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Abstract We report a case of a 5-year old girl, who initially presented with acute epiglottitis, sepsis and multi-organ failure. She was subsequently diagnosed as having Systemic Lupus Erythematosus. To the best of our knowledge, this article describes the first case of Haemophilus influenzae type f epiglottitis as the initial presentation of SLE in childhood.

We evaluated the immunogenicity of 300 mg Tixagevimab-Cilgavimab in immunocompromised children and adolescents who weighed 20 to >40 kg. Six to 18-year-old participants were divided into two groups by body weight and received 300 mg (20 to <40 kg) and 600 mg (≥40 kg) Tixagevimab-Cilgavimab, respectively. Anti-SARS-CoV-2 receptor-binding domain IgG concentrations and pseudovirus neutralizing antibody (NAb) titers were measured at 4, 12, and 24 weeks after administration and compared with reference data from healthy Thai children at 2 weeks after three BNT162b2 vaccinations. Of 59 participants, 49.2% were female, with a median (IQR) age of 12 (9, 15) years; 16 (27.1%) had cancer. NAb titers (95% CI) for the ancestral Wuhan strain were comparatively high for both dosing regimens (16363.2 [13765.9, 19450.5] vs 17768.3 [15539.5, 20316.9] in 20 to <40 kg and ≥40 kg participants, respectively) and significantly higher than reference titers ( P  < 0.001 for both). NAb titers for Omicron BA.4/5 were on par with the reference for both dosing regimens. Adverse events were mild, well tolerated, and slightly more prevalent in ≥40 kg participants who received full-dose Tixagevimab-Cilgavimab. Minimal waning in anti-RBD IgG concentrations, comparable to the reference, was observed at 12 and 24 weeks after Tixagevimab-Cilgavimab administration for both regimens. We concluded that half-dose Tixagevimab-Cilgavimab in 20 to <40 kg participants generated equivalent antibodies to standard doses in ≥40 kg participants and significantly higher antibodies than three-dose BNT162b2 vaccination. Further study of monoclonal long-acting antibodies in larger cohorts and <6-year-old children are warranted.

Objective: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are chronic life-threatening vasculitides requiring substantial immunotherapy. Adult trials identified rituximab (RTX) as an alternative to cyclophosphamide (CYC) for remission-induction of GPA/MPA. Disease rarity has limited feasibility of similar trials in pediatrics. We aim to evaluate the relative efficacy and toxicity of CYC and RTX for childhood GPA/MPA through registry-based comparative evaluation., Methods: From A Registry of Childhood Vasculitis we identified GPA/MPA patients who received induction with RTX or CYC. Pediatric vasculitis activity score (PVAS) and pediatric vasculitis damage index (pVDI) evaluated disease activity and damage. Descriptive statistics summarized patient characteristics. RTX/CYC comparisons used logistic regression for primary outcomes of post-induction remission (PVAS=0) or low disease activity (PVAS<2). Hospital admission for adverse events and pVDI were compared using logistic regression and ordinal regression, respectively., Results: Among 104 patients, 43% received RTX, 46% CYC, 11% both. Treatment groups did not significantly differ for diagnosis PVAS and onset age. There was no difference in remission between groups (63% overall; OR 1.07, 95% CI: 0.45, 2.52). Hospitalizations occurred in 22% of RTX patients versus 10% on CYC (OR 2.27, 95% CI: 0.73, 7.05). The median 12-month pVDI was one in both groups (OR 0.98, 95% CI 0.43, 2.22)., Conclusion: This is the first study comparing CYC and RTX for induction in pediatric GPA/MPA. No significant differences were shown in rates of remission, severe adverse events, or organ damage. Limitations included lack of standardized treatment regimens, retrospectivity, and lack of longitudinal adverse drug-related event data., (This article is protected by copyright. All rights reserved.)

Background: The COVID-19 pandemic has significantly impacted individuals with chronic conditions. This investigation assessed the quality of care provided to pediatric and adolescent patients with juvenile idiopathic arthritis (JIA) during the pandemic in Thailand., Methods: This cross-sectional analysis enrolled JIA patients aged ≤ 18 years at an academic tertiary care facility from April 2022 to March 2023. Retrospective reviews were performed, complemented by patient and caregiver questionnaires to assess the pandemic's impact on care quality., Results: Seventy JIA patients (37 males, 33 females) with a mean age of 13.5 ± 3.1 years were included. A total of 41.4% of the caregivers reported negative impacts on JIA care due to the pandemic and the lockdown, and 31.4% of the patients experienced pandemic-related anxiety. A comparison between the pandemic and prepandemic periods revealed a higher incidence of active disease, although the difference was statistically nonsignificant (37.1% vs 14.2%, p = 0.106). Nonadherence significantly predicted active disease status (adjusted OR 15.04, 95% CI 2.48-91.15, p = 0.03). COVID-19 vaccinations were administered to 85.7% of patients; 52.8% of whom contracted mild COVID-19. Most patients (71.4%) postponed clinic visits; 36% due to lockdowns and 28% due to concerns about COVID-19 exposure in healthcare settings. The majority of patients received telephone JIA management advice from rheumatologists during the lockdown (91.4%)., Conclusions: The COVID-19 pandemic and associated lockdown measures affected the care of JIA patients, impacting both physical and mental health. Nonadherence was a critical factor in disease flare-ups. Telemedicine is indispensable for patient care., (© 2024. The Author(s).)

Background: Enthesitis-related arthritis (ERA) is a subtype of juvenile idiopathic arthritis with high disease burden. The objectives of this study were to explore the prevalence of HLA-B27, clinical characteristics, and treatment outcomes in children with ERA and compare the differences between HLA-B27 positive and negative patients., Methods: A retrospective cohort study at a pediatric rheumatology clinic in a tertiary referral hospital in Bangkok, Thailand, including ERA patients with at least 6 months of follow-up (July 2011-April 2022) was performed. Data were collected from medical records from diagnosis to recent follow-up, assessing disease activity and treatment outcomes, with an analysis comparing HLA-B27 positive and negative patients. Descriptive statistics were used for data analysis., Results: There were 59 ERA patients with mean age ± SD at diagnosis 11.2 ± 2.5 years, 53 males (89.8%), and positive HLA-B27 in 38 patients (64.4%). The HLA-B27 positive group had significantly higher levels of inflammatory markers at initial diagnosis (p = 0.001), lower baseline hemoglobin (p = 0.001) and hematocrit (p = 0.002), higher disease activity assessed by the Juvenile Spondyloarthritis Disease Activity score at 6 and 12 months of follow-up (p = 0.028 and 0.040, respectively), increased utilization of bridging systemic corticosteroids (60.5% vs. 14.3%, p = 0.001) and anti-TNF (39.5% vs. 9.5%, p = 0.018), and longer duration of methotrexate (median[IQR] 1.7[1.1-3.1] vs. 1.3[0.6-1.9] years, p = 0.040). The HLA-B27 negative group had more prevalent hip arthritis than the positive group at initial diagnosis (66.7% vs. 28.9%, p = 0.005) and during the course of the disease (71.4% vs. 36.8%, p = 0.011)., Conclusion: Most of the ERA patients tested positive for HLA-B27. Throughout the follow-up period, these patients demonstrated greater disease activity, greater use of corticosteroids and anti-TNF, and longer duration of methotrexate to control the disease., (© 2024. The Author(s).)

Background: Childhood-onset systemic lupus erythematosus (c-SLE) is a multifaceted autoimmune disorder predominantly affecting the musculoskeletal (MSK) system. This investigation delineated the spectrum and sequelae of MSK involvement in c-SLE patients., Methods: This retrospective analysis included SLE patients aged ≤ 18 years treated at a tertiary center between 2009 and 2019. Data were extracted from electronic health records., Results: The cohort comprised 321 SLE patients (mean age 13.2 ± 2.5 years, 91.3% female). MSK manifestations were observed in 134 (41.7%) individuals, with joint pain universally present, followed by joint swelling in 32.1% and morning stiffness in 9.7%. Arthritis was documented in 52 (38.8%) patients, whereas 82 (61.2%) had arthralgia. Symmetrical joint involvement was observed in 96 (71.7%) subjects. The knees, wrists, and fingers were most commonly affected, with incidences of 43.3%, 40.3%, and 33.6%, respectively. Neither erosive arthritis nor Jaccoud's arthropathy was detected. MSK symptoms were significantly correlated with older age at diagnosis, the presence of non-scarring alopecia, neuropsychiatric manifestations, and elevated SLE disease activity index scores at diagnosis. Over a median follow-up of 53.6 months (IQR 26.1-84.6), five patients developed septic arthritis or osteomyelitis, and avascular necrosis was identified in 16 (4.9%) patients., Conclusions: Nearly half of c-SLE patients demonstrated MSK manifestations, chiefly characterized by symmetrical involvement of both large and small joints without evidence of erosive arthritis or Jaccoud's arthropathy. Avascular necrosis is a critical concern and warrants close monitoring., (© 2024. The Author(s).)

Objectives: We evaluated the immunogenicity and safety of BNT162b2 vaccination in adolescents with systemic lupus erythematosus (adoSLE) receiving either high- or low-dose immunosuppressant (High-IS and Low-IS)., Methods: Patients aged 12-18 years diagnosed with SLE were enrolled. High-IS was defined as >7.5 mg/day prednisolone or with other immunosuppressant, while Low-IS was defined as only ≤7.5 mg/day of prednisolone and no immunosuppressant. Two doses of BNT162b2 vaccination were given 4 weeks apart, followed by a booster (third) dose at 4-6 months later. Anti-spike receptor binding domain (anti-RBD) IgG against Wuhan, neutralising antibody (NT) against Wuhan and Omicron variants, and cellular immune response by IFN-γ-ELISpot assay were evaluated following vaccination. Adverse events (AEs) and SLE flare were monitored., Results: A total of 73 participants were enrolled, 40 and 33 in the High-IS and Low-IS group, respectively. At 4 weeks following the 2nd dose, overall anti-RBD IgG seropositivity was 97.3%, with no difference between the groups ( p = .498). AdoSLE on High-IS had lower anti-RBD IgG ( p < .001), Wuhan NT ( p < .001), and IFN-γ-ELISpot ( p = .022) than those on Low-IS. A 3rd dose induced significantly higher antibody responses than after the 2nd dose ( p < .001) in both groups and established seroconversion against Omicron variants, with persistent lower antibody levels in High-IS group. SELENA-SLEDAI scores within 12 weeks after 2-dose vaccination was higher than before vaccination (3.1 vs 2.5; p < .036); however, the occurrence of disease flare by SELENA-SLEDAI flare index was not different after vaccination compared to before vaccination, consistent across groups. Non-severe AEs occurred similarly in both groups., Conclusion: AdoSLE on High-IS induced lower SARS-CoV-2 vaccine immune responses than Low-IS. Vaccination can increase disease activity and requires close monitoring for disease flare., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., (© 2024. The Author(s).)

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., (© 2023. The Author(s).)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Lerkvaleekul, Charuvanij, Sukharomana, Pirojsakul, Kamolwatwong and Vilaiyuk.)

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., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

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., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Low bone mass is one of the complications of juvenile idiopathic arthritis (JIA). However, a study focusing on the low bone mass in children and adolescents with JIA in Southeast Asian countries is limited. This study aimed to evaluate the bone mineral density (BMD) of Thai patients with JIA and identify factors correlated with BMD. A cross-sectional study was conducted at a tertiary-care center. The BMD of the lumbar spines (BMDLS) and the total body (BMDTB) were measured by dual-energy X-ray absorptiometry. Thirty-eight patients were enrolled between July 2015 and January 2016. No patient had low BMDLS, and only 2 (5.3%) had low BMDTB. Serum 25-hydroxyvitamin D (25OHD) levels were significantly positively correlated with the BMDTB Z -score (coefficient: 0.047; 95% confidence interval = 0.011-0.082; P = .012). Our study demonstrated a very low prevalence of low bone mass. Optimization of the serum 25OHD level should be encouraged.

Background: Little is known about the impact of delayed initiation of anti-tumor necrosis factor (TNF) therapy in patients with enthesitis-related arthritis (ERA). Here, we compared the impact of delayed treatment on disease outcomes of ERA patients in Southeast Asia., Research Design and Methods: This retrospective study enrolled 149 ERA patients from Thailand and Singapore. Early (e-aTNF) and late (l-aTNF) treatment groups received anti-TNF therapy starting at ≤6 months and >6 months, respectively, after diagnosis. Outcomes included mean differences in disease activity parameters, Juvenile Spondyloarthritis Disease Activity (JSpADA) score, Juvenile Arthritis Diseases Activity (JADAS)-10 score, and American College of Rheumatology Pediatric (ACR Pedi) criteria, and the frequency of clinically inactive disease and first flare event., Results: The mean changes in JSpADA (p = 0.002) and JADAS-10 (p < 0.001) scores over time were significantly higher in the e-aTNF group than in the l-aTNF group. A significantly higher proportion of patients in the e-aTNF group than l-aTNF group satisfied ACR Pedi 100 criteria at 2 years (p = 0.042). All other long-term outcomes were not significantly different between the groups., Conclusions: Although early anti-TNF treatment improved disease activity parameters somewhat better than delayed anti-TNF therapy, there was no significant difference in long-term outcomes.

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., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

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.

Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients., Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children., Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019., Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset., Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study., Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

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., (© 2022 Japan Pediatric Society.)

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., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Pediatric rheumatology (PR) is a relatively new and rare subspecialty in emerging countries. This study aimed to investigate physicians' attitudes toward and real-life clinical practice in PR among residency-trained pediatricians in Thailand., Methods: An electronic questionnaire was developed and sent via email to pediatricians from Thailand who completed their residency training between 2007 and 2015. Confidence in treating and managing children with rheumatic diseases was rated using a 5-point Likert scale., Results: The response rate was 281 out of 902 (31%), and the mean ± standard deviation age of respondents was 33.8 ± 2.7 years. Confidence was rated as adequate for history taking of children with rheumatic diseases (mean 2.76, 95% confidence interval [CI]: 2.66-2.91), but low for musculoskeletal (MSK) examination (2.42, 95% CI: 2.29-2.54), arthrocentesis (2.01, 95% CI: 1.91-2.11), and rheumatology investigation (2.49, 95% CI: 2.39-2.60). Confidence was high for diagnosis and management of Henoch-Schönlein purpura (3.83, 95% CI: 3.74-3.92) and Kawasaki disease (3.46, 95% CI: 3.38-3.55), but low for juvenile idiopathic arthritis (JIA) (2.41, 95% CI: 2.30-2.52) and chronic systemic vasculitis (1.97, 95% CI: 1.85-2.08). Confidence to perform an MSK examination and arthrocentesis was significantly higher in respondents who had a full-time pediatric rheumatologist working in their pediatric residency training center (P = 0.02, P = 0.01, respectively), and in those who had experienced a PR rotation (P < 0.001, P = 0.01, respectively). Most respondents agreed that more teaching in PR is essential (95.3%) and that case-based discussion was the preferred teaching method., Conclusion: The self-rated confidence of pediatricians was low in MSK examination, arthrocentesis, and rheumatology investigation and therefore, teaching on PR is needed., (© 2021 Japan Pediatric Society.)

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.

Objectives: This study aimed to analyse the cost-utility and budget impact of adding tocilizumab to the standard treatment for patients with refractory systemic juvenile idiopathic arthritis (sJIA) in Thailand., Design: Economic evaluation using a decision-analytical model., Setting: Thailand., Participants: Patients with refractory sJIA who were ≥2 years old., Methods: The use of tocilizumab as an add-on therapy to standard treatment was compared with standard treatment alone. A simulated health state transition model was used to estimate the lifetime costs and health outcomes from a societal perspective. Direct medical costs were collected from tertiary hospital databases while direct non-medical costs were derived from interviews. Health-related quality of life (QoL) was measured using the proxy version of three-level EuroQol five-dimensional questionnaire (EQ-5D-3L). Future costs and outcomes were discounted at an annual rate of 3%. The base case population was patients aged 9.41 years old at refractory disease onset. The results were reported as incremental cost-effectiveness ratios (ICER) in US dollar (USD). One-way and probabilistic sensitivity analysis were conducted to investigate parameter uncertainty. The 5-year budget impact was estimated from a governmental perspective., Results: The ICER of standard treatment plus tocilizumab was US$35 799 per quality-adjusted life-year (QALY) gained compared with standard treatment alone, which was not cost-effective at the threshold of US$5128 per QALY gained. The estimated 5 years budget impact was approximately US$4.8 million., Conclusions: The use of standard treatment plus tocilizumab was not cost-effective in the Thai context, which has limited data. However, there is currently no second-line treatment for refractory sJIA in the Thai National List of Essential Medicines; thus, patients must receive higher doses of standard treatment which can cause many side effects. In contrast, tocilizumab showed obvious efficacy in clinical trials in improving treatment response and QoL. Therefore, the price of tocilizumab should be negotiated to reduce the financial impact on the healthcare system., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Musculoskeletal (MSK) complaints in children vary, ranging from benign, self-limited conditions to serious disorders. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease, initially presenting with MSK complaints. Delayed diagnosis and appropriate treatment have an enormous impact on the long-term outcomes and the level of disability. This study aimed to identify the features distinguishing JIA among children presenting with MSK complaints and to describe the spectrum of diseases at a large, single, tertiary center., Methods: A retrospective chart review was performed of patients evaluated by pediatric rheumatology consultation at the Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand, from July 2011 to June 2015., Results: Of 531 patients, 285 (53.6%) had at least one MSK complaint. The mean age of the patients was 9.1 ± 4.1 years. Joint pain was the most common MSK complaint (86.3%), followed by limping (33%) and refusal to walk (19.6%). Joint swelling and limited range of motion were found in 146 (51.2%) and 115 (40.4%) patients, respectively. Seventy-three (25.6%) patients were diagnosed as JIA. The other common diagnoses included Henoch-Schönlein purpura (16.1%), reactive arthritis (14.2%), and systemic lupus erythematosus (13.7%). Morning stiffness  ≥ 15 minutes [odds ratio (OR) 8.217 (3.404-19.833)]; joint swelling on MSK examination [OR 3.505 (1.754-7.004)]; a duration of MSK complaints of more than 6 weeks [OR 2.071 (1.120-3.829)]; and limping [OR 1.973 (1.048-3.712)] were significantly associated with the ultimate diagnosis of JIA., Conclusions: Morning stiffness ≥ 15 minutes is a strong predictor of JIA. Comprehensive history taking and an MSK examination will provide clues for making the ultimate diagnosis for children with MSK complaints.

Systemic lupus erythematosus (SLE) is a multisystem, autoimmune inflammatory disease which can affect any organ, including the gastrointestinal tract. Lupus enteritis is one of the manifestations of gastrointestinal involvement in SLE patients. However, it is exceedingly rare that lupus enteritis is the sole initial presentation of SLE. A 12-year-old Thai girl who had had recurrent abdominal pain for 2 months with no other signs of SLE on initial presentation is described. A single-balloon enteroscopy demonstrated segmental erythema of the proximal and mid-jejunum. Histopathology demonstrated active enteritis and submucosal vasculitis. On the basis of evidence of intestinal vasculitis, autoimmune profiles were performed; the results supported the possibility of SLE. She subsequently developed leucopenia, lymphopenia and an oral ulcer, leading to a robust diagnosis of SLE. Her clinical condition improved dramatically with prednisolone. Even though lupus enteritis is rare, it can be the initial presentation of SLE. In young adolescent girls with recurrent abdominal pain, the possibility of lupus enteritis should be borne in mind.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myopathy is less common in children but has been associated with more favorable prognosis than adult patients after immunotherapies. We report anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody positivity in a 6-year-old boy with progressive muscle weakness, scoliosis, spinal rigidity, multiple joint contractures, mild left ventricular hypertrophy, and elevated serum creatine kinase. In contrast to most of previously reported pediatric anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myopathy, he showed little response to immunotherapies. Muscle biopsy contained changes suggestive of myofiber necrosis and regeneration and reducing bodies. The diagnosis of reducing body myopathy was later confirmed by reported c.368A>G (p.His123Arg) mutation in the FHL1 gene. Although the level of association between these two conditions is still inconclusive, this is the first report of concurrent positive anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody with reducing body myopathy emphasizing the possibility of co-occurrence of immune mediated necrotizing myopathy and muscular dystrophy and importance of comprehensive diagnostic investigations in unusual cases., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease resulting in physical and psychological disability. This study aimed to measure the health-related quality of life (HRQOL) and identify factors influencing the HRQOL., Methods: We conducted a cross-sectional study in Thai children with JIA and their parents between January and December 2015, using the Thai version of the Pediatrics Quality of Life (PedsQL) 4.0 generic core scale., Results: Sixty-five children (33 girls and 32 boys) were included. The median age of the children was 9.6 (interquartile range [IQR] 6.4-12.3) years and the median disease duration was 1.1 (IQR 0.2-2.2) years. The physical health subscale had the lowest score, with a median (range) of 78.1 (34.4-100). Twenty-five (45.4%) children were classified as having suboptimal HRQOL. The school functioning subscale had the lowest score, at 60 (25-100) reported by parents. High disease activity and a disease duration <1 year were significantly associated with suboptimal HRQOL reported by parents, with adjusted odds ratios (ORs) of 20.2 (95% confidence interval [CI] 1.4, 291.7) and 5.9 (95% CI 1.2, 33.3), respectively., Conclusions: Almost half of Thai children with JIA had suboptimal HRQOL. The physical health score was the lowest subscale reported by the children. Early, effective management of disease activity could improve the HRQOL in children with JIA., (© 2019 John Wiley & Sons, Ltd.)

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., Competing Interests: Conflict of interest: the authors declare no potential conflict of interest.

Objective: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV., Methods: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4-6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months., Results: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9-15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4-6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0-6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months., Conclusion: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course., (© 2017, American College of Rheumatology.)

Objectives: To examine the descriptive epidemiology of the patient population referred to paediatric rheumatology centres (PRCs) in Southeast Asia (SEA) and to compare the frequency of conditions encountered with other PRC populations., Methods: A web-based Registry for Childhood Onset Paediatric Rheumatic Diseases was established in 2009 and seven PRCs in four SEA countries, where paediatric rheumatologists are available, participated in a prospective 24 month data collection (43 months for Singapore)., Results: The number of patients analysed was 4038 (788 from Malaysia, 711 from the Philippines, 1943 from Singapore and 596 from Thailand). Over 70% of patients evaluated in PRCs in Malaysia, the Philippines and Thailand had rheumatic diseases (RDs), as compared with one-half of the proportion seen in Singaporean PRCs, which was similar to the Western PRC experience. Among RDs diagnosed (n = 2602), JIA was the most common disease encountered in Malaysia (41%) and Thailand (61%) as compared with systemic vasculitides in the Philippines (37%) and Singapore (35%) among which Henoch-Schönlein purpura was the most prevalent. SLE and related diseases were more common, but idiopathic pain syndrome and abnormal immunological laboratory tests were rarer than those seen in the West. JIA subtype distributions were different among countries. Among non-RDs (n = 1436), orthopaedic and related conditions predominated (21.7-59.4%)., Conclusion: The frequencies of RDs seen by SEA PRCs were different from those in the West. Systemic vasculitides and SLE were common in addition to JIA. Paediatric rheumatologist availability and healthcare accessibility partially explain these observed discrepancies., (© The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Objective: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA)., Methods: The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons., Results: In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil., Conclusion: Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding., (© 2016, American College of Rheumatology.)