Predictors of first hospitalization due to disease activity and infections in systemic lupus erythematosus patients.

OBJECTIVES: To identify the predictive factors of first hospitalization and associated variables to the main causes of hospitalizations in lupus patients from a Latin American cohort. METHODS: The first hospitalization after entry into the cohort during these patients' follow-up due to either lupus disease activity and/or infection was examined. Clinical and therapeutic variables were those occurring prior to the first hospitalization. Descriptive statistical tests, multivariable logistic, and Cox regression models were performed. RESULTS: 1341 individuals were included in this analysis; 1200 (89.5%) were women. Their median and interquartile range (IQR) age at diagnosis were 27 (20-37) years and their median and IQR follow up time were 27.5 (4.7-62.2) months. A total of 456 (34.0%) patients were hospitalized; 344 (75.4%), 85 (18.6%) and 27 (5.9%) for disease activity, infections, or both, respectively. The predictors of the first hospitalization regardless of its cause were: medium (HR 2.03(1.27-3.24); p = 0.0028) and low (HR 2.42(1.55-3.79); p < 0.0001) socioeconomic status, serosal (HR 1.32(1.07-1.62); p = 0.0074) and renal (HR 1.50(1.23-1.82); p < 0.0001) involvement. Antimalarial (AM) use (HR 0.61(0.50-0.74); p < 0.0001) and achieving remission (HR 0.80(0.65-0.97); p = 0.0300) were negative predictors. CONCLUSIONS: The first hospitalization was associated with worse socioeconomic status and serosal and renal involvement. Conversely, AM use and achieving remission were associated with a lower risk of hospitalizations.

Rituximab as Treatment for Lupus Nephritis: Data From the Peruvian ALMENARA Lupus Cohort.

OBJECTIVE: The aim of this study was to evaluate the response to rituximab (RTX) as treatment for lupus nephritis (LN) in a Latin American Lupus cohort. METHODS: The medical records from LN patients from a single-center cohort spanning between January 2012 and December 2020 were reviewed. Demographic factors (age at diagnosis and baseline, gender), disease duration, previous and concomitant treatments, serum creatinine, and 24-hour proteinuria (24-HP) levels at baseline, and 6th and 12th months were obtained. Complete response (CR) or responder status was defined according to the LUNAR, AURORA-1, and BLISS-LN trials. RESULTS: Thirty-six patients received RTX as induction treatment; 32 (88.9%) were women. Their age at baseline and disease duration were 32.6 (11.7) and 7.6 (6.5) years, respectively. The time between renal biopsy and RTX use was 2.64 (4.41) years. At baseline, serum creatinine and 24-HP levels were 1.5 (1.5) mg/dL and 3.4 (2.8) g, respectively. At months 6 and 12, serum creatinine levels were 1.6 (1.6) and 1.6 (1.5) mg/dL, and 24-HP were 2.2 (2.2) and 1.6 (1.5) g, respectively. According to LUNAR and AURORA-1 criteria, CR at 6th and 12th months were 6/34 (17.6%) and 8/30 (26.7%) and 6/34 (17.6%) and 7/31 (22.6%) patients, respectively. According to BLISS-LN criteria, responders at 6th and 12th months were 9/34 (26.5%) and 10/31 (32.3%) patients, respectively. CONCLUSIONS: CR and responder status were reached in less than one third of LN patients treated with RTX, regardless of the criteria used to define them. However, serum creatinine levels did not increase, and there was a decrease in proteinuria levels during the follow-up.

Joint Damage and Low Lean Body Mass in a Cohort of Peruvian Patients With Rheumatoid Arthritis.

OBJECTIVES: To determine the association between radiologic joint damage (JD) and a lower lean body mass (LBM) in rheumatoid arthritis (RA) patients. METHODS: A cross-sectional study from a single center established RA cohort. JD and appendicular LBM (arms and legs) were measured with the Sharp/van der Heijde (SvdH) score and dual x-ray absorptiometry expressed as kg/m 2 , respectively. A univariable analysis was used to determine the association between JD an LBM; then, a multivariable regression model was performed to evaluate the persistence of this association, adjusted by age, gender, disease duration, socioeconomic status (by the Graffar method), tobacco use, anticitrullinated protein antibody levels, Disease Activity Score in 28 joints for RA with erythrocyte sedimentation rate, glucocorticoid use (as prednisone equivalent), disease-modifying antirheumatic drug use, body mass index, and disability (by the multidimensional Health Assessment Questionnaire). RESULTS: Two hundred forty-seven patients were included; the average (SD) age was 63.0 (12.8) years, disease duration 20 (15.00) years, the total SvdH was 66 (86.75), and the aLBM was 13.6 (3.82) kg/m 2 . In the univariable analysis, a lower appendicular LBM was associated with higher SvdH score on the female population, in terms of the total ( B = -8.6, p < 0.01), bone erosion (-4.4, p < 0.01), and joint space narrowing (-4.2, p < 0.01) scores; this correlation remained in the multivariable analysis in terms of total SvdH ( B = -9.5, p < 0.01), bone erosion (-5.2, p < 0.01), and joint space narrowing (-4.3, p < 0.01). CONCLUSIONS: A lower LBM in female patients was associated with more severe JD independently of other variables examined. Strategies aimed at preserving LBM could have a favorable impact on the course of disease.

Performance of the 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease in a Latin American Cohort.

BACKGROUND/OBJECTIVE: The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a significant advancement in the study of this condition. Most studies evaluating their performance have focused on White and Asian patients, leaving a knowledge gap regarding Latin American populations. Therefore, this study aimed to assess the performance of the 2019 AECC for IgG4-RD in a cohort of Latin American patients. METHODS: A multicenter medical records review study was conducted, involving centers from Argentina, Chile, Mexico, Peru, and Uruguay. Data on IgG4-RD patients and mimicker conditions were collected through a standardized online form. The criterion standard for diagnosing IgG4-RD was based on the fulfillment of the Comprehensive Diagnostic Criteria for IgG4-RD and/or the Consensus Statement on Pathology. The 2019 AECC was retrospectively applied. RESULTS: We included 300 patients, with 180 (60%) having IgG4-RD and 120 (40%) having mimicker conditions. The 2019 AECC had a sensitivity of 66.7% and a specificity of 100%. Sensitivity increased to 73.3% when disease-specific autoantibody items were removed, without affecting specificity. The true-positive cases had more involved organs, a higher availability of biopsy results, and were more likely to belong to the Mikulicz/systemic and proliferative phenotypes. CONCLUSIONS: The use of the 2019 AECC for IgG4-RD in a Latin American population confirms its high specificity in excluding those without the disease. The presence of concomitant autoimmune diseases and clinically nonsignificant disease-specific autoantibodies excludes a significant number of patients from fulfilling the criteria.

High Anti-Interferon-Alpha Autoantibody Levels in Severe/Critical COVID-19 Patients from Peru.

Innate immune responses through the production of type I interferon-α (anti-IFN-α) play an essential role in the defense against viruses. The autoantibodies (auto-Abs) anti-IFN-α are implicated in COVID-19 pathogenesis with higher levels among patients with worse prognoses. The study aimed to assess the levels of anti-IFN-α auto-Abs in Peruvian patients with severe/critical hospitalized COVID-19 compared to asymptomatic/mild COVID-19 outpatients and healthy controls. We analyzed 101 serum samples, including 56 (55.5%) severe/critical, 13 (12.3%) asymptomatic/mild COVID-19 patients, and 32 (32.2%) healthy controls, which we tested using a commercial ELISA anti-IFN-α-auto-Abs kit. We observed seropositivity of 48.2% (26/54) to anti-IFN-α auto-Abs among the severe/critical COVID-19 group, but 0% (0/13) and 3.1% (1/32) among the asymptomatic/mild COVID-19 and healthy groups (P = 0.021), respectively. Furthermore, we observed a significant association between the log10 of anti-IFN-α auto-Abs and the COVID-19 status, with the log10 of anti-IFN-α auto-Abs levels being significantly higher among the severe/critical COVID-19 group compared to the healthy controls (ß = 1.20; confidence interval [95% CI]: 0.72-1.67; P < 0.001). Such association remains significant either when adjusted by age and gender (adjusted ß = 1.16; 95% CI: 0.62-1.70; P < 0.001) and when adjusted by the subjects' age, gender, and obesity (adjusted ß = 1.16; 95% CI: 0.62-1.70; P < 0.001). Despite not measuring neutralizing activity, this study highlights the high frequency of these auto-Abs in the Peruvian population with a worse prognosis of COVID-19.

The Rise and Rise of Lupus.

Despite how difficult the early diagnosis of systemic lupus erythematosus (SLE) is, which is mainly due to the heterogeneity and non-specificity of its clinical manifestations, SLE is currently being diagnosed more frequently than in past decades. In fact, there has been an increase in the incidence and prevalence of SLE over the last four decades; this can be explained by a number of reasons including a better knowledge of the pathogenesis of the disease which allows its earlier diagnosis, the rising ethnic and racial diversity of the world population, the use of the 2019 EULAR/ACR criteria that allows classifying patients earlier, and improvements in survival over the last decades, which results in an increase in the prevalent cases of SLE. In this article, we will also review the genetic, environmental, and lifestyle factors, that are reported to increase the risk of developing SLE and how preventive strategies through a clinical care pathway may prevent or delay the development of SLE and improve these patients' outcomes.

Improving access to SLE therapies in low and middle-income countries.

SLE increases disease burden in those affected with it, and that is particularly the case in low- and middle-income countries. The 2019 Addressing Lupus Pillar of Health Advancement project is a multiphase initiative whose objective is to recognize, hierarchize and establish approaches for diligent SLE research, care and access to healthcare. Lack of access to high-cost medications that have been shown to be efficacious in the short term and that potentially reduce damage in SLE is a complex issue. In this review, we highlight opportunities and plans of action to diminish costs and improve access to therapies, which should be recognized and executed, preferably within regional strategies with multiple stakeholders (including supranational organizations, governments, the pharmaceutical industry, medical societies and the general population) connected with and grounded in structured and clear cost-effectiveness analysis.

Predictors of severe hemolytic anemia and its impact on major outcomes in systemic lupus erythematosus: Data from a multiethnic Latin American cohort.

OBJECTIVE: To determine the predictors of the occurrence of severe autoimmune hemolytic anemia (AIHA) and its impact on damage accrual and mortality in SLE patients. METHODS: Factors associated with time to severe AIHA (hemoglobin level ≤7 g/dL) occurring from the onset of SLE symptoms were examined by Cox proportional hazards regressions. The association of severe AIHA with mortality was examined by logistic regression analyses while its impact on damage was by negative binomial regression. RESULTS: Of 1,349 patients, 49 (3.6%) developed severe AIHA over a mean (SD) follow-up time of 5.4 (3.8) years. The median time from the first clinical manifestation to severe AIHA was 111 days (IQR 43-450). By multivariable analysis, male sex (HR 2.26, 95% CI 1.02-4.75, p = 0.044), and higher disease activity at diagnosis (HR 1.04, 95% CI 1.01-1.08, p = 0.025) were associated with a shorter time to severe AIHA occurrence. Of the SLEDAI descriptors, only hematologic (leukopenia and/or thrombocytopenia) showed a certain trend toward significance in the multivariable analysis (HR 2.36, 95% CI 0.91-6.13, p = 0.0772). Severe AIHA contributed neither to damage nor to mortality. CONCLUSIONS: Severe AIHA occurs during the early course of SLE. Male sex and higher disease activity at diagnosis emerged as independent predictors of a shorter time to severe AIHA occurrence. Although not statistically significant, hematological abnormalities at SLE diagnosis could predict the occurrence of severe AIHA in a shorter time. Damage and mortality did not seem to be impacted by the occurrence of severe AIHA.

Association of Systemic Lupus International Collaborating Clinics Frailty Index With Damage in Systemic Lupus Erythematosus Patients: Results From a Multiethnic, Multicenter US Cohort of Patients With Lupus.

OBJECTIVE: To evaluate the association between the Systemic Lupus International Collaborating Clinics frailty index (SLICC-FI) and damage accrual in systemic lupus erythematosus (SLE) patients. METHODS: Patients from the multiethnic, multicenter LUpus in MInorities, NAture versus nurture (LUMINA) cohort were included. Damage was ascertained with the SLICC/American College of Rheumatology Damage Index (SDI) at last visit (range 0-51). The first visit in which the SLICC-FI score could be derived was considered as the baseline (range 0-1). Univariable and multivariable negative binomial regression models were performed to determine the association between the baseline SLICC-FI score (per 0.05 increase) and the change in the SDI score (difference between last and baseline SDI score), adjusted for sex, age at diagnosis, ethnicity, insurance, prednisone daily dose, and antimalarial and immunosuppressive drug use at baseline. Age and sex were included a priori in the multivariable model; the other variables were included if they reached P < 0.10 in the univariable models. RESULTS: Of the 503 patients included, 454 (90.3%) were female, with a mean ± SD age of 37.1 ± 12.5 years at diagnosis. The mean ± SD baseline SLICC-FI score was 0.26 ± 0.06. The mean ± SD baseline SDI score was 0.6 ± 1.0, and the mean ± SD change in the SDI score was 1.9 ± 2.2. Higher SLICC-FI scores at baseline (per 0.05 increase) were associated with greater damage accrual in the multivariable model after adjustment for possible confounders (incidence rate ratio 1.20 [95% confidence interval 1.08-1.33], P = 0.0015). CONCLUSION: The SLICC-FI is associated with damage accrual in SLE patients from a multiethnic cohort, supporting the importance of this index in the evaluation of SLE patients, combining several aspects of their disease.

Association Between Race/Ethnicity and COVID-19 Outcomes in Systemic Lupus Erythematosus Patients From the United States: Data From the COVID-19 Global Rheumatology Alliance.

OBJECTIVE: To determine the association between race/ethnicity and COVID-19 outcomes in individuals with systemic lupus erythematosus (SLE). METHODS: Individuals with SLE from the US with data entered into the COVID-19 Global Rheumatology Alliance registry between March 24, 2020 and August 27, 2021 were included. Variables included age, sex, race, and ethnicity (White, Black, Hispanic, other), comorbidities, disease activity, pandemic time period, glucocorticoid dose, antimalarials, and immunosuppressive drug use. The ordinal outcome categories were: not hospitalized, hospitalized with no oxygenation, hospitalized with any ventilation or oxygenation, and death. We constructed ordinal logistic regression models evaluating the relationship between race/ethnicity and COVID-19 severity, adjusting for possible confounders. RESULTS: We included 523 patients; 473 (90.4%) were female and the mean ± SD age was 46.6 ± 14.0 years. A total of 358 patients (74.6%) were not hospitalized; 40 patients (8.3%) were hospitalized without oxygen, 64 patients (13.3%) were hospitalized with any oxygenation, and 18 (3.8%) died. In a multivariable model, Black (odds ratio [OR] 2.73 [95% confidence interval (95% CI) 1.36-5.53]) and Hispanic (OR 2.76 [95% CI 1.34-5.69]) individuals had higher odds of more severe outcomes than White individuals. CONCLUSION: Black and Hispanic individuals with SLE experienced more severe COVID-19 outcomes, which is consistent with findings in the US general population. These results likely reflect socioeconomic and health disparities and suggest that more aggressive efforts are needed to prevent and treat infection in this population.

Zonulin, a marker of gut permeability, is associated with mortality in a cohort of hospitalised peruvian COVID-19 patients.

Zonulin has previously been related to intestinal permeability in various inflammatory diseases, and more recently to the physiopathology of severe COVID-19 infections. We analysed serum samples from a previous study of a Peruvian cohort of hospitalised COVID-19 patients, for the quantification of zonulin by sandwich ELISA. Comparisons with clinical data, haematological and biochemical parameters and cytokine/chemokine levels were made. We found higher baseline zonulin levels in deceased patients, and zonulin was associated with fatal outcome in multivariable analyses, even after adjustment for age, gender, and obesity. There were also positive correlations between zonulin, creatinine, D-dimer values and prothrombin time, while inverse correlations were found for Sa/FiO2 ratio and CCL5 (RANTES). Further longitudinal studies are recommended to analyse the variation of zonulin levels over time as well as their relationship with long-COVID.

Addressing health disparities as a function of ethnicity in systemic lupus erythematosus patients.

Systemic lupus erythematosus (SLE) is an autoimmune disorder with significant health disparities, as it disproportionately and more severely affects vulnerable and disadvantaged population groups in the United States and around the world, that is, women, ethnic minorities, individuals living in poverty, less educated, and lacking medical insurance. Both, genetic and non-genetic factors, contribute to these disparities. To overcome these health disparities and reduce poor outcomes among disadvantaged SLE populations, interventions on non-genetic amendable factors, especially on social health determinants, are necessary.

Lupus Eritematoso Sistémico y Fatiga

El Lupus Eritematoso Sistémico (LES) es una enfermedad autoinmune inflamatoria crónica que tiene como uno de sus síntomas más frecuentes la fatiga. La etiología de ésta es multifactorial, existen factores relacionados a la propia enfermedad, estilos de vida, comorbilidades y tratamiento que están asociados. Asimismo, se cuenta con diversos factores para poder medirla, siendo los instrumentos más utilizados: Escala Análoga Visual (EVA), "Krupp Fatigue Severity Scale" (FSS) y el "Functional Assessment of Chronic Illness Therapy Fatigue" (FACIT-Fatigue). El impacto de la fatiga en el paciente con LES es importante ya que repercute en su calidad de vida.

Systemic Lupus Erythematosus (SLE) is a chronic inflammatory autoimmune being fatigue one of its more frequent symptoms. The etiology of this is multifactorial, there are factors related to the disease itself, lifestyles, comorbidities and treatment that are associated. Also, there are several factors to measure it, being the most used instruments: Visual Analog Scale (VAS), "Krupp Fatigue Severity Scale" (FSS) and the "Functional Assessment of Chronic Illness Therapy Fatigue" (FACIT-Fatigue). The impact of fatigue on the SLE patient is important because it affects the patient's quality of life.

Predictors of Remission and Low Disease Activity in Rheumatoid Arthritis Patients: Results From the Follow-up of a Real-World Peruvian Cohort.

BACKGROUND: Clinical remission is the goal in rheumatoid arthritis (RA) management; however, this can be difficult to achieve in several parts of the world. Our objective was to determine predictors of remission and remission/low disease activity (LDA) in RA. METHODS: A longitudinal real-setting RA cohort was followed up (January 2016-2020). Predictors examined were sex, age at diagnosis, disease duration, socioeconomic status, tobacco use, rheumatoid factor titer, comorbidities (Charlson index), Simple Disease Activity Index (SDAI) score, disability (Multidimensional Disease Health Assessment Questionnaire), health-related quality of life (Short Form-36 questionnaire), glucocorticoid dose, biological/target synthetic disease-modifying antirheumatic drugs, and conventional DMARD (c-DMARD) use. Univariable and multivariable generalized estimating equation models were done to determine predictors of remission (at a given visit) and sustained remission (2 consecutives visits), using the SDAI definition (0 or <3.3). Similarly, remission/LDA (SDAI <11) predictors were examined. RESULTS: Five hundred thirty RA patients included the following: 160 patients (30.2%) achieved remission in at least 1 visit, and 126 patients (23.77%) achieved sustained remission. On the multivariable analysis glucocorticoid dose (odds ratio [OR], 1.060; 95% confidence interval [CI], 1.027-1.094; p = 0.004) and current (OR, 2.293; 95% CI, 1.811-2.903; p < 0.001) or past (OR, 1.383; 95% CI, 1.127-1.698; p = 0.002) use of c-DMARDs predicted remission/LDA in at least 1 visit, whereas the SDAI (OR, 0.951; 95% CI, 0.942-0.959; p < 0.001), Multidimensional Disease Health Assessment Questionnaire (OR, 0.648; 95% CI, 0.549-0.764; p < 0.001), and age at diagnosis (OR, 0.994; 95% CI, 0.990-0.998; p = 0.004) were negative predictors. As to sustained remission/LDA, current (OR, 2.012; 95% CI, 1.458-2.776: p < 0.001) or past (OR, 1.517; 95% CI, 1.155-1.993; p = 0.003) use of c-DMARDs, having a better Short Form-36 questionnaire physical component summary (OR, 1.022; 95% CI, 1.014-1.029; p < 0.001), and older age at diagnosis (OR, 1.013; 95% CI, 1.003-1.022; p = 0.008) predicted it, whereas SDAI (OR, 0.949; 95% CI, 0.933-0.965; p < 0.001) and medium low/low socioeconomic status (OR, 0.674; 95% CI, 0.500-0.909; p = 0.010) were negative predictors. CONCLUSION: During follow-up of this real-world RA cohort, c-DMARD use predicted remission and remission/LDA. In contrast, disease activity was a negative predictor.

Different epidemiologic profiles of systemic vasculitis between Brazil and Peru-preliminary results in two referral centers from both countries.

Little is known about the epidemiology of systemic vasculitis in South American countries. The aim of this study is to compare the prevalence of systemic vasculitides in two vasculitis referral centers from Brazil and Peru. A cross-sectional study was performed and all patients above 18 years of age, with at least 6 months of follow-up and who met classification or diagnosis criteria for the most common forms of vasculitis, were included. A total of 562 patients with systemic vasculitis were analyzed, 345 (61.4%) from Brazil and 217 (38.6%) from Peru. The frequency of Behçet's disease (37.9% vs. 1.8%; p < 0.0001), Takayasu arteritis (TAK) (25.2% vs. 6.9%; p < 0.0001), and giant cell arteritis (9.8% vs. 0.9%; p < 0.0001) was higher in the Brazilian center than the Peruvian one. On the other hand, the frequency of microscopic polyangiitis (MPA) (67.3% vs. 2.8%; p < 0.0001) and renal-limited vasculitis (2.8% vs. 0.0%; p = 0.009) was higher in the Peruvian center. No differences were found concerning other forms of vasculitis. At diagnosis, Brazilian patients with TAK, granulomatosis with polyangiitis, and MPA were younger than Peruvian patients. Epidemiologic differences in the frequency of systemic vasculitis are observed between a vasculitis referral center from Brazil and another from Peru. Key Points • Significant differences are observed regarding the epidemiologic profile of systemic vasculitis between Brazil and Peru. • MPA is the predominant form of vasculitis in Peru while BD and TAK are the most frequent forms of vasculitis in Brazil. • The age at diagnosis of TAK, MPA, and GPA was lower in Brazilian patients than in Peruvian patients.

Controversies in Systemic Lupus Erythematosus: Are We Treating Our Patients Adequately?

ABSTRACT: Systemic lupus erythematosus (SLE) is characterized by great clinical heterogeneity. The objectives of its management are to make a timely diagnosis and to initiate treatment as promptly as possible so organ damage can be avoided while at the same time exposure to potentially toxic drugs is minimized so that its overall course and outcome improve. In reviewing the current literature, it became quite clear that there are specific topics in which controversies do exist. These include how to treat patients with incomplete lupus erythematosus, the real possibility of abandoning altogether the use of oral glucocorticoids, and the pros and cons of the use of cyclophosphamide and mycophenolate mofetil for the induction treatment of lupus nephritis. Herein we discuss different points of view regarding these still unresolved issues; these comments represent a debate that took place during the PANLAR Virtual Congress (Pan American League of Associations for Rheumatology) and that was organized by the PANLAR Lupus study group, GLADEL (Grupo Latino Americano De Estudio del Lupus) on September 19, 2020.

Relationship between care model and disease activity states and health-related quality of life in systemic lupus erythematosus patients.

OBJECTIVE: To assess whether the care model (comprehensive vs regular) has any impact on the clinical outcomes of systemic lupus erythematosus patients. METHODS: Between August 2019 and January 2020, we evaluated SLE patients being cared for at two Peruvian hospitals to define the impact of care model on disease activity state and health-related quality of life (HRQoL). Disease activity was ascertained with the SLEDAI-2K and the Physician Global Assessment (PGA) which allows to define Lupus Low Disease Activity State (LLDAS) and Remission. HRQoL was measured with the LupusQoL. The association between care model and disease activity (Remission and LLDAS) state was examined using a binary logistic regression model. The association with HRQoL was examined with a linear regression model. All multivariable analyses were adjusted for possible confounders. RESULTS: 266 SLE patients were included, 227 from the comprehensive care model and 39 from the regular care model. The regular care model was associated with a lower probability of achieving remission (OR 0.381; CI: 95% 0.163-0.887) and LLDAS (OR 0.363; CI: 95% 0.157-0.835). Regular care was associated with a better HRQoL in two domains (pain and emotional health). We found no association between the care model and the other HRQoL domains. CONCLUSION: A comprehensive care model was associated with the probability of achieving remission and LLDAS but had no apparent impact on the patients' HRQoL.

Performance of the 2017 American College of Rheumatology/European League Against Rheumatism Provisional Classification Criteria for Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in a Peruvian Tertiary Care Center.

AIM: To validate the new classification criteria for antineutrophil cytoplasmic antibody-associated vasculitis in a real-life Peruvian cohort of antineutrophil cytoplasmic antibody-associated vasculitis patients. METHODS: We reviewed medical records from a Peruvian tertiary care center from January 1990 to December 2019. Antineutrophil cytoplasmic antibody-associated vasculitis was diagnosed based on the 1990 American College of Rheumatology (ACR) criteria, the 2012 Chapel Hill Consensus Conference definitions, the European Medicines Agency (EMEA) algorithm, and the clinical acumen of the treating rheumatologists. We classified all patients using the "former criteria" (the 1990 ACR criteria for granulomatosis with polyangiitis [GPA] and eosinophilic GPA [EGPA] and the 1994 Chapel Hill Consensus Conference definition for microscopic polyangiitis [MPA]), the EMEA algorithm, and the "new criteria" (the 2017 ACR/European League Against Rheumatism Provisional Criteria). The level of agreement (using Cohen κ) was calculated using the clinical diagnosis as the criterion standard. RESULTS: We identified 212 patients, 12 of whom were excluded. One hundred fifty-four (77%) had MPA, 41 (20.5%) GPA, and 5 (2.5%) EGPA. The new criteria performed well for MPA (κ = 0.713) and EGPA (κ = 0.659), whereas the EMEA algorithm performed well for GPA (κ = 0.938). In the overall population, the new criteria showed better agreement (κ = 0.653) than the EMEA algorithm (κ = 0.506) and the former criteria (κ = 0.305). CONCLUSIONS: The 2017 ACR/European League Against Rheumatism Provisional Criteria showed better agreement for the clinical diagnosis of all the patients overall and had the best performance for MPA and EGPA. The EMEA algorithm had the best performance for GPA.

Global excellence in rheumatology in Latin America: The case of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) affects more severely non-White populations, due to their genetic background and sociodemographic characteristics. Several studies have evaluated Latin American SLE patients to determine their genetic and clinical characteristics as well as prognostic factors; these studies have not only allowed the development of treatment guidelines aimed at the region but also to support regional and global projects. Additionally, educational activities in Spanish and Portuguese have been started to reduce our patients' health illiteracy. Despite the relatively low research output from Latin American countries, we consider that studies from our region coupled with the networks developed to increase our capabilities, could be a model for other rare autoimmune diseases.