Implementation and assessment of a structured curriculum for a 4-week pediatric rheumatology rotation for pediatric residents.

BACKGROUND: General pediatricians often initially address children's musculoskeletal (MSK) issues and play a crucial role in triaging and managing patients' rheumatologic conditions. This study assessed the effectiveness of a structured curriculum in enhancing pediatric residents' knowledge, MSK examination skills, and confidence during a 4-week pediatric rheumatology rotation. METHODS: Pediatric residents in their either second or third year who participated in the 4-week rheumatology rotation once across three academic years (July 2020-June 2023) were enrolled. Residents' knowledge, MSK examination skills, and confidence were assessed at pre- and post-rotation by using 25 multiple-choice questions, the Thai pediatric Gait Arms Legs Spine examination, and a questionnaire, respectively. The curriculum comprised instruction on MSK examinations, interactive lectures, case-based discussion, topic reviews, MSK radiology conference, clinical experience in rheumatology clinic and consultations, with self-guided learning with educational resources. RESULTS: Fifty-eight pediatric residents (48 females, 10 males) with a mean age of 28.9 ± 0.8 years participated. Significant improvements were noted postrotation. Knowledge scores rose from 63.0 ± 12.2 to 79.7 ± 9.1 (mean difference 16.7 ± 10.3, p < 0.001). Similarly, MSK examination scores increased from 67.5 ± 14.4 to 93.6 ± 8.7 (mean difference 26.1 ± 14.6, p < 0.001). Residents also reported a marked increase in confidence across all evaluated areas, including history taking, MSK examination, arthrocentesis, and diagnosing and treating rheumatologic conditions (p < 0.001). CONCLUSIONS: The 4-week structured curriculum in the pediatric rheumatology rotation significantly enhanced pediatric residents' knowledge, MSK examination skills, and confidence. These findings support the integration of pediatric rheumatology rotations into pediatric residency training programs.

Disability and disease-related damage in Thai children and adolescents with juvenile idiopathic arthritis.

BACKGROUND: Children and adolescents with juvenile idiopathic arthritis (JIA) may suffer from disability and disease-related damage. This study aimed to investigate the prevalence of disability and damage, and identify the factors associated with articular and extra-articular damage in children and adolescents with JIA in a resource-restricted setting in Thailand. METHODS: This cross-sectional study enrolled JIA patients during June 2019-June 2021. Disability was assessed using the Child Health Assessment Questionnaire (CHAQ) and Steinbrocker classification criteria. Damage was evaluated using the Juvenile Arthritis Damage Index (JADI) and the modified-JADI (mJADI) tools. RESULTS: There were 101 patients (50.5% female) with median age of 11.8 years. Median disease duration was 32.7 months. Enthesitis-related arthritis (ERA) was the most common subtype (33.7%), followed by systemic JIA (25.7%). Thirty-three (32.7%) patients had delayed diagnosis ≥ 6 months. Moderate to severe disability was found in 20 (19.8%) patients. Patients with Steinbrocker functional classification > class I were seen in 17.9%. Thirty-seven (36.6%) patients had articular damage. Extra-articular complications were observed in 24.8%. Growth failure and striae were the most common complications in 7.8%. Leg-length discrepancy was documented in 5.0%. Ocular damage was found in 1 patient with ERA. Multivariable logistic regression analysis revealed Steinbrocker functional classification > class I (aOR: 18.1, 95% CI: 3.9-84.6; p < 0.001), delayed diagnosis ≥ 6 months (aOR: 8.5, 95%CI: 2.7-27.0; p < 0.001), and ERA (aOR: 5.7, 95%CI: 1.8-18.3; p = 0.004) as independent predictors of articular damage. Systemic corticosteroids use was the independent predictor of extra-articular damage (aOR: 3.8, 95%CI: 1.3-11.1; p = 0.013). CONCLUSIONS: Disability and disease-related damage was identified in one-fifth and one-third of JIA patients. Early detection and treatment are essential for preventing permanent damage.

Outcomes in children with rheumatic diseases following COVID-19 vaccination and infection: data from a large two-center cohort study in Thailand.

Introduction: Vaccination against coronavirus disease 2019 (COVID-19) is effective in protecting patients from severe COVID-19 infection. Disease flare-up following immunization in children with rheumatic disorders may result in patient reluctance to receive the vaccine. Underlying rheumatic diseases or the use of immunosuppressive drugs may influence the outcomes of COVID-19 vaccination and infection. We aimed to describe outcomes in children with rheumatic diseases following COVID-19 immunization and infection. Methods: This retrospective study was performed at two large academic centers in Thailand. During the COVID-19 pandemic, all patients were routinely queried about COVID-19-related conditions. We included patients with rheumatic diseases aged <18 years who received at least one dose of a COVID-19 vaccine or had a history of COVID-19 infection with more than 6 months of recorded follow-up after the last vaccine dose or COVID-19 illness. Demographic information and data on clinical symptoms, disease activity, treatment, outcomes, and COVID-19 vaccination and infection were collected. Results: A total of 479 patients were included. Most (229; 47.81%) patients had juvenile idiopathic arthritis, followed by connective tissue diseases (189; 39.46%), vasculitis syndromes (42; 8.76%), and other rheumatic diseases (19; 3.97%). Approximately 90% of patients received at least one dose of COVID-19 vaccination, and half of the patients had COVID-19 infection. Among patients, 10.72% and 3.27% developed a flare after COVID-19 vaccination and COVID-19 illness, respectively. Flare severity after COVID immunization and infection was mainly mild to moderate. The predictor of flare after COVID-19 vaccination was the use of prednisolone ≥10 mg/day before vaccination (hazard ratio: 2.04, 95% confidence interval: 1.05-3.97, p = 0.037). Inactive disease before receiving the COVID-19 vaccination was a predictor of inactive status after a flare (hazard ratio: 2.95, 95% confidence interval: 1.04-8.40; p = 0.043). Overall, 3.36% and 1.61% of patients experienced a new onset of rheumatic disease after receiving the COVID-19 vaccine and after COVID-19 infection, respectively. Conclusion: The COVID-19 vaccine is recommended for children with rheumatic disease, particularly those who are in stable condition. After COVID-19 vaccination, patients-especially those with active disease before vaccination or those receiving concurrent prednisolone doses of ≥10 mg/day-should be closely monitored.

Outcomes of intraarticular triamcinolone acetonide injection in children with non-systemic juvenile idiopathic arthritis.

OBJECTIVES: The objectives were to explore the response to intraarticular triamcinolone acetonide (TA) injection in children with non-systemic juvenile idiopathic arthritis (JIA) and factors associated with time to arthritis flare. METHODS: This was a retrospective cohort study of children with non-systemic JIA who received intraarticular TA injections at a tertiary care hospital in Bangkok, Thailand. Response to intraarticular TA injection was defined as absence of arthritis at 6 months after procedure. Time from joint injection to arthritis flare was recorded. Kaplan-Meier survival analysis with logarithmic rank test and multivariable Cox proportional hazards regression analysis were used for outcome analyses. RESULTS: Intraarticular TA injection was performed in 177 joints among 45 children with non-systemic JIA, most common in the knees (57 joints, 32.2%). Response to intraarticular TA injection at 6 months was observed in 118 joints (66.7%). Ninety-seven joints (54.8%) had arthritis flare following injection. The median time to arthritis flare was 12.65 months (95%CI 8.20-17.10 months). The significant risk factor associated with arthritis flare was the JIA subtypes other than persistent oligoarthritis (HR 2.62, 95%CI 1.085-6.325, p = 0.032); the significant protective factor was concomitant sulfasalazine use (HR 0.326, 95%CI 0.109-0.971, p = 0.044). Adverse effects included pigmentary changes (3, 1.7%) and skin atrophy (2, 1.1%). CONCLUSION: Intraarticular TA injection in children with non-systemic JIA had favorable response in two thirds of injected joints at 6 months. The JIA subtypes other than persistent oligoarthritis was a predictor of arthritis flare following intraarticular TA injection. Key Points • Intraarticular TA injection in children with non-systemic JIA had a favorable response in two-thirds of injected joints at 6 months. • The median time from intraarticular TA injection to arthritis flare was 12.65 months. • The risk factor predicting arthritis flare was the JIA subtypes other than persistent oligoarthritis (extended oligoarthritis, polyarthritis, ERA, and undifferentiated JIA), while the concomitant use of sulfasalazine was a protective factor. • Local adverse reactions from intraarticular TA injection were less than 2% of injected joints.

Outcomes of achieving lupus low disease activity state and damage accrual in childhood-onset systemic lupus erythematosus.

INTRODUCTION: At present, the treat-to-target approach has been proposed with the lupus low disease activity state (LLDAS) as an achievable target. OBJECTIVES: To determine damage accrual and baseline clinical characteristics associated with achieving LLDAS within 12 months of treatment in patients with childhood-onset systemic lupus erythematosus (c-SLE). METHODS: This retrospective cohort study was conducted at the largest university-based tertiary referral center in Thailand. Data of c-SLE patients (≤ 18 years) at diagnosis who were followed ≥ 12 months during January 2009 to December 2019 were collected. SLE disease status was categorized into LLDAS and non-optimally controlled state. SLEDAI-2K score was used to assess disease activity. Damage accrual was assessed by a pediatric version of the SLICC/ACR damage index. RESULTS: A total of 232 c-SLE patients (85.8% female) were included. At 12 months of treatment, 109 (47%) patients achieved LLDAS. Damage accrual was observed in 93 (40.1%) patients at the mean follow-up time of 6.2 ± 3.7 years. Damage accrual was significantly lower in patients who achieved LLDAS within 12 months than in those non-optimally controlled (p = 0.002). The median time to achieving LLDAS was 12.6 months (95%CI: 11.19-13.97). The median time to achieving LLDAS was significantly shorter in those without renal involvement (10.8 months, 95%CI: 9.62-12.00 vs. 15.6 months, 95%CI: 13.76-17.52, respectively; p = 0.044). Multivariable logistic regression analysis revealed absence of renal involvement as the predictor of achieving LLDAS within 12 months of treatment (aOR: 2.430, 95%CI: 1.420-4.158; p = 0.001). CONCLUSIONS: Achieving LLDAS within 12 months of treatment was associated with lower damage accrual. Absence of renal involvement was the predictor of achieving LLDAS within 12 months of treatment. Key Points • LLDAS is a promising and achievable treatment target in c-SLE. • Achieving LLDAS within 12 months of treatment is associated with lower damage accrual. • Absence of renal involvement is the predictor of achieving LLDAS within 12 months of treatment.

Clinical profiles of post-infectious arthritis and transient synovitis of the hip in children.

BACKGROUND: Acute inflammatory arthritides can present as a result of immune reaction following infections. Post-infectious arthritis and transient synovitis of the hip in children are included in this disease entity. The aim of this study was to describe the clinical profiles of post-infectious arthritis and transient synovitis of the hip in Thai children. METHODS: A retrospective review was performed at a tertiary care hospital in Bangkok, Thailand from January 2005 to July 2017. RESULTS: Eighty-six patients (56 boys and 30 girls) were included in this study. Mean age was 8.4 ± 4.8 years. Reactive arthritis was diagnosed in two patients (2.3%) following Salmonella spp. and Chlamydia trachomatis infections. Post-streptococcal reactive arthritis was present in 10 patients (11.6%). Transient synovitis of the hip was found in 30 patients (34.9%). Forty-four patients (51.2%) were clinically diagnosed with post-infectious arthritis. Mono/oligoarthritis was the most common clinical profile (84.9%). The distribution of lower-extremity involvement was as follows: hip, 47.6%; knee, 46.5%; and ankle joints, 30.2%. The documented preceding illness consisted mostly of upper respiratory tract symptoms (30.2%). Non-steroidal anti-inflammatory drugs were prescribed for 70 patients (81.4%). CONCLUSION: Mono/oligoarthritis of the lower extremity was the main clinical profile. Preceding viral illness was documented in one-third of children. Reactive arthritis was rarely seen.

Synovial osteochondromatosis mimicking juvenile idiopathic arthritis in an adolescent: a case-based review.

Synovial osteochondromatosis is an extremely rare benign condition in children and adolescents that have joint pain as a presenting manifestation. It is usually monoarticular with the knee as the most common affected joint. In this article, we describe the case of a female adolescent suffering from debilitating chronic right knee pain initially mimicking juvenile idiopathic arthritis, who was subsequently diagnosed with primary synovial osteochondromatosis. We present a review of synovial osteochondromatosis focusing on the clinical manifestations, radiographic features, histopathologic findings, and treatment, with a summarized review of pediatric patients with initial musculoskeletal presentations who were ultimately diagnosed as synovial osteochondromatosis. Although synovial osteochondromatosis is rare in children and adolescents, this condition should be included in the differential diagnosis of joint pain and may mimic juvenile idiopathic arthritis. Appropriate diagnostic radiography, including both plain radiography and magnetic resonance imaging, is necessary to accurately diagnose this condition. We also emphasize the importance of a multidisciplinary team approach to managing patients with synovial osteochondromatosis.

Nonspecific interstitial pneumonia in refractory systemic juvenile idiopathic arthritis responded to tocilizumab treatment.

BACKGROUND: Nonspecific interstitial pneumonia (NSIP) is a rare pulmonary complication in systemic juvenile idiopathic arthritis (SJIA). OBJECTIVE: To present a case with NSIP in SJIA. METHODS: Case report. RESULTS: We report the case of a 4-year-old boy with SJIA complicated by macrophage activation syndrome (MAS) refractory to conventional therapy, and who later developed NSIP confirmed by high-resolution computerized tomography of the chest and lung histopathology. The patient received tocilizumab, a monoclonal antibody to interleukin-6 receptor, to control his disease. Data relating to tocilizumab treatment of NSIP in refractory SJIA is limited. CONCLUSIONS: The data from this case report suggests that NSIP could be a pulmonary complication of SJIA complicated by MAS that is refractory to conventional therapy. Early initiation of tocilizumab should be considered to achieve disease remission in this pediatric patient population.

Delivery of paediatric rheumatology care: a survey of current clinical practice in Southeast Asia and Asia-Pacific regions.

BACKGROUND: Paediatric rheumatic diseases are a leading cause of acquired disability in Southeast Asia and Asia-Pacific Countries (SE ASIA/ASIAPAC). The aims of this study were to identify and describe the challenges to the delivery of patient care and identify solutions to raise awareness about paediatric rheumatic diseases. METHODS: The anonymised online survey included 27 items about paediatric rheumatology (PR) clinical care and training programmes. The survey was piloted and then distributed via Survey-Monkey™ between March and July 2019. It was sent to existing group lists of physicians and allied health professionals (AHPs), who were involved in the care pathways and management of children with rheumatic diseases in SE ASIA/ASIAPAC. RESULTS: Of 340 participants from 14 countries, 261 participants had been involved in PR care. The majority of the participants were general paediatricians. The main reported barriers to providing specialised multidisciplinary service were the absence or inadequacy of the provision of specialists and AHPs in addition to financial issues. Access to medicines was variable and financial constraints cited as the major obstacle to accessing biological drugs within clinical settings. The lack of a critical mass of specialist paediatric rheumatologists was the main perceived barrier to PR training. CONCLUSIONS: There are multiple challenges to PR services in SE ASIA/ASIAPAC countries. There is need for more specialist multidisciplinary services and greater access to medicines and biological therapies. The lack of specialist paediatric rheumatologists is the main barrier for greater access to PR training.

The Thai Translation of the Pediatric Gait, Arms, Legs, Spine Tool is Useful for Pediatric Residents in Detecting Musculoskeletal Abnormalities in Children.

BACKGROUND: The pediatric Gait, Arms, Legs, Spine (pGALS) tool is used to screen musculoskeletal (MSK) abnormalities in children. This study aimed to evaluate the application of the Thai translation of pGALS tool in outpatient clinical settings. METHODS: This cross-sectional study included patients aged 4 to 16 years, recruited from the Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand. Pediatric residents performed the Thai pGALS tool, and a pediatric rheumatologist performed the MSK examination. RESULTS: One hundred Thai patients were enrolled with a mean age of 9.87 ± 3.01 years. Forty percent of patients had MSK complaints. The median duration of the assessment was 3.96 minutes (interquartile range, 2.07-5.84 minutes). Abnormal pGALS examination by pediatric residents was found in 43 patients, all of which had abnormal MSK examination by a pediatric rheumatologist. Seventeen patients did not have abnormal pGALS examination by pediatric residents, but had abnormal MSK examination confirmed by a pediatric rheumatologist; these patients all had noninflammatory/mechanical conditions. The 3 screening questions of the Thai pGALS tool had a sensitivity and specificity of 71.67% and 77.5%, respectively; the examination had a sensitivity and specificity of 74.14% and 100%, respectively. For acceptability, 99% of parents and 89% of patients reported no discomfort. For practicality, 99% of parents and 89% of patients reported the highest level of practicality. CONCLUSIONS: The Thai pGALS tool is useful for MSK screening in children performed by pediatric residents in outpatient clinical settings. Adding maneuvers to the pGALS examination to detect more common noninflammatory MSK conditions would be beneficial.

Takayasu arteritis with an initial presentation of chronic monoarthritis mimicking oligoarticular juvenile idiopathic arthritis.

Patients with Takayasu arteritis (TA) generally present with non-specific symptoms that, if unrecognized and untreated, may develop vessel stenosis and/or aneurysm. There is limited data regarding chronic monoarthritis as the initial presentation in children with TA. We report a 6-yearold girl diagnosed and treated as oligoarticular juvenile idiopathic arthritis (JIA). She later developed stroke with malignant hypertension and was definitively diagnosed with TA. She additionally developed proteinuria secondary to focal segmental glomerulosclerosis. This is the report of a patient with chronic monoarthritis mimicking oligoarticular JIA which chronic monoarthritis was the presentation of TA. Since JIA is a diagnosis of exclusion, any atypical features of oligoarticular JIA should illuminate the possibility of an alternative diagnosis. Our literature review focused on musculoskeletal presentations of children with TA.

Corrigendum to: Increased high molecular weight adiponectin, but decreased total adiponectin and kisspeptin, in central precocious puberty compared with aged-matched prepubertal girls.

The aim of the present study was to compare serum leptin, kisspeptin, total adiponectin, high molecular weight (HMW) adiponectin and neuropeptide Y (NPY) levels between girls with central precocious puberty (CPP; n=26, 7-9.5 years old) and age-matched controls (n=29) including or excluding obese girls. Leptin and NPY levels were comparable between CPP and control girls. Kisspeptin levels were lower in the CPP than control group, and were positively correlated with oestrogen in the control group and with systolic and diastolic blood pressure in the CPP group. Kisspeptin levels were negatively correlated with FSH and LH in the CPP group. Total adiponectin levels were lower in CPP than control girls, and were negatively correlated with Tanner stage and body mass index, but positively correlated with the quantitative insulin sensitivity check index in the control group. HMW adiponectin was higher in the CPP than control group, and was positively correlated with Tanner stage and LH in all girls. Total adiponectin had a strong positive correlation with HMW adiponectin in the CPP group (r=0.915) compared with the control group (r=0.371). In conclusion, kisspeptin may be associated with increased oestrogen in prepubertal girls, but with increased blood pressure in girls with CPP. In girls entering puberty, HMW adiponectin was increased and associated with reproductive parameters. Based on these observations, HMW adiponectin probably plays an essential role in the initiation of puberty and is a candidate marker for the prediction of CPP.

Increased high molecular weight adiponectin, but decreased total adiponectin and kisspeptin, in central precocious puberty compared with aged-matched prepubertal girls.

The aim of the present study was to compare serum leptin, kisspeptin, total adiponectin, high molecular weight (HMW) adiponectin and neuropeptide Y (NPY) levels between girls with central precocious puberty (CPP; n=26, 7-9.5 years old) and age-matched controls (n=29) including or excluding obese girls. Leptin and NPY levels were comparable between CPP and control girls. Kisspeptin levels were lower in the CPP than control group, and were positively correlated with oestrogen in the control group and with systolic and diastolic blood pressure in the CPP group. Kisspeptin levels were negatively correlated with FSH and LH in the CPP group. Total adiponectin levels were lower in CPP than control girls, and were negatively correlated with Tanner stage and body mass index, but positively correlated with the quantitative insulin sensitivity check index in the control group. HMW adiponectin was higher in the CPP than control group, and was positively correlated with Tanner stage and LH in all girls. Total adiponectin had a strong positive correlation with HMW adiponectin in the CPP group (r=0.915) compared with the control group (r=0.371). In conclusion, kisspeptin may be associated with increased oestrogen in prepubertal girls, but with increased blood pressure in girls with CPP. In girls entering puberty, HMW adiponectin was increased and associated with reproductive parameters. Based on these observations, HMW adiponectin probably plays an essential role in the initiation of puberty and is a candidate marker for the prediction of CPP.