scholarly journals AB0729 CLINICO-EPIDEMIOLOGICAL PROFILE OF JUVENILE IDIOPATHIC ARTHRITIS (JIA) PATIENTS IN KENYA; DATA FROM THE KENYA PEDIATRIC RHEUMATOLOGY (KAPRI) REGISTRY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1395.2-1395
Author(s):  
A. Migowa ◽  
R. Odhiambo ◽  
J. Orwa ◽  
J. Shah
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Rheumatoid Factor ◽  
Clinical Features ◽  
Rheumatic Diseases ◽  
Pediatric Rheumatology ◽  
Antinuclear Antibody ◽  
Clinical Characteristics ◽  
The Other ◽  
Biological Therapies ◽  
Systemic Jia

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

P010 Clinico-epidemiological characteristics and outcomes of Juvenile Idiopathic Arthritis (JIA) patients in Kenya: data from the KAPRI registry

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
A Migowa ◽  
R Odhiambo ◽  
J Orwa ◽  
J Shah
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Rheumatoid Factor ◽  
Clinical Features ◽  
Antinuclear Antibody ◽  
Well Being ◽  
The Other ◽  
Biological Therapies ◽  
Systemic Jia ◽  
Epidemiological Characteristics ◽  
The Impact

Abstract Background Pediatric rheumatic diseases pose a significant disease burden upon children and their families (1–3). They lead to physical disability and diminished quality of life (1–3). Determination of the burden and clinical characteristics of these diseases is a critical first step to improving access to care and optimizing use of existing health systems for the well-being of these patients (4). This is in line with the goal of the Bone and Joint decade (2000–2010) aimed at increasing recognition and understanding of the impact of musculoskeletal diseases (5,6). The Kenya Pediatric Rheumatology Registry (KAPRI) offers a unique opportunity to pioneer and spearhead a systematic and organized format to inform policy and better healthcare provision. Our objective was to determine the patient characteristics, clinical features and outcomes of Juvenile Idiopathic Arthritis (JIA) patients assessed at the Aga Khan University Medical College East Africa from March 2019 to December 2020. Methods Data of JIA patients on age, gender, laboratory and clinical features at diagnosis and treatment options offered were extracted from the database. A further detailed chart review was undertaken to determine the proportion of patients who achieved remission or minimally active diseases. Results Among the 207 patients enrolled thus far, 16 (7.7%) were diagnosed to have JIA. Majority of the patients were females (75%; n = 12) with a mean age of 7 years and 3 months (Range : 1 year—13 years 7 months). All patients had joint pain and swelling as the initial presenting complaints. Majority of the patients had polyarticular JIA (75%, n = 12). The other 4 patients were oligoarticular (n = 2) and systemic JIA (n = 2). Among the polyarticular JIA patients (n = 12), only 3 (25%) were rheumatoid factor (RF) positive and 1 was antinuclear antibody (ANA) positive. The oligoarticular and systemic JIA patients were all negative for antinuclear antibody, rheumatoid factor and cyclic citrullinated peptide antibodies (anti-ccp). Seven patients (43.8%) required biological therapies; tocilizumab (n = 2: systemic JIA), adalimumab (n = 2: polyarticular JIA), etanercept (n = 2: polyarticular JIA) andtofacitnib (n = 1: polyarticular JIA). One patient with systemic JIA on tocilizumab developed herpes simplex which was successfully managed with oral acyclovir. All the other patients did not develop any infections, allergic reactions or any other untoward events as adverse outcomes following the use of biological therapies. Five patients have attained remission as illustrated in the table below. Two patients have been lost to follow up. Conclusion Seronegative polyarticular JIA was the predominant form of JIA observed with a predilection to affect more girls and boys. Over a period of 2 years, remission has been attained among 31.25% of the patients (5 of 16) with use of synthetic disease modifying anti-rheumatic drugs and biological therapies.

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

2017 ◽  
Vol 23 (1) ◽  
Author(s):  
Marta Dzhus
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Rheumatoid Factor ◽  
Rheumatic Diseases ◽  
Damage Index ◽  
Health Assessment ◽  
Juvenile Arthritis ◽  
Adult Age ◽  
Enthesitis Related Arthritis ◽  
Long Term Observation

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

scholarly journals STAT4 rs7574865 G/T and IRF5 rs2004640 G/T polymorphisms as markers of predisposition to juvenile idiopathic arthritis. What can genetics give to understand its heterogeneity?

2019 ◽  
Vol 13 (4) ◽  
pp. 55-60
Author(s):  
E. S. Fedorov ◽  
M. Yu. Krylov ◽  
S. O. Salugina ◽  
E. Yu. Samarkina ◽  
A. N. Latypova
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
High Risk ◽  
Antinuclear Antibody ◽  
Pediatric Population ◽  
Human Leukocyte ◽  
Control Group ◽  
Entire Group ◽  
Hla B27 ◽  
Systemic Jia ◽  
Allele Specific

Juvenile idiopathic arthritis (JIA) is a multifactorial immune-mediated inflammatory disease in childhood, the most common type of rheumatic disease in children. It is characterized by the polygenic type of hereditary predisposition.Objective: to study the association of STAT4 rs7574865 G/T and IRF5 rs2004640 G/T polymorphisms with the predisposition to certain JIA subtypes in the Russian pediatric population.Patients and methods. The investigation enrolled 177 patients, including 66 patients diagnosed with JIA and 111 healthy unrelated volunteers (a control group). Of the 66 patients with JIA there were 30 (45%) with oligoarthritis: 20 (67%) with human leukocyte antigen B27(HLA-B27)-positive JIA (that was associated with enthesitis, HLA-B27 positive JIA (JIA-B27), 10 (33%) with anterior uveitis concurrent with antinuclear antibody-positive JIA (JIA-uveitis); 20 (30%) with polyarticular JIA (JIA-poly), seronegative for rheumatoid factor; and 16 (24%) with systemic JIA (JIA-sys). As a control for genotyping STAT4 rs7574865 G/T and IRF5 rs2004640 G/T polymorphisms, the investigators studied 103 and 111 DNA samples from healthy adult volunteers, respectively. STAT4 rs7574865 G/T and IRF5 rs2004640 G/T polymorphisms were investigated using allele-specific real-time polymerase chain reaction (RT-PCR).Results and discussion. In the oligoarticular JIA group, the frequency of the STAT4 T allele was significantly higher than that in the control group (38.3 and 20.4%, respectively; p=0.004). This allele was also significantly more common in the JIA-B27 (35.0 and 20.4%, respectively; p=0.044) and JIA-uveitis (45.0 and 20.4%, respectively; p=0.021) groups compared with the control one. No significant differences were found in the frequency of the mutant STAT4 T allele between the control group and the JIA-sys and JIA-poly groups. Regression analysis showed that the identification of the STAT4 T allele was associated with the high risk of a predisposition to oligoarticular JIA as a whole (odds ratio, OR 2.43; 95% confidence interval (CI) 1.23–4.70; p=0.007), as well as to the antinuclear antibody-positive oligoarticular JIA with uveitis (JIA-uveitis): the risk in T allele carriers was 3.2 times higher than that in the control (OR 3.19; 95% CI 1.09–9.06; p= ). A high risk for predisposition was also found in the JIA-B27 subgroup compared with the control (OR 2.10; 95% CI 0.38–4.60; p=0.070). There were no statistical differences in the frequency of genotypes and alleles of the IRF5 rs2004640 G/T polymorphism between the entire group of JIA as a whole and its individual clinical types, as well as the control group.Conclusion. This pilot study confirmed that the STAT4 rs7574865 G/T polymorphism was associated with the risk of oligoarticular JIA, mainly that of JIA-uveitis and JIA-B27.

scholarly journals Long-term follow-up on effectiveness and safety of etanercept in juvenile idiopathic arthritis: the Dutch national register

2008 ◽  
Vol 68 (5) ◽  
pp. 635-641 ◽  
Author(s):  
F H M Prince ◽  
M Twilt ◽  
R ten Cate ◽  
M A J van Rossum ◽  
W Armbrust ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Adverse Events ◽  
Rheumatoid Factor ◽  
Serious Adverse Events ◽  
American College Of Rheumatology ◽  
Systemic Jia ◽  
Long Term Follow Up ◽  
Remission Criteria

Objective:We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes.Methods:At baseline we collected patient and disease characteristics of all Dutch patients with JIA who started treatment with etanercept. Disease activity was evaluated (at start of the study, after 3 months and then yearly) according to the JIA core set of the American College of Rheumatology paediatric definition for 30, 50 and 70% improvement (ACR Pedi 30, 50 and 70). Use of etanercept and concomitant drugs was monitored. Adverse events were recorded.Results:We included 146 patients with JIA with a median follow-up of 2.5 years per patient (range 0.3–7.3). JIA subtypes represented: 27% systemic, 8% polyarticular rheumatoid factor positive, 38% polyarticular rheumatoid factor negative, 19% oligoarticular extended, 3% enthesitis-related and 5% psoriatica. Most patients (77%) met the criteria of the ACR Pedi 30 in the first 3 months of treatment. For the majority of patients this improvement was sustained; 53 (36%) of all patients met the remission criteria. No other second-line agents were needed in 43 patients. Although patients with systemic JIA responded initially less to etanercept therapy than patients from other subtypes, those who did respond showed equal effectiveness in the long term. Serious adverse events rate was low (0.029 per patient year).Conclusions:Etanercept is effective and safe in JIA, even for a large proportion of the patients with systemic JIA. The greatest improvement occurred in the first 3 months of treatment, and was sustained for a long time in most patients (up to 75 months).

scholarly journals Differences in the clinical characteristics of chronic pulmonary aspergillosis according to spirometric impairment

PLoS ONE ◽  
2021 ◽  
Vol 16 (11) ◽  
pp. e0260274
Author(s):  
Myoung Kyu Lee ◽  
Sae Byol Kim ◽  
Beomsu Shin
Keyword(s):  
Clinical Features ◽  
Clinical Characteristics ◽  
Pulmonary Function Tests ◽  
Forced Expiratory Volume ◽  
The Other ◽  
Pulmonary Aspergillosis ◽  
Younger Age ◽  
Chronic Pulmonary Aspergillosis ◽  
Low Bmi ◽  
Wbc Count

The clinical features by declining lung function remain uncharacterized in chronic pulmonary aspergillosis (CPA) patients. We investigated the clinical characteristics of CPA patients based on spirometric impairments (restrictive spirometric pattern [RSP] and obstructive spirometric pattern [OSP]) and their severity. We retrospectively analyzed medical records of CPA patients who underwent pulmonary function tests from March 2017 to February 2020. We used Global Lung Initiative 2012 equations with lower limit of normal. The clinical characteristics of patients with RSP were compared to those with OSP. Additionally, RSP patients’ characteristics were analyzed according to forced vital capacity (FVC) tertile, and OSP patients’ characteristics were analyzed according to forced expiratory volume in 1 second (FEV1) tertile. Among the 112 patients with CPA (52 [46%] with RSP and 60 [54%] with OSP), body mass index (BMI) was significantly lower in patients with RSP than in those with OSP (17.6 kg/m2 versus 20.3 kg/m2; P = 0.003), and non-tuberculous mycobacterial disease was more frequently observed in patients with RSP than in those with OSP (28.8% versus 11.7%; P = 0.004). Additionally, for patients with RSP, younger age and bilateral pulmonary lesions were more frequently observed in the first tertile group than in the other groups (P for trend: 0.025 and 0.001, respectively). For patients with OSP, low BMI, paracavitary infiltrates, and elevated WBC count were more frequently observed in the first tertile group than in the other groups (P for trend: < 0.001, 0.011, and 0.041, respectively). Differences in the clinical features of CPA patients were identified according to heterogeneous spirometric patterns and their severity. Further studies are needed to investigate the clinical significance of these findings.

scholarly journals The Question of Whether to Remain on Therapy for Chronic Rheumatic Diseases in the Setting of the Covid-19 Pandemic

2020 ◽  
pp. jrheum.200492 ◽  
Author(s):  
Randy Q. Cron ◽  
W. Winn Chatham
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Rheumatic Diseases ◽  
Pediatric Rheumatology ◽  
Cytokine Storm ◽  
Disease Modifying ◽  
Rheumatic Drug ◽  
Chronic Rheumatic Diseases

We appreciate our Italian colleagues’ interest in our editorial denoting the rheumatologist’s role in helping to diagnose and treat cytokine storm syndrome (CSS) in the setting of the Covid-19 panemic (1). It is encouraging that none of the 123 pediatric rheumatology patients (primarily juvenile idiopathic arthritis) on background biological disease modifying anti-rheumatic drug (bDMARD) therapies in Milan, Italy surveyed over a 7-week period from February 25 through April 14, 2020 (during which time Covid-19 was hyper-endemic there) had either confirmed or suspected Covid-19 (2).

scholarly journals Transient global amnesia: clinical features and prognostic factors suggesting recurrence

2019 ◽  
Vol 77 (1) ◽  
pp. 3-9 ◽  
Author(s):  
Lucas Alessandro ◽  
Ismael L. Calandri ◽  
Marcos Fernández Suarez ◽  
María L. Heredia ◽  
Hernán Chaves ◽  
...  
Keyword(s):  
Clinical Features ◽  
Clinical Characteristics ◽  
Brain Mri ◽  
Transient Global Amnesia ◽  
Recurrence Risk ◽  
The Other ◽  
Multivariate Logistic Regression Model ◽  
Risk Of Recurrence ◽  
Increased Risk ◽  
Global Amnesia

ABSTRACT The risk of recurrence of new amnesia events in patients having previously experienced transient global amnesia (TGA) ranges between 2.9-23.8%. Objective: Our objective was to search for recurrence predictors in TGA patients. Methods: Retrospective analysis to identify recurrence predictors in a cohort of 203 TGA patients from a single center in Buenos Aires, Argentina, diagnosed between January 2011 and March 2017 Clinical features and complementary studies (laboratory results, jugular vein Doppler ultrasound and brain MRI) were analyzed. Comparison between patients with recurrent versus single episode TGA was performed, applying a multivariate logistic regression model. Results: Mean age at presentation was 65 years (20-84); 52% were female. Median time elapsed between symptom onset and ER visit was two hours, with the average episode duration lasting four hours. Mean follow-up was 22 months. Sixty-six percent of patients referred to an identifiable trigger. Jugular reflux was present in 66% of patients; and 22% showed images with hippocampus restriction on diffusion-weighted MRI. Eight percent of patients had TGA recurrence. Patients with recurrent TGA had a more frequent history of migraine than patients without recurrence (37.5% vs. 14%; p = 0.03). None of the other clinical characteristics and complementary studies were predictors of increased risk of recurrence. Conclusions: Patients with migraine may have a higher risk of recurrent TGA. None of the other clinical characteristics evaluated allowed us to predict an increased risk of recurrence. Although the complementary studies allowed us to guide the diagnosis, they did not appear to have a significant impact on the prediction of recurrence risk.

scholarly journals Bone mineral density in children with juvenile idiopathic arthritis after one year of treatment with etanercept

2018 ◽  
Vol 146 (5-6) ◽  
pp. 297-302
Author(s):  
Gordana Susic ◽  
Marija Atanaskovic ◽  
Roksanda Stojanovic ◽  
Goran Radunovic
Keyword(s):  
Bone Mineral Density ◽  
Juvenile Idiopathic Arthritis ◽  
Rheumatoid Factor ◽  
Bone Mineral ◽  
Mineral Metabolism ◽  
Z Score ◽  
Mineral Density ◽  
Bone Mineral Metabolism ◽  
Systemic Jia ◽  
One Year

Introduction/Objective. Juvenile idiopathic arthritis (JIA) is the most frequent chronic inflammatory, rheumatic disease of childhood, associated with disturbance of bone mineral metabolism, which develops gradually and progressively, and if untreated eventually leads to osteoporosis in adulthood. The aim of our study was to evaluate bone mineral density (BMD) in patients with JIA treated with etanercept over a period of one year. Methods. The prospective cohort study included 94 JIA patients (66 female, 28 male), their median age being 14.77 years. BMD was measured by dual-energy X-ray absorptiometry on the lumbar spine. Disease activity was assessed using the American College of Rheumatology Pedi 50 criteria. Results. After one year of treatment with etanercept, we found a statistically significant increment in all osteodensitometry variables (p < 0.001). Annual enhancement for the whole group was as follows: bone mineral content 15.8%, BMD 7.2%, BMDvol 4.2%. Z-score improved from -0.86 to -0.58 SD at the last visit, but decreased in rheumatoid factor-positive polyarthritis patients. Patients with systemic JIA had the lowest Z-score. Z-score correlated with functional disability level. BMD was lower in the group treated with glucocorticoids. Conclusion. Our results showed significant improvement of bone mineral density in children with JIA after one year of treatment with etanercept. Rheumatoid factor-positive and systemic JIA subtypes and treatment with glucocorticoids are the risk factors for impairing bone mineral metabolism.

Sign in / Sign up

Export Citation Format

Share Document