remained with JIA diagnosis. 57 patients were rediagnosed. 31 patients met the criteria for spondyloarthropathy, among them 18 patients for ankylosing spondylitis, 10 patients for psoriatic arthritis, and 3 patients for non-radiographic axial spondyloarthritis. Rheumatoid arthritis was diagnosed in 24 patients and adults’ Still disease in 2 patients. 84 patients of all adults with JIA received one biologic agent, 40 received two biologic agents, and 14 received three or more biologic therapies. 10 patients received biologic agents out of recommendations for JIA. Of the adult JIA patients treated with biologics, 41% met the classification criteria for adult inflammatory diseases. Spondyloarthropathy and rheumatoid arthritis were most commonly diagnosed. Nearly 40% of adult JIA patients required at least one modification of biological treatment. Therefore, it is worth considering a revision of JIA to adult-onset inflammatory disease entities, as it broadens the spectrum of disease-modifying drugs.

Disease activity, treatment and long-term prognosis of adult juvenile idiopathic arthritis patients compared with rheumatoid arthritis patients

Objective: To evaluate the difference between adult juvenile idiopathic arthritis (JIA, starting at <16 years) and rheumatoid arthritis (RA).Methods: Data on 128 adult JIA patients were from the National Database of Rheumatic Diseases in Japan (NinJa), 2014, divided into 4 groups by period of disease onset (Group 1: 2000–2013, n = 32; Group 2: 1981–1999, n = 32; Group 3: 1966–1980, n = 31; Group 4: ∼1965, n = 33). Disease activity, treatment and long-term prognosis of adult JIA patients were compared with RA patients matched for sex- and disease duration in each era.Results: In Groups 1 and 2, adult JIA patients had significantly lower clinical disease activity indices (CDAI) (Group 1: adult JIA 1.5 [0.4–6.9]-vs-RA 5.3 [2.5–10.3], p = .001, Group 2: 2.6 [0.6–9.0]-vs-6.9 [3.5–11.0], p = .001, shown as median [quartile range], p-value, respectively), and had higher CDAI remission rates than RA patients (Group 1: 54.8%-vs-28.2%, p = .002, Group 2: 51.7%-vs-17.0%, p < .001). More adult JIA than RA patients in Group 1 used biologics (62.5%-vs-24.7%, p < .001). However, there were no adult JIA-vs-RA differences in joint destruction and physical function in any group.Conclusions: Adult rheumatologists must recognize that adult JIA patients are different from RA patients even when disease duration is the same.

Non-steroidal anti-inflammatory drug induces luteinized unruptured follicle syndrome in young female juvenile idiopathic arthritis patients.

To assess prospectively luteinized unruptured follicle (LUF) syndrome in juvenile idiopathic arthritis (JIA) patients with and without non-steroidal anti-inflammatory drugs (NSAIDs) and healthy controls. Twenty-three adolescent and young adult female JIA patients (ILAR criteria) and 11 female healthy subjects were studied by pelvic ultrasound monitoring for follicular development and ovulation in one menstrual cycle. LUF syndrome was prospectively investigated by pelvic ultrasound with a dominant ovarian follicle without signs of follicular rupture, with elevation of serum progesterone in the luteal phase of the menstrual cycle and luteinizing hormone (LH) detected in the urine. Comparison between JIA patients with (n = 8) vs. without NSAIDs (n = 15) and healthy controls (n = 11) revealed that LUF syndrome was significantly higher in the former group (2 (25%) vs. 0% vs. 0%, p = 0.049). These two patients with LUF syndrome had normal menstrual cycles without reduced ovarian reserve, and they were under naproxen 500mg bid during the menstrual cycle. Disease duration was comparable in JIA with and without NSAIDs [19.8 (4.4-25) vs. 13 (3.1-33) years, p = 0.232]. Further comparison between JIA patients with and without NSAIDs and healthy controls showed similar mean anti-Müllerian hormone levels (p = 0.909), estradiol (p = 0.436), FSH (p = 0.662), LH (p = 0.686), and mean antral follicle count (p = 0.240) and ovarian volume (p = 0.363). No differences were evidenced in three groups regarding Caucasian race, body mass index, duration, and length of menstrual cycles (p > 0.05). This is the first study to identify that JIA patients have a high frequency of LUF without impaired ovarian reserve. Future prospective studies are necessary to determine if chronic/continuous use of NSAIDs in JIA will have an impact in these patients' fertility.

Impact of Uveitis on Quality of Life in Adult Patients With Juvenile Idiopathic Arthritis.

To establish the impact of uveitis on the quality of life (QoL) in adult patients with juvenile idiopathic arthritis (JIA). Adult patients with a history of JIA, both with (n = 31) or without (n = 51) chronic anterior uveitis, were included. Their scores on 3 validated QoL questionnaires (National Eye Institute Visual Functioning Questionnaire [NEI VFQ-25], Medical Outcomes Study 36-Item Short Form health survey [SF-36], and EuroQol 5-domain questionnaire [EQ-5D]) were analyzed to find factors that could influence QoL. The median overall composite score (OCS) of the NEI VFQ-25 was significantly worse in the uveitis group compared to the non-uveitis group (respectively, 83.4 [range 15.2-94.7] and 94.9 [range 46.3-100]; P < 0.001). Nearly all subscale scores were lower in patients with uveitis than in patients without uveitis (P < 0.001 for all). After adjusting for duration of arthritis, JIA subtype, arthritis onset before or after 1990, and the use of systemic immunomodulatory medication, the QoL was still worse in patients with uveitis (NEI VFQ-25 OCS regression coefficient = -11.7; P = 0.002). No significant differences were found between the groups for the SF-36 and the EQ-5D. In the total JIA group, the use of systemic medication appeared to negatively influence some general QoL scores. Having a history of uveitis has a substantial negative effect on the vision-related QoL in JIA in adulthood, despite good visual acuity. General QoL scores did not differ between uveitis and non-uveitis patients, but the use of systemic immunomodulatory treatment, independent of uveitis, did negatively influence general QoL scores in adult JIA patients.

Long-Term Health-Related Quality of Life in German Patients with Juvenile Idiopathic Arthritis in Comparison to German General Population.

ObjectiveAims of the study were to investigate health-related quality of life (HRQOL) in adult patients with former diagnosis of Juvenile Idiopathic Arthritis (JIA), to compare their HRQOL with the general population and to identify factors related to a poor outcome.MethodsIn 2012, a cross-sectional survey was performed by mailing a questionnaire to a large cohort of former and current patients of the German Centre for Rheumatology in Children and Adolescents. Only adult patients (≥18 years) with a diagnosis compatible with JIA were included (n = 2592; response 66%). The questionnaire included information about HRQOL (EQ5D), disease-related questions and socio-demographics. Prevalence and 95% confidence intervals (CI) of problems with mobility, self-care, usual activities, pain and anxiety/depression were standardized to the German general population. Factors associated with low HRQOL in JIA patients were identified using logistic regression models.ResultsSixty-two percent of the study population was female; age range was 18–73 years. In all dimensions, JIA patients reported statistically significantly more problems than the general population with largest differences in the pain dimension (JIA patients 56%; 95%CI 55–58%; general population 28%; 26–29%) and the anxiety/depression dimension (28%; 27–29% vs. 4%; 4–5%). Lower HRQOL in JIA patients was associated with female sex, older age, lower level of education, still being under rheumatic treatment and disability.ConclusionsHRQOL in adult JIA patients is considerably lower than in the general population. As this cohort includes historic patients the new therapeutic schemes available today are expected to improve HRQOL in future.

Long-term outcome of 114 adult JIA patients in a non-pediatric rheumatology institute in Japan

Objective. To evaluate the long-term outcome of patients with juvenile idiopathic arthritis (JIA) using data from a large cohort database, Institute of Rheumatology, Rheumatoid Arthritis, managed by the Tokyo Women's Medical University.Methods. Of 182 patients identified from the database from 2000 to 2013, 114 were verified as having JIA. The transition of medical care and the contributions of biological DMARDs were evaluated.Results. The mean age of the patients (93 females, 81.6%) at the latest examination was 36.6 ± 13.3 years. The mean age at disease onset and mean disease duration were 11.6 ± 3.4 and 25.0 ± 13.3 years, respectively. Of the 114 patients, 106 (93.0%) had poly- or oligoarthritis. Only one-fourth transferred from general pediatricians or pediatric rheumatologists. More patients with recent disease onset were treated with biological DMARDs (16.7% in the 1970s, vs. 80.0% in the 2000s). Disease activity assessed with DAS28 was significantly lower when disease onset was more recent (3.9 ± 1.3 for onset in the 1960s vs. 2.2 ± 1.1 for onset in the 2000s, p = 0.04). The percentage of patients requiring orthopedic surgery has decreased (53.8% before the 1970s vs. 10.0% in the 2000s).Conclusion. Patients with more recent disease onset showed an improved outcome. Establishing and sharing a transition program among pediatric and non-pediatric rheumatologists is desirable.

FRI0543 Pregnancy Outcome in Adult Juvenile Idiopathic Arthritis Patients Treated with Biologic Agents

BackgroundBiologic drugs are effective therapeutic option in patients with juvenile idiopathic arthritis (JIA). However, data regarding their safety during pregnancy and breastfeeding in adult JIA women are rare.ObjectivesThe purpose of...

FRI0151 Steroids and leflunomide, not biologics, are the major risk factors for infection following total joint arthroplasty

BackgroundRheumatoid Arthritis (RA) patients are at risk for infectious complications following orthopaedic procedures due to underlying comorbidities and RA treatment. The effect of biologic therapies on the risk for infection...

SAT0469 Treatment of Adult Juvenile Idiopathic Arthritis Patients with Biologic Agents. Data from the Czech National Registry

ObjectivesTo analyze the efficacy and safety of biologic agent in adult patients with juvenile idiopathic arthritis (JIA).MethodsATTRA is a Czech national registry of patients with different forms of chronic arthritis...

Craniofacial growth disturbance is related to temporomandibular joint abnormality in patients with juvenile idiopathic arthritis, but normal facial profile was also found at the 27-year follow-up

Objectives: To assess the long-term outcome of craniofacial morphology related to disease variables and temporomandibular joint (TMJ) involvement as demonstrated with computed tomography (CT) and magnetic resonance imaging (MRI) in adult patients with juvenile idiopathic arthritis (JIA).Methods: Sixty of 103 patients participated in a re-examination on average 27 years after baseline. Craniofacial morphology, with emphasis on size and position of the mandible, was assessed in lateral cephalographic images and related to disease variables and TMJ involvement by CT and MRI. Definitions of craniofacial growth disturbances were based on measurements outside 2 SD from the mean of healthy adult controls.Results: Sagittal craniofacial growth disturbances were found in 57% and micrognathia in 27% of the 60 patients. Of those with JIA TMJ involvement, 70% had some form of growth disturbance. Micrognathia occurred only in patients with bilateral TMJ involvement. The bilateral TMJ group had significantly different craniofacial morphology than healthy controls and patients without TMJ involvement. Growth disturbances and TMJ involvement were present in all subtypes of JIA, except for one subtype comprising one patient. Patients with growth disturbances had more severe disease than patients with normal craniofacial growth, regarding both present and previous disease activity. Unexpectedly, half of the patients without craniofacial growth disturbances also had TMJ involvement, many from before the age of 12.Conclusions: Craniofacial growth disturbances were found to be frequent in adult JIA patients, especially in those with bilateral TMJ involvement. However, growth disturbances did not always follow TMJ involvement, not even when affected early.

Juvenile idiopathic arthritis in adulthood and orthopaedic intervention

There have been marked changes in the management of juvenile idiopathic arthritis (JIA) over recent decades, mainly with earlier use of methotrexate (MTX). Our aim was to describe orthopaedic interventions in a large group of adults with JIA followed up over several decades. This was a retrospective observational study of adult JIA patients attending a teaching hospital clinic, with information collated on JIA subtype, disease duration, orthopaedic interventions, and exposure to MTX. The study included 144 patients with median disease duration of 19 years. Survival analysis showed that joint surgery was observed in the majority (75%) of patients with disease duration over 40 years with a trend for less joint surgery in patients with oligoarticular JIA. In total, 41 patients (28.5%) had received joint surgery, and 17/41 (41%) have required multiple procedures. Of those who have required joint surgery, 20/41 (48%) had started MTX in their adult years, with only 5/41 (12%), starting MTX prior to first joint replacement and none within 5 years of disease onset. Of the patients who have not had joint surgery to date, most (46/103, 45%) were receiving MTX or another immunosuppressive agent; in the majority of cases, MTX was started within 2 years of disease onset. Many adults with JIA require joint replacement surgery and ongoing immunosuppressive treatments, emphasising that JIA is not a benign disease. Many patients who have had joint replacement surgery have had exposure to MTX albeit after many years after disease onset; it remains to be seen whether patients who have received MTX therapy early in their disease course will ultimately have less requirement for joint surgery.