P036 Juvenile Idiopathic Arthritis in adulthood: outcomes of oligo and polyarticular forms?

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Miladi Saoussen ◽  
Makhlouf Yasmine ◽  
Fazaa Alia ◽  
Sellami Mariem ◽  
Ouenniche Kmar ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory rheumatic disease of childhood that can cause major physical disability, impairing patients' ability to lead a normal adult life. The objective of this study was to assess the outcomes of oligo- and polyarticular JIA in adulthood as well as the progression to authentic rheumatoid arthritis (RA). Methods We conducted a retrospective study including adult patients with JIA (diagnosed according to the International League of Associations for Rheumatology [ILAR]). Data collected included age, gender, disease characteristics (JIA subtype, disease duration). The progression to an authentic RA was determined by checking the response to the ACR/EULAR 2010 classification criteria. Results Twenty-eight patients were included in this study. There was a female predominance with a sex ratio of 1.5. The mean age of disease onset was 6 years and 2 months [2–17]. The mean age at inclusion was 29.5 years [18–64]. The frequency of each JIA subtype was as follows: polyarticular with RF + (n = 14), polyarticular with RF- (n = 9), oligoarthritis (n = 5). The polyarticular forms: RF+ and RF- evolved into genuine seropositive and seronegative RA in 71.4% and 66.7% of cases, respectively. Among the oligoarticular JIA subtype, an extension of the disease to a seronegative RA was noted in one patient (20%). Hip involvement was noted in 20% of patients. Carpal tunnel syndrome and elbow arthritis were found in 28.5% and 14.3% of patients, respectively. Regarding treatment modalities and at the time of recruitment, 36.3% of RAs were treated with Methotrexate, and 33% were on biological treatment. Ten percent of the patients dropped out of school because of disease flares and deformities. Conclusion Our study showed oligo- and polyarticular JIA evolves in most of the cases towards an authentic RA causing deformities and handicaps. Earlier management is necessary to allow a better transition to adulthood.

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1397.3-1398
Author(s):  
S. Miladi ◽  
M. Yasmine ◽  
S. Mariem ◽  
A. Fazaa ◽  
K. Ouenniche ◽  
...  

Background:Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood that may lead to physical disability and reduced quality of life, thus hindering the ability of the patients to achieve a meaningful adult life.Objectives:The aim of this study was to assess the trends of the disease and socio-professional future.Methods:We conducted a retrospective study including adult patients aged > 18 years with a history of JIA according to the International League of Associations for Rheumatology (ILAR). Collected data included age, sex, the characteristics of the disease (subtype of JIA, disease duration). The level of education, marital status as well as the profession were recorded.Results:The study included 32 patients with a female perdominance: sex ratio was 1,5. The mean age was 29,5 years old [18-64]. The mean age of onset of the disease was 6 years and 2 months [2-17]. The frequency of each JIA subset was at follows: polyarticular with rheumatoid factor (n= 14), polyarticular without rheumatoid factor (n=9), systemic (n= 2), enthesitis-related arthritis (n=7), oligoarthritis (n= 5). Four patients suffered from bilateral cataract due to corticosteroid intake. Polyarticular RF+ and RF- progressed into an authentic seropositive and seronegative RA in 71.4% and 66.7% respectively. Among oligoarticular subtype, an extension of the disease to a polyarticular FR+ form (n=1) and to a seronegative rheumatoid arthritis (n=1) was noted. Systemic JIA forms remained in remission with an articular involvement. All the patients with ERA developed spondylarthritis. Although forty–two percent of the patients were married, only half of them had children. Ten percent of patients stopped attending school because of disease flares and deformation. A university level was found in 16% of cases. Only Thirty patients had a profession. Of the patients, 74.4% had received disease-modifying anti-rheumatic drugs (DMARDs) and 36.3% of them were still taking a DMARD. Ten patients were on biologics. Severe disability was found in 20% of patients and concerned mainly the hip (57,2%), the wrist (28,5%) and the elbows (14,3%). Prosthetic joint replacement was found in 2% of cases with a revision of the latter in one patient.Conclusion:Adults with JIA often have significant levels of disability, usually related to severe joint complications. There is a clear requirement for a better transition to adulthood and a socio-professional rehabilitation.Disclosure of Interests:None declared


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
S Miladi ◽  
S Bouzid ◽  
A Fazaa ◽  
L Souabni ◽  
M Sellami ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) is a heterogeneous group of rheumatic diseases characterized by onset before the age of 16. Since the disease starts at an early age, it may lead to socio-professional difficulties in adulthood for JIA patients. This study aimed to describe the professional outcome of a series of 20 JIA patients. Methods A cross-sectional study including patients aged 20 years and more and fulfilling the ILAR criteria for the diagnosis of JIA was carried out. Telephone interviews were conducted. The responders answered questions about family status, current occupation, working h, eventual workplace adjustments, and sick leave frequency. Results Twenty patients answered the questionary; 14 males and 6 females. The mean age of the disease onset was 8 years. The mean age of patients at the time of the study was 24.27 years [20–36]. Polyarticular form was the most frequent (10 cases). Other subtypes diagnosed were systemic (4 cases), enthesitis-related arthritis (5 cases), oligoarticular (one case). Hip arthritis was observed in 8 patients and surgical intervention was needed in three. Eight patients were treated with csDMARDSs and 12 with bDMARDs. Three of our patients were married (aged 24, 34, and 36). Five were still studying: 4 had good grades without absenteeism. However, one patient needed special aid to go to school and had a higher absence rate. Five other patients were full-time college students without absenteeism. Four patients were college graduates. Among them, two were searching for a job for >6 months. The other two were full-time administrative workers with no absenteeism. One patient did need workplace adjustments. Six patients could not work because of their physical disabilities. Conclusion According to our results, a quarter of our patients could not access professional life. Disease activity and hip destruction are the two main factors causing JIA patients to miss out on important personal and professional opportunities.


2011 ◽  
Vol 38 (5) ◽  
pp. 954-958 ◽  
Author(s):  
MICHELLE BATTHISH ◽  
BRIAN M. FELDMAN ◽  
PAUL S. BABYN ◽  
PASCAL N. TYRRELL ◽  
RAYFEL SCHNEIDER

Objective.Hip involvement occurs in 20%–40% of all cases of juvenile idiopathic arthritis (JIA). Patients with systemic JIA (sJIA) are affected most frequently. The aim of our study was to investigate the predictors of clinical hip disease and radiographic hip damage in sJIA.Methods.The medical records (1997–2007) of all children (n = 98) with sJIA were reviewed. Potential clinical and laboratory predictors were examined at presentation and at 3 and 6 months. To account for censored observations, we used survival analysis.Results.During the study period, 59 children met our inclusion criteria. The mean age at diagnosis was 7.8 years. Thirty patients (51%) developed clinical hip disease, with 12 (20%) developing radiographic evidence of hip damage. The median time to develop clinical hip disease was 24 months. Using Kaplan-Meier estimates, 25% of patients develop radiographically evident hip damage within 43 months. At presentation, patients in whom clinical hip disease later developed had polyarthritis (hazard ratio 2.51, p = 0.01), elevated IgG (HR 1.12, p = 0.01) and IgM (HR 2.71, p = 0.02), and higher CHAQ scores (HR 1.65, p = 0.02). At 3 months after disease onset, patients in whom radiographic hip damage later developed had fever (HR 4.78, p = 0.02), polyarthritis (HR 4.63, p = 0.02), and higher CHAQ scores (HR 3.20, p = 0.005). At 6 months, polyarthritis was the strongest predictor of both clinical hip disease and radiographic hip damage.Conclusion.Half of patients with sJIA develop clinical hip disease a median time of 24 months from diagnosis. Early identification of predictors of hip disease and damage in patients with sJIA may suggest earlier, more aggressive interventions to prevent joint destruction.


2016 ◽  
Vol 12 (1) ◽  
pp. 90-95 ◽  
Author(s):  
Marika Saarela ◽  
Satu Mustanoja ◽  
Johanna Pekkola ◽  
Tiina Tyni ◽  
Juha Hernesniemi ◽  
...  

Background and purpose Moyamoya vasculopathy, a rare steno-occlusive progressive cerebrovascular disorder, has not been thoroughly studied in Caucasian populations. We established a registry of Finnish patients treated at the Helsinki University Hospital, to collect and report demographic and clinical data. Methods We collected data both retrospectively and prospectively from all the patients with a moyamoya vasculopathy referred to our hospital between January 1987 and December 2014. All patients underwent a neurological outpatient clinic visit. Results We diagnosed 61 patients (50 females, 10 children) with moyamoya vasculopathy. The mean age at the disease-onset was 31.5 ± 17.9 years. The two most common presenting symptoms were ischemic stroke (n = 31) and hemorrhage (n = 8). Forty-four percent underwent revascularization surgery, and 70% were prescribed antithrombotic treatment. Conclusions The results support in part the Western phenotype of the disease considering the later presentation and larger female predominance compared to the Asian moyamoya vasculopathy reports. However, the proportion of ischemic strokes and hemorrhagic strokes is closer to Japanese population than German population. The absence of familial cases points to a different genetic profile in the Finnish patients.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Makhlouf Yasmine ◽  
Wafa Triki ◽  
Kaouther Maatallah ◽  
Hanene Ferjani ◽  
Dorra Ben Nessib ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) is characterized by a widely variable clinical course and outcome. If uncontrolled, joint damage may occur. In this context, coxitis is a feared complication. The aim of our study was to determine the prevalence and patterns of hip involvement in Tunisian JIA patients. Methods A retrospective study including children with JIA according to the International League of Associations for Rheumatology (ILAR)) was conducted between 2012and 2021. Sociodemographic data as well as disease characteristics were collected. Laboratory markers including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were recorded. Hip involvement was assessed clinical exam and imaging (standard radiograph, ultrasound or magnetic resonance imaging (MRI)). We compared these parameters between the two groups: G1: presence of coxitis and G2: absence of coxitis. Results The study included 40 patients with a male predominance: sex ratio was 1.6. The mean age was 11.1 years-old [3–16]. The mean age at onset of the disease was 18.1 years old [8–30]. The distribution of the different subsets was as follows: polyarticular with rheumatoid factor (n = 1), polyarticular without rheumatoid factor (n = 2), enthesitis-related arthritis (n = 29), oligoarthritis (n = 7), psoriatic arthritis (n = 1). Extra-articular manifestations were found in 21.2% of cases: ocular (n = 4), pulmonary (n = 2) and cardiovascular (n = 1). The mean ESR and CRP was 30.9 mm/h [2–90] and 15.8 mg/l [1–70] respectively. A high ESR or CRP were found in 67% of cases. Hip involvement concerned 70% of the patients and was bilateral in 67.9% of them. Hip radiographs were normal in 50% of cases. Ultrasound was performed in 9 patients and revealed a positive Doppler synovitis (n = 2), a negative Doppler synovitis (n = 7) and joint effusion (n = 2). MRI was performed in 20% of cases and revealed synovitis (67%) and joint effusion (33%). Overall, 79.3% of patients had medical treatment combining NSAIDs and rehabilitation, 39% of the patients had had local infiltration with Hexatrione and only two patients had hip replacement. Hip involvement was not correlated with age at onset (P = 0.2), subtype (P = 0.8), sex (P = 0.7), extraarticular manifestations (P = 0.4). Similarly, there was no correlation between the presence of coxitis and ESR (P = 0.07) as well as CRP (P = 0.5). Conclusion Our study showed that hip involvement is frequent among Tunisian patients with JIA. Although not correlated with disease characteristics, hip involvement should be assessed frequently and carefully.


2009 ◽  
Vol 36 (7) ◽  
pp. 1532-1535 ◽  
Author(s):  
ALFREDOMARIA LURATI ◽  
ALESSANDRA SALMASO ◽  
VALERIA GERLONI ◽  
MAURIZIO GATTINARA ◽  
FLAVIO FANTINI

Objective.To evaluate disease course and clinical usefulness in some categories of juvenile idiopathic arthritis (JIA) by applying newly developed Wallace definitions of remission off drugs.Methods.In a retrospective study, charts of patients with chronic form of primary (idiopathic) arthritis followed from our center since 1970 were reviewed and clinical/laboratory variables were collected for further analysis.Results.The cohort included 761 eligible patients [516 (67.8%) female, 245 (32.2%) male] with JIA. Mean disease onset age (± standard deviation) was 6.25 ± 4.4 years (range 0.5–15.9). Disease mean duration to last visit was 10.02 ± 4.31 years. Followup mean period was 7.6 ± 6.4 years (range 1.5–35 yrs). A total of 247 (32.46%) patients achieved remission according to criteria [persistent oligoarthritis 153 (42.9%); extended oligoarthritis 15 (13.1%); seronegative polyarthritis 21 (22.4%); systemic arthritis 33 (33.7%); enthesitis related arthritis (ERA) plus juvenile psoriatic arthritis (JPsA) 25 (33.4%)]. No patients with seropositive polyarthritis achieved remission status (p < 0.001). In remitted patients the mean survival function (± standard error of the mean) before relapse calculated by Kaplan-Meier was of 20.9 (± 1.3) months overall: 21.7 (± 0.46) in persistent oligoarthritis, 25.0 (± 6.6) in extended oligoarthritis, 26.7 (± 13.2) in seronegative polyarthritis, and 17.6 (± 2.44) in ERA+JPsA (p > 0.1).Conclusion.In our cohort about one-third of cases obtained a remission episode in 4 decades of observation, with a significant difference between oligoarthritis and other categories (p < 0.001) using the Kaplan-Meier method; the remission status duration before a relapse has been about 20 months, without a significant difference between JIA categories.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1396.2-1396
Author(s):  
N. Ben Chekaya ◽  
S. Bouden ◽  
A. Ben Tekaya ◽  
O. Ben Saidane ◽  
R. Tekaya ◽  
...  

Background:Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of unknown aetiology in childhood. Approximately 40- 60% of patients with juvenile idiopathic arthritis (JIA) have continuous or recurrent disease activity extending into adulthood.Objectives:The aim of this study was to evaluate the clinical, biological and radiological course of JIA in adulthood.Methods:A retrospective study including 35 patients was conducted between 2010 and 2019. The patients enrolled met the ILAR criteria for the diagnosis of JIA. Patients with JIA older than 20 years were included. Data regarding sociodemographic features, Physical growth, disease activity, biological and radiological parameters were analysed.Results:Thirty one patients were recruited. The sex ratio was 0.53. The mean age at the time of the study was 38.9 years [20-69]. The mean age of the disease onset was 9 years [3-16]. These patients were assigned to discrete JIA categories: rheumatoid factor positive polyarthritis (47.7%), rheumatoid factor negative polyarthritis (23.9%), oligoarthritis (14.2%), enthesitis-related arthritis (9.5%), and psoriatic arthritis (4.7%).A failure to thrive was seen in 33.3% of patients. Overweight and obesity were found in 38% and 19% of patients, respectively. Biological inflammatory syndrome was noted in 52% of patients, and 65.2% had active disease. Hip involvement was noted in 43.5% of patients and 17.4% of them had a total hip replacement. Sixteen patients had neck pain and the imagining showed an atloid-axoid dislocation in 50% of them. Radiographs showed a joint destruction in 60.9% of patients and a wrist arthritis was the most frequent involvement.Conclusion:Most of our included patients maintain active disease and have functional impairment in adulthood.Disclosure of Interests:None declared


2020 ◽  
Author(s):  
Céline La ◽  
Phu Quoc Lê ◽  
Alina Ferster ◽  
Laurence Goffin ◽  
Bernard Lauwerys ◽  
...  

Abstract BackgroundJuvenile idiopathic arthritis (JIA) represents a very heterogeneous disease, and our objectives were to describe the first Belgian cohort of children with JIA, assess their disease characteristics and outcome and identify potential markers of prognosis.MethodsThe CAP48 cohort is a multicentric observational study of children with a recent or well-established diagnosis of JIA (naïve or not to treatment at baseline), evaluated every 6 months during a follow-up of 5 to 10 years.ResultsThere were 125 children included in the cohort, composed of 25 naïve and 100 established patients. The patients had a median age of 6.2 and 4.2 years at onset in the naïve and established cohort respectively, with a predominance of female. All subtypes of JIA were represented in both cohorts. The mean DAS28-CRP and JADAS10-CRP at baseline in naïve patients was 2.52 and 6.0 respectively. Uveitis occurred in 19% of patients and was strongly associated with presence of antinuclear antibodies (odds ratio of 6). Fifty-five percent of naïve patients were in remission at 12 months of follow-up according to the ACR criteria and JADAS10 scores, in contrast with 100% achieving DAS28 remission. ConclusionThis first cohort study in Belgium allowed to compare its data to other existing cohorts and to evaluate quality of care in Belgian French-speaking hospitals. Additionally, it highlighted a superiority of JADAS10 over DAS28 to monitor and evaluate remission in JIA. This study also underlined a need for more accurate markers of prognosis to improve treatment and long-term outcomes.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
S Miladi ◽  
S Bouzid ◽  
A Fazaa ◽  
L Souabni ◽  
M Sellami ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease characterized by onset before the age of 16. This term encompasses several disease categories, each of which has distinct methods of presentation, clinical signs, and prognosis. The study aimed to determine JIA complications in 51 patients. Methods A cross-sectional study including patients diagnosed with JIA according to ILAR criteria was conducted for 26 years [1995– 2021]. Epidemiological, clinical, therapeutic, and evolutive aspects were noted. Results Twenty-nine males and 22 females were included. The mean age of the disease onset was 7.6 years [1,5–16]. The mean age of patients at the time of the study was 23.29 years [9–45]. Polyarticular and seronegative form was the most frequent (34.5%). Other subtypes diagnosed were systemic (25%), enthesitis-related arthritis (21.2%), oligoarticular (12.5%), and seropositive polyarticular (5.8%). Standard X-Ray imaging showed articular damage in 50% of the cases. Hip arthritis was observed in 32% and surgery was needed in 16.9%. One patient presented with atlantoaxial subluxation. Growth retardation was noted in 28.6%. Cardiac manifestations were seen in 3 patients (pericarditis = 2, myocarditis = 1), uveitis in 3 cases, renal manifestation (extra membranous glomerulonephritis) in one patient with polyarticular form. One patient was diagnosed with multiple sclerosis. Small doses of corticosteroids were prescribed in 71.7%. Methotrexate was prescribed in 70.5% (interrupted for adverse effects in 3 patients), sulfasalazine in 30.6%, hydroxychloroquine in 5.7%, leflunomide in 15.4%. bDMARDs were needed in 16 patients: 14 patients received TNF alpha inhibitors, rituximab was prescribed for one patient with a polyarticular form, and tocilizumab in a patient with a systemic form. A switch of bDMARDs was conducted in 10 patients: for inefficiency in 4 cases and adverse effects in other 4 cases. Three patients developed uveitis under Etanercept, septicemia under Adalimumab, an allergic reaction, and depression under Infliximab. One patient died from a convulsive seizure at the age of 9. Conclusion The presence of complications is an additional burden to JIA patients. A multidisciplinary approach is required for the management of these complications.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Hiba Bettaieb ◽  
Wafa Triki ◽  
Kaouther Maatallah ◽  
Hanene Ferjani ◽  
Dorra Ben Nessib ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in the pediatric population. Methotrexate (MTX) has been considered as the cornerstone of treatment of poly and oligoarticular subtypes of JIA. However, this treatment is supposed to be for long term, which may involve an obstacle for adherence. The aims of the study were to evaluate adherence of Tunisian JIA patients to MTX and to identify factors associated with high adherence to MTX. Methods A cross-sectional study including patients with confirmed JIA diagnosis, according to the International League of Associations for Rheumatology (ILAR) criteria, was performed. Demographic data as well as disease characteristics were obtained from medical records. Laboratory markers including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were collected. Adherence was measured, for patients under MTX for &gt;3 months, using the 5-item Compliance Questionnaire Rheumatology (CQR5) (1). Patients were divided into two groups: group 1: High adherence (HA) for patients having a CQR5 ≥ 80% and group 2: low adherence (LA) for patients having CQR5 &lt; 80%. A p inferior to 0.05 was considered statistically significant. Results The study included 29 patients (10 males and 19 females) with a mean age at disease onset of 9.1 ± 3.4 years. The mean disease duration was 61 ± 79 months [7–336]. JIA subtypes were in decreasing order of frequency as follows: enthesitis-related arthritis (n = 13), oligoarticular (n = 8), Polyarticular without rheumatoid factor (n = 4), Polyarticular with rheumatoid factor (n = 2), systemic (n = 1) and, psoriatic arthritis (n = 1). A biologic inflammatory syndrome was found in 48.3% (n = 14) of cases. The mean ESR and CRP were 20 mm/h ± 11.3 [3–98] and 5 ± 17.8 mg/l [0–56] respectively. Nineteen (65.5%) patients had coxitis. Overall, 55.17% of patients (n = 16) were treated with MTX with a mean weekly dose of 9.2 ± 3.2 mg [5–15]. MTX was orally administrated in all patients. NSAIDs and prednisone were prescribed in 51.7% (n = 15) and 17.2% (n = 5) of cases respectively. The MTX was associated with biological DMARDs in five patients (17.2%). It was about Etanercept in 4 patients and Tocilizumab in 1 patient. Mean CQR5 score was 70.8% ± 18 [25–100]. Only seven patients (43.8%) showed high adherence to MTX. The statistical study revealed no difference between HA and LA in term of gender (P = 0.84), age at disease onset (P = 0.39), disease duration (P = 0.9), prednisone use (P = 0.22), the occurrence of coxitis (P = 0.2), ESR (P = 0.83) and CRP (P = 0.033) rates. Conclusion In this study, less than one half of JIA patients were highly adherent to MTX according to CQR5. Low adherence should be considered before declaring MTX treatment failure.


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