The Lupus Foundation of America-Rapid Evaluation of Activity in Lupus Clinician-Reported Outcome Predicts Damage in Patients With Systemic Lupus Erythematosus. Data From the Almenara Lupus Cohort.

OBJECTIVE: To evaluate the predictive value of the LFA-REAL ClinRO (Lupus Foundation of America Rapid Evaluation of Activity in Lupus clinician-reported outcome) on damage accrual in systemic lupus erythematosus patients. METHODS: Data from a prevalent lupus cohort were used. The LFA-REAL ClinRO includes 9 domains: mucocutaneous (global and 3 subdomains), musculoskeletal (global and 2 subdomains), cardiorespiratory, neuropsychiatric, renal, hematological, constitutional, vasculitis, and other (it allows for other or rare manifestations). For each domain, a 0- to 100-mm visual analog scale is used, and global domains are included except for the mucocutaneous and musculoskeletal domains where the subdomains are included; it allows for 3 manifestations under "other," so the score ranges from 0 to 1400 (sum of 14 in the visual analog scale). Damage was assessed with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index. Generalized estimating equations were performed, being the outcome the increase in the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index; confounders from the previous visit were included; adjusted multivariable models were done. Incidence rate ratios per 10-unit increase in the LFA-REAL ClinRO were reported. Similar models were performed to evaluate the impact of the SLEDAI-2K (SLE Disease Activity Index) and physician global assessment on damage to determine which measure would better predict damage accrual. RESULTS: Three-hundred thirty-one patients and 1425 visits were included, 1.9 (SD 1.2) years of follow-up. Disease duration at baseline was 10.7 (7.4) years. The mean LFA-REAL ClinRO was 18.2 (SD 30.7). During the follow-up visits, 63 (17.9%) patients accrued damage once; 4 (1.1%) accrued damage twice. The LFA-REAL ClinRO was predictive of damage accrual even after adjustment for possible confounders (incidence rate ratio 1.10 (95% confidence interval 1.04-1.16; p < 0.001). Similar results were obtained using the SLEDAI-2K and the physician global assessment. CONCLUSION: The LFA-REAL ClinRO is predictive of damage accrual, even after adjusting for possible confounders.

A better self-efficacy is predictive of better health-related quality of life (HRQoL) in patients with systemic lupus erythematosus: data from the Almenara Lupus Cohort.

OBJECTIVE: To determine the possible predictive value of self-efficacy on health-related quality of life (HRQoL) in patients with SLE. METHODS: Patients with SLE from the Almenara Lupus Cohort were included. Self-efficacy was ascertained with the six domains from the Patient-Reported Outcomes Measurement Information System (PROMIS) self-efficacy for managing chronic conditions. For PROMIS domains, a score of 50 is the average for a clinical population (people with a chronic condition), a higher score indicates that the respondent has greater self-efficacy. HRQoL was ascertained with the physical and mental component summary (PCS and MCS) measures of the Short-Form 36 (SF-36). Generalised estimating equations were performed, using as outcome the PCS or MCS in the subsequent visit, and the self-efficacy domain in the previous visit; multivariable models were adjusted for possible confounders. The confounders were measured in the same visit as the self-efficacy domain. RESULTS: Two-hundred and nine patients for a total of 564 visits were included; 194 (92.8%) patients were women and mean age at diagnosis was 36.4 (14.0) years. In the multivariable models, a better PCS was predicted by a better self-efficacy for managing symptoms, managing medications and treatments and managing social interactions and general self-efficacy; a better MCS was predicted by a better self-efficacy for managing daily activities, managing symptoms, managing medications and treatments and managing social interactions. CONCLUSION: A better self-efficacy is predictive of subsequent better HRQoL, even after adjustment for possible confounders. These results should encourage clinicians to develop strategies to improve self-efficacy in patients with SLE.

The SLICC-FI Predicts Damage Accrual in SLE Patients. Data From the Almenara Lupus Cohort.

OBJECTIVE: To evaluate the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI) as a predictor of damage accrual in a primarily Mestizo SLE patient cohort. METHODS: Patients from a single-center prevalent cohort were included. Damage accrual was defined as the increase in the SLICC/American College of Rheumatology (ACR) damage index (SDI) scores between the baseline and the last visits. The SLICC-FI was measured at baseline. Univariable and multivariable Cox regression models were performed to determine the association between the baseline SLICC-FI (per 0.05 increase) and the increase in the SDI, adjusted for possible confounders. Alternative analyses using negative binomial regression models including the difference between the last and the first SDI as outcome were performed. RESULTS: Of the 265 patients included, 248 (93.6%) were female with mean (SD) age of 35.1 (13.6) years at diagnosis. At baseline, mean (SD) SLE disease duration was 7.3 (6.5) years, SDI was 1.0 (1.2) and the SLICC-FI was 0.22 (0.05). After a mean (SD) of 5.2 (2.2) years of follow-up, the SDI increased in 126 (47.5%) patients, and the final mean (SD) SDI score was 1.7 (1.7). Higher SLICC-FI scores at baseline predicted greater damage accrual in the univariable analysis [Hazard Ratio (HR) =1.38, (CI95% 1.16-1.65); p < 0.001] and in the multivariable model, after adjustment for possible confounders [HR = 1.30 (CI95% 1.02-1.66); p = 0.033]. CONCLUSION: SLICC-FI predicts the occurrence of damage accrual in a prevalent SLE Latin-American cohort with short or long disease duration, supporting the relevance of this index in the evaluation of SLE patients.

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.

Severe flares are associated with a poorer health-related quality of life (HRQoL) in patients with SLE: data from the Almenara Lupus Cohort.

BACKGROUND: Flares in patients with SLE, regardless of their severity, have been associated with damage accrual. However, their impact on health-related quality of life (HRQoL) has not been fully evaluated. In fact, disease activity is only minimally associated with HRQoL. OBJECTIVE: To determine the association between flares and HRQoL. METHODS: Patients from the Almenara Lupus Cohort were included. Visits occurring between December 2015 and February 2020 were evaluated. Flares were defined as an increase on the SLE Disease Activity Index 2000 (SLEDAI-2K) of at least 4 points; severe flares were those with a final SLEDAI-2K ≥12 and mild-moderate flares all the others. HRQoL was measured using the LupusQoL. Univariable and multivariable generalised estimating regression equations were performed, adjusting for possible confounders. Confounders were determined at one visit, whereas the outcome was determined on the subsequent visit; flares were determined based on the variation of the SLEDAI-2K between these visits. RESULTS: Two hundred and seventy-seven patients were included; 256 (92.4%) were female, mean age at diagnosis was 36.0 (SD: 13.3) years and mean disease duration at baseline was 9.1 (SD: 7.1) years. Patients had mean of 4.8 (SD: 1.9) visits and a mean follow-up of 2.7 (1.1) years. Out of 1098 visits, 115 (10.5%) flares were defined, 17 were severe and 98 mild-moderate. After adjustment for possible confounders, only severe flares were associated with a poorer HRQoL in planning, pain, emotional health and fatigue. CONCLUSIONS: Severe flares, but not mild-moderate, flares are associated with poorer HRQoL.

LLDAS (lupus low disease activity state) and/or remission are associated with less damage accrual in patients with systemic lupus erythematosus from a primarily Mestizo population: data from the Almenara Lupus Cohort.

OBJECTIVE: To determine if achieving lupus low disease activity state (LLDAS) or remission prevents damage accrual in a primarily Mestizo population. METHODS: Patients with SLE from a single-centre cohort with at least two visits occurring every 6 months were included. The definitions used were the following: for remission, the 2021 Definition Of Remission In SLE; and for LLDAS, the Asia Pacific Lupus Collaboration. Damage accrual was ascertained with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Univariable and three multivariable interval-censored survival regression models were done: (1) remission versus not on remission; (2) LLDAS/remission versus active; and (3) remission and LLDAS (not on remission) versus active. Three similar multivariable models were also examined considering the duration on each state. Possible confounders included in these analyses were gender, age at diagnosis, socioeconomic status, educational level, disease duration, antimalarial use and SDI at baseline. RESULTS: Two hundred and eighty-one patients were included. Eighty-three patients (29.5%) showed increased SDI during the follow-up. In the analyses of remission, being on remission predicted a lower probability of damage (HR=0.456; 95% CI 0.256 to 0.826; p=0.010). In the analyses of LLDAS/remission, being on LLDAS/remission predicted a lower damage (HR=0.503; 95% CI 0.260 to 0.975; p=0.042). When both states were considered, remission but not LLDAS (not on remission) predicted a lower probability of damage (HR=0.423; 95% CI 0.212 to 0.846; p=0.015 and HR=0.878; 95% CI 0.369 to 2.087; p=0.768, respectively). When the duration of these states was taken into account, remission, LLDAS/remission and LLDAS not on remission were associated with a lower probability of damage accrual. CONCLUSIONS: LLDAS and/or remission were associated with a lower probability of damage accrual.

Metabolic syndrome predicts new damage in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort.

OBJECTIVES: This study aims to determine whether the MetS predicts damage accrual in SLE patients. METHODS: This longitudinal study was conducted in a cohort of consecutive SLE patients seen since 2012 at one single Peruvian institution. Patients had a baseline visit and then follow-up visits every 6 months. Patients with ≥ 2 visits were included. Evaluations included interview, medical records review, physical examination, and laboratory tests. Damage accrual was ascertained with the SLICC/ACR damage index (SDI) and disease activity with the SLEDAI-2K. Univariable and multivariable Cox-regression survival models were carried out to determine the risk of developing new damage. The multivariable model was adjusted for age at diagnosis; disease duration; socioeconomic status; SLEDAI; baseline SDI; the Charlson Comorbidity Index; daily dose; and time of exposure of prednisone (PDN), antimalarials, and immunosuppressive drugs. RESULTS: Two hundred and forty-nine patients were evaluated; 232 of them were women (93.2%). Their mean (SD) age at diagnosis was 35.8 (13.1) years; nearly all patients were Mestizo. Disease duration was 7.4 (6.6) years. The SLEDAI-2K was 5.2 (4.3) and the SDI, 0.9 (1.3). One hundred and eight patients (43.4%) had MetS at baseline. During follow-up, 116 (46.6%) patients accrued at least one new point in the SDI damage index. In multivariable analyses, the presence of MetS was a predictor of the development of new damage (HR: 1.54 (1.05-2.26); p < 0.029). CONCLUSIONS: The presence of MetS predicts the development of new damage in SLE patients, despite other well-known risk factors for such occurrence.

Factores asociados a infecciones serias en pacientes hospitalizados con lupus eritematoso sistémico / Factors associated with serious infections in patients hospitalized with systemic lupus erythematosus

RESUMEN Introducción. En pacientes con lupus eritematoso sistémico (LES) existe incremento de infecciones debido a la propia enfermedad, al uso de inmunosupresores y corticoides. Objetivo. Identificar los factores asociados a infecciones serias en pacientes lúpicos en un hospital de referencia nacional. Estudio retrospectivo, analítico, de casos y controles en el Servicio de Reumatología del Hospital Nacional Guillermo Almenara Irigoyen, Lima, Perú. Métodos. Se analizó el registro de pacientes hospitalizados en el periodo de estudio, los casos fueron pacientes en los que se demostró la etiología de la primera infección durante su hospitalización. Los controles fueron pacientes lúpicos hospitalizados sin infecciones en el mismo periodo de estudio. Se analizaron variables asociadas al desarrollo de infecciones. Resultados. 61 pacientes de 390 hospitalizados desarrollaron infecciones durante su hospitalización. 48 desarrollaron 1 solo evento infeccioso (en 40 se demostró etiología). Los casos tuvieron mayor actividad, daño y comorbilidad en comparación con los controles. En el análisis univariado, el salario (p=0,031), el uso de inmunosupresores a la admisión (previo: p=0,004 y actual: p=0,004), el uso de glucocorticoides (<30 días: p=0,015 y >30-360 días: p=0,028), la actividad (p=0,029) y el daño (p=0,026) producido por la enfermedad, y el tiempo de hospitalización (p=0,045) tuvieron asociación estadísticamente significativa. En el análisis multivariado, los días de hospitalización se asociaron al desarrollo de infecciones. Conclusiones. Existió asociación entre días de hospitalización y el desarrollo de infecciones serias en pacientes lúpicos durante el periodo de estudio.

ABSTRACT Introduction. Lupus patients have an increased risk of developing infections due to the disease, use of immunosuppressants and corticosteroids. Objective. To identify the associated factors for serious infections in lupus patients in a national referral hospital. Retrospective, analytical, case-control study in the Rheumatology Service of the Guillermo Almenara Irigoyen National Hospital, Lima, Peru. Methods. The registry of hospitalized patients in the study period was analyzed, the cases were patients in whom the etiology of the first infection developed their hospitalization. Controls were hospitalized lupus patients without infections in the same study period. Variables predisposing to the development of infections were analyzed. Results. 61 patients out of 390 hospitalized developed infections during their hospitalization. 48 developed 1 only infectious event (in 40 an etiology developed). The cases had higher damage, activity and comorbidity compared to the controls. In the univariate analysis, salary (p = 0.031), use of immunosuppressants upon admission (previous: p = 0.004 and current: p = 0.004), use of glucocorticoids (<30 days: p = 0.015 and> 30-360 days: p = 0.028), activity (p = 0.029) and damage (p = 0.026) produced by the disease and length of hospitalization (p = 0.045), had a statistically significant association. In the multivariate analysis, the days of hospitalization were associated with the development of infections. Conclusions. There is an association between days of hospitalization and the development of serious infections in lupus patients in the study period.

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.

Health-Related Quality of Life and fatigue are associated with a higher work productivity impairment in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort.

Objectives: This study aims to determine the factors associated with absenteeism, presenteeism, and overall work impairment in patients with systemic lupus erythematosus (SLE).Methods: A total of 133 consecutive working patients with SLE were assessed between October 2017 and December 2018, using a standardized data collection form. Sociodemographic, disease, and work-related variables were collected. Work productivity and activity impairment (WPAI) was assessed with the respective questionnaire; absenteeism and presenteeism due to overall health and symptoms during the past 7 days were scored. Linear regression models were performed to determine the factors associated with absenteeism, presenteeism, and overall work impairment. Potential factors included were age at diagnosis, gender, socioeconomic status, educational level, SLEDAI, SLICC/ACR damage index (SDI), FACIT-Fatigue, and the domains of the LupusQoLResults: The mean age at diagnosis was 32.2 years (11.8); 121 (91.7%) were female. Nearly all patients were Mestizo. The mean percent of time for absenteeism was 5.0 (12.9), it was 28.5 (26.4) for presenteeism, and it was 31.3 (27.2) for overall work impairment. In the multiple regression analysis, factors associated with absenteeism were disease duration (B = -0.34; SE = 0.12; p = 0.007), pain (B = -0.14; SE = 0.06; p = 0.046), intimate relationship (B = -0.07; SE = 0.03; p = 0.046), and emotional health (B = 0.16; SE = 0.06; p = 0.006); factors associated with presenteeism were physical health (B = -0.43; SE = 0.14; p = 0.002) and FACIT (B = -0.87; SE = 0.30; p = 0.005); and factors associated with overall work impairment were pain (B = -0.40; SE = 0.11; p = 0.001) and FACIT-Fatigue (B = -0.74; SE = 0.28; p = 0.010).Conclusion: A poor HRQoL and higher levels of fatigue were associated with a higher percentage of absenteeism, presenteeism, and overall work impairment in SLE patients.

Survival in ANCA-Associated Vasculitides in a Peruvian Center: 28 Years of Experience.

AIM: The aim of this study was to identify demographic and clinical risk factors for mortality in patients with antineutrophil cytoplasmic antibodies-associated vasculitides (AAVs) in a Peruvian tertiary referral hospital. METHODS: Medical records of patients with AAV according to classification criteria or diagnosed by an experienced rheumatologist, covering the period between January 1990 and December 2018, were reviewed. Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis were included. Potential predictors of mortality were demographic factors, clinical manifestations, antineutrophil cytoplasmic antibodies status, diagnosis, disease categorization, the 2009 Five Factor Score (FFS), and treatment. Cox regression models were used to determine the risk factors for mortality. Univariable and multivariable analyses using a backward selection method were performed. RESULTS: One hundred ninety-six patients were included; female-to-male ratio was 2:1. The median (interquartile range) age at diagnosis and follow-up were 60.0 (51.0-68.0) and 4.8 (1.3-11.6) years, respectively. One hundred forty-eight patients (75.5%) had microscopic polyangiitis, 37 (18.9%) granulomatosis with polyangiitis, 5 (2.6%) eosinophilic granulomatosis with polyangiitis, and 6 (3.0%) renal-limited vasculitis. Overall survival rates at 1, 5, and 10 years were 83.4%, 68.2%, and 51.7%, respectively. Ocular involvement was protective (hazards ratio [HR], 0.36; 95% confidence interval [CI], 0.17-0.74; p = 0.006), whereas renal (HR, 2.09; 95% CI, 1.33-3.28; p = 0.001) and lung involvement (HR, 2.07; 95% CI, 1.31-3.28; p = 0.002) and the 2009 FFSs were predictive of mortality (2009 FFS = 1: HR, 2.46; 95% CI, 1.50-4.04; p < 0.001; 2009 FFS = 2: HR, 3.07; 95% CI, 1.54-6.10; p = 0.001; 2009 FFS = 3: HR, 13.29; 95% CI, 3.69-47.88; p < 0.001). CONCLUSIONS: Ocular involvement was protective, whereas 2009 FFS ≥ 1 and renal and lung involvement were predictive factors of mortality in Peruvian AAV patients.

Demographic and Clinical Features of ANCA-Associated Vasculitides in a Peruvian Tertiary Center.

AIM: The aim of this study was to identify the demographic and clinical features of patients with ANCA-associated vasculitides (AAVs) in a Peruvian tertiary referral hospital. METHODS: Medical records of patients with AAV according to classification criteria or diagnosed by an experienced rheumatologist, and covering the period between January 1990 and December 2019, were reviewed. Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal-limited vasculitis (RLV) were included. Demographic factors (age at diagnosis, sex), disease duration, clinical manifestations (per organ involvement), creatinine level at diagnosis (milligram per deciliter), ANCA status, diagnosis, 2009 Five Factor Score, disease categorization, and treatment were recorded. RESULTS: Two hundred twelve patients were included. Their female-to-male ratio was 1.9:1 (139 [65.6%]/73 [34.4%]), and their mean (SD) age at diagnosis was 59.2 (12.5) years. One hundred fifty-eight patients (74.5%) had MPA, 42 (19.8%) GPA, 7 (3.3%) RLV, and 5 (2.4%) EGPA. Neurological, lung, and renal involvements were the most frequently affected systems. Myeloperoxidase preferentially occurred in MPA (82.5%), whereas proteinase 3 did occur in GPA (79.5%). Microscopic polyangiitis patients were older (61.1 [11.5] years). Female sex predominated in MPA and RLV (2.4:1 and 6:1, respectively), but the opposite was the case for EGPA (1:4). Ear-nose-throat and ocular involvement were more frequent in GPA (both p's < 0.001), and neurological and cardiovascular involvement were more frequent in EGPA (p < 0.001 and p = 0.002, respectively). CONCLUSIONS: This is one of the largest series of AAV patients in Latin America. Overall, female sex predominated. Microscopic polyangiitis was the most frequent AAV, and myeloperoxidase-ANCA was the most frequent antibody in Peruvian AAV population.

Evaluation of the LFA-REAL clinician-reported outcome (ClinRO) and patient-reported outcome (PRO): data from the Peruvian Almenara Lupus Cohort.

OBJECTIVE: The Lupus Foundation of America Rapid Evaluation of Activity in Lupus (LFA-REAL) clinician-reported outcome (ClinRO) and the LFA-REAL patient-reported outcome (PRO) were developed in order to capture manifestations of SLE from the perspective of both the clinician and the patient. The aim of this study is to compare the LFA-REAL ClinRO and PRO with other lupus disease activity measures. METHODS: A cross-sectional analysis of patients from a single-centre cohort was performed using Spearman's correlation. Disease activity measures included were LFA-REAL ClinRO (range 0-1400), LFA-REAL PRO (range 0-1200), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), clinical SLEDAI-2K and Physician Global Assessment (PGA, range 0-100). RESULTS: Two hundred and twenty-seven patients with SLE were studied. The mean age was 46.3 (SD: 13.8); 212 (93.4%) were female. The mean (SD) LFA-REAL ClinRO was 25.4 (34.7), LFA-REAL PRO was 241.1 (187.6), PGA was 11.9 (15.4), SLEDAI-2K was 2.3 (3.3) and clinical SLEDAI-2K was 1.6 (2.9). The LFA-REAL ClinRO correlated with PGA (r=0.758, p<0.001), SLEDAI-2K (r=0.608, p<0.001) and clinical SLEDAI-2K (r=0.697, p<0.001); the LFA-REAL PRO correlated modestly with PGA (r=0.160, p=0.016), SLEDAI-2K (r=0.121, p=0.069), clinical SLEDAI-2K (r=0.143, p=0.031) and LFA-REAL ClinRO (r=0.161, p=0.015). CONCLUSIONS: The LFA-REAL ClinRO and the LFA-REAL PRO had good and weak correlations, respectively, with several physician-based disease activity measures in a cross-sectional study, suggesting their potential usefulness in establishing disease severity. Longitudinal studies will be required to determine their value in monitoring patients with SLE.

Age at diagnosis and health-related quality of life are associated with fatigue in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort.

OBJECTIVE: To define the factors associated with fatigue in Mestizo patients with Systemic Lupus Erythematosus (SLE). METHODS: This is a cross-sectional study of SLE patients from a single center cohort. Visits were performed every six months. For these analyses, the first visit between October 2017 and December 2018 was included. Demographic and clinical characteristics as well as treatment were recorded at every visit. Fatigue was ascertained with the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-FT), Health-Related Quality of Life (HRQoL) with the LupusQoL, disease activity with the Systemic Lupus Erythematosus Disease Activity Index -2 K (SLEDAI-2K), and damage with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI). Prednisone use was recorded as current daily dose. Immunosuppressive drugs and antimalarial use were recorded as current, past or never. Univariable and multivariable analyses were performed using linear regression models. For the multivariable analyses, model selection followed a backward elimination procedure. RESULTS: Two hundred and twenty-six patients were evaluated. The mean (SD) age at diagnosis was 35.6 (13.1) years, 211 (93.4%) were female; and disease duration was 11.0 (7.3) years. The mean SLEDAI and SDI were 2.4 (3.5) and 1.3 (1.5), respectively. The mean FACIT-FT was 33.1 (10.8). On the multivariable analysis, age at diagnosis and some domains of HRQoL (physical health, emotional health and fatigue) remained associated. CONCLUSIONS: Age at diagnosis is negatively associated with fatigue whereas HRQoL domains like physical health, emotional health and fatigue are positively associated with fatigue.

Serum uric acid is associated with damage in patients with systemic lupus erythematosus.

Introduction: Serum uric acid levels have been reported as predictors of cardiovascular, pulmonary, neurological and renal morbidity in patients with SLE. However, their role in cumulative global damage in these patients has not yet been determined. Objective: To determine whether serum uric acid levels are associated with new damage in patients with SLE. Methods: This is a longitudinal study of patients with SLE from the Almenara Lupus Cohort, which began in 2012. At each visit, demographic and clinical characteristics were evaluated, such as activity (Systemic Lupus Erythematosus Disease Activity Index-2K or SLEDAI-2K) and cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index or SDI). Treatment (glucocorticoids, immunosuppressive drugs and antimalarials) was also recorded. Univariable and multivariable Cox regression models were used to determine the impact of serum uric acid levels on the risk of new damage. Results: We evaluated 237 patients, with a mean age (SD) at diagnosis of 35.9 (13.1) years; 220 patients (92.8%) were women, and the duration of the disease was 7.3 (6.6) years. The mean SLEDAI-2K and SDI scores were 5.1 (4.2) and 0.9 (1.3), respectively. Serum uric acid level was 4.5 (1.4) mg/dL. Follow-up time was 3.1 (1.3) years, and 112 (47.3%) patients accrued damage during follow-up. In univariable and multivariable analyses, serum uric acid levels were associated with new damage (HR=1.141 (95% CI 1.016 to 1.282), p=0.026; HR=1.189 (95% CI 1.025 to 1.378), p=0.022, respectively). Conclusion: Higher serum uric acid levels are associated with global damage in patients with SLE.

Reliability of Visual Analog Scale and Numeric Rating Scale for the Assessment of Disease Activity in Systemic Lupus Erythematosus.

OBJECTIVE: To determine the reliability of SLE patients' disease activity measurements. METHODS: This was a cross-sectional study conducted (August 2016-December 2017) at 2 main public Peruvian hospitals, 1 with a comprehensive lupus care program. Patients assessed their disease activity with a visual analog scale (VAS) (0-100 mm) or a numerical rating scale (NRS) (0-4) before and after their physician's (MD's) assessment. Demographic and disease-related characteristics were recorded. Reliability of patients' disease activity before and after MD's assessment was determined using Spearman rank correlation. Factors possibly associated with this variability were examined with Spearman rank correlation and Mann-Whitney U test. RESULTS: Two hundred forty, mostly Mestizo, SLE patients were included; mean (SD) age and disease duration (diagnosis) were 34.9 (12.9) years and 10.1 (7.0) years, respectively. The Mexican version of the Systemic Lupus Erythematosus Disease Activity Index was 1.9 (2.7), and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was 1.2 (1.5). The correlations between NRS and VAS before and after the MD's assessment were ρ = 0.839; p < 0.001; and ρ = 0.872; p < 0.001, respectively. Visual analog scale and NRS were higher before than after the MD's assessment (VAS 29.3 [26.5] and 26.5 [24.9], p = 0.052; and NRS (1.5 [1.2] and 1.3 [1.1], p = 0.003); only the comprehensive program explained this variability (p = 0.043). The reliability of VAS and NRS was ρ = 0.917 and ρ = 0.861, p < 0.001, before and after for the comprehensive program and ρ = 0.710 and ρ = 0.785, p < 0.001, for before and after for the regular program. CONCLUSIONS: Both VAS an NRS are highly reliable. Patients scored higher before than after their physicians' assessment but that these differences were smaller for the patients in the comprehensive care program than in the regular one.

Discrepant Perception of Lupus Disease Activity: A Comparison Between Patients' and Physicians' Disease Activity Scores.

OBJECTIVE: The aim of this study was to compare patient and physician (MD) assessment of disease activity in systemic lupus erythematosus patients. METHODS: This cross-sectional study was conducted between August 2016 and December 2017 at 2 Peruvian hospitals. One group assessed disease activity using a visual analog scale (VAS, 0-100 mm) and the other one using a numerical rating scale (NRS, 0-4), before and after their MD's visit. MDs assessed it with the Mexican Systemic Lupus Erythematosus Disease Activity (Mex-SLEDAI) (0-32) and with the SLICC/ACR Damage Index (SDI) for damage. Health-related quality of life was ascertained with the LupusQoL. Visual analog scale and NRS were compared using the Wilcoxon signed-rank test and the correlation between disease activity as assessed by the patient and the Mex-SLEDAI, SDI, and LupusQoL with the Spearman rank correlation. RESULTS: Two hundred forty patients were included; mean (SD) age at diagnosis was 34.9 (12.9) years; most patients were Mestizo. Disease duration was 10.1 (7.0) years. The Mex-SLEDAI was 1.9 (2.7) and the SDI 1.2 (1.5). Disease activity as assessed by the patient, either by VAS or NRS, did not correlate with the Mex-SLEDAI or the SDI. In contrast, patient assessment of disease activity, by VAS or NRS, significantly correlated with several components of the LupusQoL (physical health, pain, planning, emotional health, and fatigue). CONCLUSIONS: Physician's and patient's assessments of disease activity are discrepant; overall, patients score higher than their MDs. Patients score how they perceive the disease is affecting them, rather than disease activity per se. The VAS could be more useful than the NRS as a measurement of disease activity.

Better Health-Related Quality of Life in Systemic Lupus Erythematosus Predicted by Low Disease Activity State/Remission: Data From the Peruvian Almenara Lupus Cohort.

OBJECTIVE: To determine if low disease activity state (LDAS)/remission predicts a better health-related quality of life (HRQoL). METHODS: Patients with systemic lupus erythematosus from a single center and having completed at least 2 visits were included. Visits were performed every 6 months. HRQoL was measured with the LupusQoL questionnaire. The definition of remission included a Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score of 0, prednisone daily dosage of ≤5 mg/day, and immunosuppressive drugs on maintenance dose. LDAS was defined as a SLEDAI-2K score of ≤4, prednisone daily dosage of ≤7.5 mg/day, and immunosuppressive drugs as maintenance therapy. For these analyses, remission and LDAS were combined as one variable. Generalized estimating equations were calculated, using as the outcome 1 of each of the 8 components of the LupusQoL questionnaire in the subsequent visit and the activity state in the previous visit. Multivariable models were adjusted for possible confounders. RESULTS: A total of 243 patients were included. During the follow-up, 590 visits (61.6%) were categorized as LDAS/remission. LDAS/remission predicted a better HRQoL in the components of physical health (B = 4.17 [95% confidence interval (95% CI) 1.20, 7.14]; P = 0.006), pain (B = 6.47 [95% CI 3.18, 9.76]; P < 0.001), planning (B = 4.97 [95% CI 1.43, 8.52]; P = 0.006), burden to others (B = 4.12 [95% CI 0.24, 8.01]; P = 0.037], emotional health (B = 4.50 [95% CI 1.56, 7.44]; P = 0.003), and fatigue (B = 3.25 [95% CI 0.04, 6.47]; P = 0.048). CONCLUSION: Being in LDAS/remission predicts a better HRQoL, especially in the components of physical health, pain, planning, burden to others, emotional health, and fatigue.

IFN-α kinoid in systemic lupus erythematosus: results from a phase IIb, randomised, placebo-controlled study.

OBJECTIVE: To evaluate the efficacy and safety of the immunotherapeutic vaccine interferon-α kinoid (IFN-K) in a 36-week (W) phase IIb, randomised, double-blind, placebo (PBO)-controlled trial in adults with active systemic lupus erythematosus (SLE) despite standard of care. METHODS: Patients with SLE (185) with moderate to severe disease activity and positive interferon (IFN) gene signature were randomised to receive IFN-K or PBO intramuscular injections (days 0, 7 and 28 and W12 and W24). Coprimary endpoints at W36 were neutralisation of IFN gene signature and the BILAG-Based Composite Lupus Assessment (BICLA) modified by mandatory corticosteroid (CS) tapering. RESULTS: IFN-K induced neutralising anti-IFN-α2b serum antibodies in 91% of treated patients and reduced the IFN gene signature (p<0.0001). Modified BICLA responses at W36 did not statistically differ between IFN-K (41%) and PBO (34%). Trends on Systemic Lupus Erythematosus Responder Index-4, including steroid tapering at W36, favoured the IFN-K and became significant (p<0.05) in analyses restricted to patients who developed neutralising anti-IFN-α2b antibodies. Attainment of lupus low disease activity state (LLDAS) at W36 discriminated the two groups in favour of IFN-K (53% vs 30%, p=0.0022). A significant CS sparing effect of IFN-K was observed from W28 onwards, with a 24% prednisone daily dose reduction at W36 in IFN-K compared with PBO (p=0.0097). The safety profile of IFN-K was acceptable. CONCLUSIONS: IFN-K induced neutralising anti-IFN-α2b antibodies and significantly reduced the IFN gene signature with an acceptable safety profile. Although the clinical coprimary endpoint was not met, relevant secondary endpoints were achieved in the IFN-K group, including attainment of LLDAS and steroid tapering. TRIAL REGISTRATION NUMBER: NCT02665364.