Immunoglobulin A vasculitis (IgAV), also known as Henoch- Schönlein purpura (HSP), is the most common systemic vasculitis in childhood. Potential differences in demographic characteristics, clinical presentation, laboratory findings, and treatments approaches across age groups, remain poorly explored. To the best of our knowledge, no multicenter study in Latin America has systematically addressed these features. We aimed to assess demographic, clinical and laboratory features, and treatments in children versus adolescents with (IgAV)/ (HSP) in a large multicenter study. A multicenter study involving four tertiary centers evaluated 687 children and adolescents (≤ 18 years-old) with IgAV/HSP (EULAR/PRINTO/PRES classification criteria) at first 3 months after diagnosis. The charts were retrospectively assessed for demographic data, initial clinical manifestations, laboratory tests and treatments. Data were compared between children (< 10 years-old) and adolescents (≥ 10 years-old), according to WHO definition. IgAV/HSP was diagnosed in 599/687(87

IntroductionYouth with special health care needs (YSHCN) are particularly vulnerable during the transition from pediatric to adult healthcare. Although nutritional status is a potentially modifiable determinant of health, its relationship with transition readiness and health-related quality of life (HRQoL) during this period remains poorly understood.ObjectiveTo investigate the association of nutritional status and socioeconomic factors with transition readiness and HRQoL in YSHCN undergoing healthcare transition.MethodsThis cross-sectional study included 218 consecutive YSHCN followed at a tertiary university hospital in Brazil. Transition readiness was assessed using the Transition Readiness Assessment Questionnaire (TRAQ), and HRQoL was evaluated using the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0). Nutritional status was assessed using body mass index-for-age (zBMI/A) and height-for-age (zH/A) z-scores according to World Health Organization standards. Multivariable linear regression models were used to identify factors independently associated with TRAQ and PedsQL scores. Logistic regression analyses were performed to identify predictors of low transition readiness (TRAQ <3.5).ResultsNutritional status assessed by BMI-for-age (zBMI/A) was not associated with transition readiness or HRQoL. In multivariable analyses, female sex (β = 0.18, p = 0.002) and older age (β = 0.12, p = 0.001) were independently associated with higher TRAQ scores. Higher height-for-age (zH/A) (β = 1.86, p = 0.031) and higher family income (β = 2.87, p = 0.025) were independently associated with better HRQoL, whereas female sex was associated with lower HRQoL (β = −7.25, p = 0.001). In logistic regression analyses, younger age (OR = 0.82, 95% CI 0.70–0.96, p = 0.023) and male sex (OR = 0.48, 95% CI 0.25–0.92, p = 0.023) were significant predictors of low transition readiness, while nutritional risk was not associated with the outcome. The logistic model demonstrated modest discrimination (AUC = 0.689).ConclusionIn YSHCN undergoing healthcare transition, BMI-for-age was not associated with transition readiness or HRQoL. In contrast, linear growth and family income were independently associated with better HRQoL, highlighting the importance of developmental and socioeconomic factors in patient-reported outcomes. These findings reinforce the multifactorial nature of transition readiness and HRQoL and support the incorporation of nutritional, developmental, and psychosocial assessment into multidisciplinary healthcare transition programs.

OBJECTIVES:To compare the effectiveness, safety, and retention of rituximab biosimilar (RTX-GP2013) and RTX-originator in RTX-naive Rheumatoid Arthritis (RA) patients in a real-world setting. METHODS:This single-center, longitudinal, observational study included 107 RA patients who initiated RTX therapy between January/2010 and September/2023 (40 RTX-GP2013 and 67 RTX-originator). A convenience sample of consecutive eligible RTX-naïve patients was analyzed. Disease activity, treatment retention, and infusion-related reactions were assessed over six months (6M). Multivariate logistic regression identified predictors of low disease activity (CDAI ≤ 10) at 6M. RESULTS:RTX-GP2013 patients were older (58.3 [53.1-66.4] vs. 54.9 [45.6-60.8], p = 0.029), had longer disease duration (18.4 ± 9.5 vs. 14.7 ± 8.9, p = 0.045), and a greater number of prior biologic therapies (3 [1-5] vs. 1 [0-2], p < 0.001). At 6M, frequency of low disease activity (30.0% vs. 47.8%, p = 0.103), retention rates for a second RTX cycle (77.5% vs. 85.1%, p = 0.433) and mild/moderate infusion-related reactions (17.5% vs. 9.0%, p = 0.231), were comparable between RTX-GP2013 and RTX-originator groups, respectively. Multivariate analysis identified lower baseline disease activity (per 1-point increase in CDAI: OR = 0.93, 95% CI 0.88-0.97, p = 0.003) and fewer prior advanced therapies (OR = 0.56, 95% CI 0.38-0.82, p = 0.003) as predictors of low disease activity at 6M, whereas RTX-GP2013 was not associated with poorer clinical response (OR = 1.85, 95% CI 0.63-5.45, p = 0.265). CONCLUSION:In this real-world study, RTX-GP2013 and RTX-originator demonstrated comparable effectiveness, safety, and treatment retention in RTX-naive RA patients. Baseline disease activity and fewer prior therapies were predictors of clinical response. These findings reinforce the role of biosimilars in expanding access to effective therapies while maintaining clinical outcomes.

BACKGROUND:Immunocompromised Sjögren's disease-(SjD) patients are susceptible to herpes zoster reactivated infection-(HZ/shingles), yet data on the recombinant zoster vaccine-(Shingrix®/RZV) in these patients are limited. OBJECTIVES:To assess RZV humoral/cellular immunogenicity and safety in immunocompromised SjD patients. METHODS:Randomized double-blind placebo-controlled study comprising 67 immunocompromised SjD patients and 201 age-/sex-balanced non-immunosuppressed controls. SjD patients were randomized to receive vaccine-(SjD-V) or placebo-(SjD-P) on day-0-(D0) and D42. SjD-P were immunized after unblinding on D84 and D126. Controls were vaccinated on D0 and D42. Adverse events-(AEs) and disease activity were monitored. Humoral immunogenicity was assessed by anti-gE concentrations, with seroconversion defined as ≥4-fold increase from baseline to 6-weeks after the 2nd RZV dose. gE-specific CD4+ T cells were counted in ≅10% of participants. Vaccine impact on SjD symptoms/biological parameters was explored. RESULTS:SjD and controls were comparable regarding age [55.0 (48.3-63.8) vs. 55.0 (52.0-64.0) years; P = 0.087] and sex (P = 0.303). Seroconversion rate was lower in SjD than controls (90.6% vs. 99.5%; P < 0.001), with reduced geometric-mean-titer-(GMT) [7.1 (5.3-9.6) vs. 12.6 (11.4-13.9) mIU/mL; P < 0.001] and factor-increase-GMT [23.9 (16.0-35.8) vs. 70.4 (58.8-84.4); P < 0.001] in SjD. The cellular response was comparable between SjD patients and controls (P > 0.05). Disease activity, SjD symptoms/biological parameters remained stable (P > 0.05). No serious vaccine-related AEs occurred. CONCLUSION:RZV showed a favorable safety profile in immunocompromised SjD patients. Despite the high seroconversion rate, the response was significantly lower in magnitude compared to controls. This may compromise long-term immunity, underscoring the need for optimizing immune responses in this population (ClinicalTrials.gov-NCT05879419).

ObjectiveTo evaluate whether hydroxychloroquine (HCQ) dose reduction (2-3 mg/kg/day) sustain lipoprotein levels achieved with higher doses of 2016-American Academy of Ophthalmology (2016-AAO) in stable lupus nephritis (LN) patients.MethodsForty-seven consecutive stable LN patients using HCQ 2016-AAO recommended dose for ≥6 months were enrolled and assigned to one of two groups: Reduced HCQ group (n = 21):LN patients who, upon inclusion, reduced 2016-AAO dose (2-3 mg/kg/day); and Maintenance HCQ group (n = 26):LN patients who continued on the standard 2016-AAO recommended HCQ dose (4.0-5.5 mg/kg actual body weight, maximum 400 mg/day) throughout 12-month study period. Blood HCQ levels, lipid profile and SLE parameters (including SLEDAI-2K) were assessed at baseline, 3 and 12-month.ResultsBaseline demographics, comorbidities and disease parameters were similar among groups (p > .05). Initial blood levels of Reduced HCQ group were 1219.4 (1041.7-1926.6)ng/mL and a progressive significant decrease were identified after 3 and 12 months [651.2 (538.4-832.6)vs.468.8 (228.4-925.6)ng/mL, p < .001]. Maintenance HCQ group had no changes in HCQ levels during the study [1179.7 (905.5-1607.3)vs.1026.2 (710.5-1345.8)vs.907.9 (663.9-1304.2)ng/mL, p = .158]. No changes were observed on the longitudinal total cholesterol levels of Reduced HCQ [166 (113-198)vs.153 (85-192)vs.150 (90-231)mg/dL, p = .964] and Maintenance HCQ [155 (114-244)vs.154 (122-210)vs.155 (112-213)mg/dL, p = .395]. LDL cholesterol levels of Reduced HCQ [91 (52-163)vs.83 (41-136vs.87 (47-165)mg/dL, p = .917] and Maintenance HCQ [79 (36-114)vs.76.5 (33-111)vs.75.5 (63-131)mg/dL, p = .412] remained similar. Other lipoprotein levels remained stable during 1 year of study.ConclusionThis is the first study to show that reducing HCQ to 2-3 mg/kg/day preserves lipid stability over 12 months in stable lupus nephritis patients.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood, but its outcomes are still difficult to determine. We aimed to obtain outcome measurements of disease activity, functional capacity, disease damage, and therapeutic response, at one-year follow-up study on a real-life basis. An observational JIA cohort from two referral centers for pediatric rheumatology in Brazil Pediatric Rheumatology Centers was carried out over a period of one year. Clinimetric validated outcome measurements were applied over four visits. Multivariable logistic regression was performed to evaluate baseline variables associated with the following outcomes after one year of follow-up: disease activity, Minimal Disease Activity (MDA), disease flare, remission on medication and remission off medication. A total of 127 patients were included in the study. Eighty-three (65.4

Background Patients with autoimmune rheumatic diseases (ARDs) are at an increased risk for herpes zoster (HZ). Vaccination is recommended for this population. Objective The aim of this study was to evaluate the safety of vaccination with the recombinant zoster vaccine (Shingrix) in ARD patients, humoral immunogenicity (HI), cellular immunogenicity (CI), and the incidence of HZ. Methods This randomized, double-blind, placebo-controlled phase 4 study involves 1180 ARD patients and a control group (CG) of 393 balanced healthy individuals, aged >= 50 years. ARD patients will be randomly assigned in a blinded manner (1:1 ratio) to 2 groups: vaccine or placebo (on days 0 and 42), administered intramuscularly. Outcomes will be assessed at baseline, 6 weeks, and 12 weeks after vaccination, including disease activity (using specific disease activity scores), HI, and CI. Adverse events will be assessed using a standardized questionnaire after each vaccine dose. Incident HZ cases will be monitored throughout the study. One year following the second dose, the persistence of HI and CI will be evaluated in both ARD patients and CG. HI and CI will be assessed using serum concentrations of anti-gE antibodies and the frequencies of gE-specific CD4+ T cells, respectively. Comparisons of anti-gE titers between ARD patients and CG at different time points will be analyzed using 2-way repeated-measures analysis of variance. Multiple regression analysis will be conducted, with a positive immune response as the dependent variable, and variables with p < 0.2 from univariate analysis as independent variables. Conclusions This large trial addresses a critical gap by examining disease safety, efficacy, adverse effects, and immunogenicity, considering the impact of diverse therapies following recombinant zoster vaccine administration in ARD patients.

Patients with chronic Chagas disease (CD) and autoimmune rheumatic disorders (ARD) receiving immunosuppressive therapy are at risk for CD reactivation (CDR). This study monitored parasitemia over 9-121 months in six patients with CD and ARD using conventional and quantitative PCR and parasitology. Five patients showed parasitemia; two had elevated levels of parEq/mL (49-458.7). One patient received benznidazole with a marked decrease in parasitemia; in the other, treatment was discontinued after 12 days due to toxicity. No CDR occurred. These findings support the need for qPCR standardization for preemptive therapy and suggest that benznidazole may prevent CDR in patients with high parasitemia.

The objective of the present study was to evaluate biochemical quantitative metabolites in peripheral blood serum samples of Juvenile Idiopathic Arthritis (JIA) patients and healthy controls. A cross-sectional study included 33 post-pubertal JIA (21 without and 12 with Methotrexate (MTX) women and 28 age-matched healthy controls. Metabolomic analyses based on targeted electrospray ionization tandem mass spectrometry were used to identify possible biochemical pathway modifications in serum from JIA patients. The mean current age (p = 0.065) was similar in JIA patients and healthy controls. Current MTX use in all subtypes of JIA patients was associated with an increase in concentrations of free carnitine [21.74 (12.7‒35.2) vs. 27.49 (14.5‒41.3) µM/L, p = 0.02], suggesting an enhanced mitochondrial metabolism and intestinal absorptive function. In contrast, a decreased mitochondrial metabolism was observed in polyarticular and systemic JIA subtypes, with a decrease of several acylcarnitines' concentrations (p < 0.05). In conclusion, the present study identified a distinctive pattern of serum metabolic signatures in JIA patients under MTX therapy. Our findings indicate that MTX use is associated with a more efficient mitochondrial function.

Patients with autoimmune rheumatic diseases (ARDs) are at an increased risk for herpes zoster (HZ). Vaccination is recommended for this population. The aim of this study was to evaluate the safety of vaccination with the recombinant zoster vaccine (Shingrix) in ARD patients, humoral immunogenicity (HI), cellular immunogenicity (CI), and the incidence of HZ. This randomized, double-blind, placebo-controlled phase 4 study involves 1180 ARD patients and a control group (CG) of 393 balanced healthy individuals, aged ≥50 years. ARD patients will be randomly assigned in a blinded manner (1:1 ratio) to 2 groups: vaccine or placebo (on days 0 and 42), administered intramuscularly. Outcomes will be assessed at baseline, 6 weeks, and 12 weeks after vaccination, including disease activity (using specific disease activity scores), HI, and CI. Adverse events will be assessed using a standardized questionnaire after each vaccine dose. Incident HZ cases will be monitored throughout the study. One year following the second dose, the persistence of HI and CI will be evaluated in both ARD patients and CG. HI and CI will be assessed using serum concentrations of anti-gE antibodies and the frequencies of gE-specific CD4+ T cells, respectively. Comparisons of anti-gE titers between ARD patients and CG at different time points will be analyzed using 2-way repeated-measures analysis of variance. Multiple regression analysis will be conducted, with a positive immune response as the dependent variable, and variables with p < 0.2 from univariate analysis as independent variables. This large trial addresses a critical gap by examining disease safety, efficacy, adverse effects, and immunogenicity, considering the impact of diverse therapies following recombinant zoster vaccine administration in ARD patients.

Aim: The use of anti-TNF drugs is well-established for treating axial spondyloarthritis (axSpA). The introduction of biosimilars offers a more accessible alternative, but data on the switching of adalimumab biosimilars in the axSpA population remain somewhat controversial and are limited to SB5 and ABP 501 and to the European population. This study aims to evaluate the clinical efficacy of switching from originator adalimumab to the biosimilar adalimumab-AACF in Latin American axSpA patients over a 12-month period in a real-life analysis. Methods: This observational study included patients with axSpA who had been treated with originator adalimumab for at least three months and switched to the biosimilar. Disease activity parameters and C-reactive protein (CRP) levels were assessed at baseline (T0) and compared at 6 (T6) and 12 months (T12) following the switch. Results: Twenty-eight patients were included, with a mean duration of originator adalimumab use of 87.6 months. Ankylosing Spondylitis Disease Activity Score (ASDAS)-CRP remained stable when comparing T0 to T6 [1.56 (± 0.88) vs. 1.50 (± 0.82), P = 0.73] and T12 [1.56 (± 0.88) vs. 1.26 (± 0.86), P = 0.13]. A similar pattern was observed for ASDAS-erythrocyte sedimentation rate (ESR; P > 0.05) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI; P > 0.05). The rate of remission/low disease activity was consistent, recorded at 71.4% at baseline, 78.6% at T6 (P = 0.62) and 78.6% at T12 (P = 0.68). CRP levels did not show significant variation (P > 0.05) across time points. Notably, the one-year drug retention rate was 94.6%. Conclusions: This real-world study highlights for the first time the feasibility and efficacy of transitioning from originator adalimumab to biosimilar AACF in axSpA, providing support for its use in long-term management and offering enhanced accessibility without compromising therapeutic outcomes. These results add valuable Latin American data to the body of evidence on biosimilar integration into clinical practice.