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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., Competing Interests: M.F.U.-G. has grant support from Janssen and Pfizer, and he has been speaker for GSK and AztraZeneca. The other authors have disclosed no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: M.F.U.-G. has grant support from Janssen and Pfizer, and he has been a speaker for GSK. The other authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: M.F.U.-G. has grant support from Janssen and Pfizer, has been speaker for GSK and AztraZeneca, and has been member of the advisory boards for AztraZeneca and Ferrer; R.V.G.-C. has grant support from Pfizer; C.R.-S. and V.R.P.-Q. have grant support from Janssen. The other authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: Competing interests: MFU-G has grant support from Janssen and Pfizer and he has been speaker for GSK; RVG-C has grant support from Pfizer; CR-S and VRP-Q have grant support from Janssen. All other authors declare to have no conflicts of interest., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Aim: To determine characteristics associated with more severe outcomes in a global registry of people with systemic lupus erythematosus (SLE) and COVID-19., Methods: People with SLE and COVID-19 reported in the COVID-19 Global Rheumatology Alliance registry from March 2020 to June 2021 were included. The ordinal outcome was defined as: (1) not hospitalised, (2) hospitalised with no oxygenation, (3) hospitalised with any ventilation or oxygenation and (4) death. A multivariable ordinal logistic regression model was constructed to assess the relationship between COVID-19 severity and demographic characteristics, comorbidities, medications and disease activity., Results: A total of 1606 people with SLE were included. In the multivariable model, older age (OR 1.03, 95% CI 1.02 to 1.04), male sex (1.50, 1.01 to 2.23), prednisone dose (1-5 mg/day 1.86, 1.20 to 2.66, 6-9 mg/day 2.47, 1.24 to 4.86 and ≥10 mg/day 1.95, 1.27 to 2.99), no current treatment (1.80, 1.17 to 2.75), comorbidities (eg, kidney disease 3.51, 2.42 to 5.09, cardiovascular disease/hypertension 1.69, 1.25 to 2.29) and moderate or high SLE disease activity (vs remission; 1.61, 1.02 to 2.54 and 3.94, 2.11 to 7.34, respectively) were associated with more severe outcomes. In age-adjusted and sex-adjusted models, mycophenolate, rituximab and cyclophosphamide were associated with worse outcomes compared with hydroxychloroquine; outcomes were more favourable with methotrexate and belimumab., Conclusions: More severe COVID-19 outcomes in individuals with SLE are largely driven by demographic factors, comorbidities and untreated or active SLE. Patients using glucocorticoids also experienced more severe outcomes., Competing Interests: Competing interests: MFU-G has received research grants from Pfizer and Janssen, not related to this manuscript. AD-G is supported by the Rheumatology Research Foundation (Scientist Development Award) and the Centers for Disease Control and Prevention. CR-S has received research grants from Janssen, not related to this manuscript. AEC has received consulting fees from AstraZeneca, BMS and GSK, all unrelated to this manuscript. LW has received consulting fees and speaker’s honoraria from Aurinia Pharma unrelated to this manuscript. GJP-E reports no competing interests related to this work. Outside of this work, he reports personal consulting and/or speaking fees from Pfizer, GSK, Janssen and Sanofi (all

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., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: Competing interests: MFU-G has grant support from Janssen and Pfizer. RVG-C has grant support from Pfizer. CR-S and VRP-Q have grant support from Janssen. FZ and CSK are employees of Janssen Scientific Affairs. All other authors declare to have no other conflicts of interest., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Competing Interests: Competing interests: MFU-G has grant support from Janssen and Pfizer. RVG-C has grant support from Pfizer. CR-S and VRP-Q have grant support from Janssen. JL, FZ and CSK are employees of Janssen Scientific Affairs. All other authors declare no other conflicts of interest., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: This study aimed to determine the prevalence of ultrasound-detected tendon abnormalities in healthy subjects (HS) across the age range., Methods: Adult HS (age 18-80 years) were recruited in 23 international Outcome Measures in Rheumatology ultrasound centres and were clinically assessed to exclude inflammatory diseases or overt osteoarthritis before undergoing a bilateral ultrasound examination of digit flexors (DFs) 1-5 and extensor carpi ulnaris (ECU) tendons to detect the presence of tenosynovial hypertrophy (TSH), tenosynovial power Doppler (TPD) and tenosynovial effusion (TEF), usually considered ultrasound signs of inflammatory diseases. A comparison cohort of patients with rheumatoid arthritis (RA) was taken from the Birmingham Early Arthritis early arthritis inception cohort., Results: 939 HS and 144 patients with RA were included. The majority of HS (85%) had grade 0 for TSH, TPD and TEF in all DF and ECU tendons examined. There was a statistically significant difference in the proportion of TSH and TPD involvement between HS and subjects with RA (HS vs RA p<0.001). In HS, there was no difference in the presence of ultrasound abnormalities between age groups., Conclusions: Ultrasound-detected TSH and TPD abnormalities are rare in HS and can be regarded as markers of active inflammatory disease, especially in newly presenting RA., Competing Interests: Competing interests: There are no declared competing interests from authors except the following: CC declared grants from NIHR Versus Arthritis, Lilly sponsored EULAR conference travel, Modern Biosciences payment as DSM committee member, Roche consultancy fee and Novartis sponsored writing of one medical paper. KI declared a Mitsubishi-Tanabe research grant for RA; Abbvie, Eli Lilly, Mitsubishi-Tanabe, Bristol-Myers-Squib and Novartis speaker’s fees; and participation on a DSM board for Abbvie, Eli Lilly and Mitsubishi-Tanabe. RK declared support from Abbvie, Roche, Novartis and UCB with payments for travel to meetings/lectures, presentations, speakers’ bureaus, manuscript writing/educational events., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.)

Objective: In systemic lupus erythematosus (SLE), disease activity and glucocorticoid (GC) exposure are known to contribute to irreversible organ damage. We aimed to examine the association between GC exposure and organ damage occurrence., Methods: We conducted a literature search (PubMed (Medline), Embase and Cochrane January 1966-October 2021). We identified original longitudinal observational studies reporting GC exposure as the proportion of users and/or GC use with dose information as well as the occurrence of new major organ damage as defined in the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. Meta-regression analyses were performed. Reviews, case-reports and studies with <5 years of follow-up, <50 patients, different outcomes and special populations were excluded., Results: We selected 49 articles including 16 224 patients, 14 755 (90.9%) female with a mean age and disease duration of 35.1 years and of 37.1 months. The mean follow-up time was 104.9 months. For individual damage items, the average daily GC dose was associated with the occurrence of overall cardiovascular events and with osteoporosis with fractures. A higher average cumulative dose adjusted (or not)/number of follow-up years and a higher proportion of patients on GC were associated with the occurrence of osteonecrosis., Conclusions: We confirm associations of GC use with three specific damage items. In treating patients with SLE, our aim should be to maximise the efficacy of GC and to minimise their harms., Competing Interests: Competing interests: JB and RFvV are Editors-in-Chief of Lupus Science & Medicine., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The identification of risk factors for COVID-19 adverse course in autoimmune rheumatic diseases (ARDs) is of the utmost importance when approaching patient management; however, data are scarce in relation to the Latin American population. The objective of this study was to determine predictors of hospitalization for COVID-19 patients from an ARD community cohort., Methods: A real setting longitudinal study (March to November 2020) in an ARD community cohort was carried out. Potential predictors of hospitalization for COVID-19 examined included (1) sociodemographic variables (age, gender, education, tobacco use, socioeconomic status, and co-inhabitants), (2) comorbidities, (3) time to COVID-19 diagnosis, and (4) ARD's features: clinical (disease type, disease duration, activity), treatment [corticosteroids use/doses, use of synthetic DMARDs (cDMARDs, tsDMARDs, and bDMARDs)], treatment schedule and non-adherence, and the Multidimensional Health Assessment Questionnaire (MDHAQ). Univariable and multivariable regression analysis were conducted; OR and 95% CI (p < 0.05) were determined., Results: One thousand and one hundred forty-eight patients with ARDs were included; 154 had COVID-19; of these 139 (90.3%) were women, aged 52.5 (13.7) years; 33.1% had hypertension and 61.0% an affected organ by ARD. Infection was detected 8.4 (10.1) days after symptoms started; there were 33 hospitalized patients (rate 21.4%). Predictors of hospitalization by multivariable analysis were age (OR: 1.06; CI: 1.01-1.10; p: 0.01), comorbidities: hypertension (OR: 3.95; 95% CI: 1.40-10.95, p: 0.01) and neoplasm (OR: 9.0; 95% CI: 1.6-52.3; p: 0.01), number of organs involved by ARD (OR: 2.26; 95% CI: 1.16-4.41; p: 0.02), and infection diagnosis delay (OR: 1.36; 95% CI: 1.03-1.80; p: 0.01)., Conclusions: In our ARD patients with COVID-19, older age, comorbidities (neoplasm and hypertension), and a delay in COVID-19 diagnosis were predictors of hospitalization. The only ARD-associated predictor feature was the number of organs involved. Key Points • Patients with ARD and COVID-19 have an adverse course in comparison to the general population. • Previous predictors of COVID-19 hospitalization, including known risk factors (such as older age and comorbidities) and systemic manifestations, should be taken into account in the management of these patients. • Delayed diagnosis of COVID-19 impacts negatively on prognosis. • Availability of diagnostic tests is of utmost importance., (© 2021. International League of Associations for Rheumatology (ILAR).)

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

Background: Remission and low disease activity (LDA) have been proposed as the treatment goals for patients with systemic lupus erythematosus (SLE). Several definitions for each have been proposed in the literature., Objective: To assess the impact of remission/LDA according to various definitions on relevant outcomes in patients with SLE., Methods: This systematic literature review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses using PubMed (1946-week 2, April 2021), Cochrane library (1985-week 2, week 2, April 2021) and EMBASE (1974-week 2, April 2021). We included longitudinal and cross-sectional studies in patients with SLE reporting the impact of remission and LDA (regardless their definition) on mortality, damage accrual, flares, health-related quality of life and other outcomes (cardiovascular risk, hospitalisation and direct costs). The quality of evidence was evaluated using the Newcastle-Ottawa Scale., Results: We identified 7497 articles; of them, 31 studies met the inclusion criteria and were evaluated. Some articles reported a positive association with survival, although this was not confirmed in all of them. Organ damage accrual was the most frequently reported outcome, and remission and LDA were reported as protective of this outcome (risk measures varying from 0.04 to 0.95 depending on the definition). Similarly, both states were associated with a lower probability of SLE flares, hospitalisations and a better health-related quality of life, in particular the physical domain., Conclusion: Remission and LDA are associated with improvement in multiple outcomes in patients with SLE, thus reinforcing their relevance in clinical practice., Prospero Registration Number: CRD42020162724., Competing Interests: Competing interests: MFU-G: Grant/research support from Jannsen, Pfizer, not related to this article. CM-P declared no conflict of interest. CR-S: Grant/research support from Jannsen, not related to this article. GP-E: Grant/research support from JANSSEN and GSK; consultant of JANNSEN, GSK and SANOFI; speakers bureau: Pfizer, JANNSEN and GSK, not related to this article. RFvV: Grant/research support from AbbVie, Arthrogen, Bristol-Myers Squibb, GlaxoSmithKline, Lilly, Pfizer and UCB; consultant of AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, GSK, Janssen, Lilly, Medac, Merck, Novartis, Pfizer, Roche and UCB, not related to this article. GB: Grant/research support from GSK, Pfizer; consultant of Novartis, SOBI, not related to this article. GA declared no conflict of interest. BAP-E: Grant/research support from GSK, Jannsen; consultant of GSK, Janssen; speakers bureau: GSK, Janssen, not related to this article., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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

Introduction: After more than 20 years of sustained work, the Latin American Group for the Study of Lupus (GLADEL) has made a significant number of contributions to the field of lupus, not only in the differential role that race/ethnicity plays in its course and outcome but also in several other studies including the beneficial effects of using antimalarials in lupus patients and the development of consensus guidelines for the treatment of lupus in our region. Methods: A new generation of "Lupus Investigators" in more than 40 centers throughout Latin America has been constituted in order to continue the legacy of the investigators of the original cohort and to launch a novel study of serum and urinary biomarkers in patients with systemic lupus erythematosus. Results: So far, we have recruited 807 patients and 631 controls from 42 Latin-American centers including 339 patients with SLE without renal involvement, 202 patients with SLE with prevalent but inactive renal disease, 176 patients with prevalent and active renal disease and 90 patients with incident lupus nephritis. Conclusions: The different methodological aspects of the GLADEL 2.0 cohort are discussed in this manuscript, including the challenges and difficulties of conducting such an ambitious project., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: Serological biomarkers will be carried out centrally by INOVA/Werfen laboratories. Janssen Pharmaceutical Companies of Johnson & Johnson is planned to provide logistic and financial support for transcriptome studies. Neither Inova laboratories nor Janssen Pharmaceutical Companies have participated or influenced the development of the project, nor do they have access to the information collected in the database.

Aim: A decrease in proteinuria has been considered protective from renal damage in lupus nephritis (LN), but a cut-off point has yet to be established. The aim of this study was to identify the predictors of renal damage in patients with LN and to determine the best cut-off point for a decrease in proteinuria., Methods: We included patients with LN defined clinically or histologically. Possible predictors of renal damage at the time of LN diagnosis were examined: proteinuria, low complement, anti-double-stranded DNA antibodies, red cell casts, creatinine level, hypertension, renal activity (assessed by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)), prednisone dose, immunosuppressive drugs and antimalarial use. Sociodemographic variables were included at baseline. Proteinuria was assessed at baseline and at 12 months, to determine if early response (proteinuria <0.8 g/day within 12 months since LN diagnosis) is protective of renal damage occurrence. Renal damage was defined as an increase of one or more points in the renal domain of The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI). Cox regression models using a backward selection method were performed., Results: Five hundred and two patients with systemic lupus erythematosus patients were included; 120 patients (23.9%) accrued renal damage during their follow-up. Early response to treatment (HR=0.58), antimalarial use (HR=0.54) and a high SES (HR=0.25) were protective of renal damage occurrence, whereas male gender (HR=1.83), hypertension (HR=1.86) and the renal component of the SLEDAI (HR=2.02) were risk factors for its occurrence., Conclusions: Early response, antimalarial use and high SES were protective of renal damage, while male gender, hypertension and higher renal activity were risk factors for its occurrence in patients with LN., 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.)

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.

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., 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.)

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.

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., (© 2019, American College of Rheumatology.)

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., 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.)

The affiliations of Manuel F. Ugarte-Gil, one of the author of the above article has a discrepancy between the online version of the publisher's internet portal ( Springerlink.com ) and the online pdf version.

Objective: To determine the impact of homocysteine levels on damage accrual in systemic lupus erythematosus (SLE) patients., Methods: This longitudinal study was conducted in consecutive patients seen every 6 months at our Rheumatology Department since 2012. Patients with available homocysteine levels and who had at least one subsequent visit were included. Univariable and multivariable Cox regression models were done to determine if homocysteine levels were predictive of damage accrual as per the SLICC Damage Index (SDI). The multivariable model was adjusted for pertinent variables (age at diagnosis, gender, socioeconomic status, disease duration, disease activity (SLEDAI), Framingham score, antimalarial and immunosuppressive drug use, average daily dose, and exposure time to prednisone (PDN))., Results: One hundred forty-five patients were included; their mean (SD) age at diagnosis was 43.70 (12.09) years, 136 (93.8%) were female, and nearly all were Mestizo. At baseline, disease duration was 7.55 (6.73) years; patients were followed for 3.54 (1.27) years. The SLEDAI was 5.60 (4.34), and the SDI 0.97 (1.35). The average daily PDN dose was 7.30 (5.78) mg/day and the time of PDN exposure was 7.36 (6.73) years. Mean homocysteine levels were 10.07 (3.71) μmol/L. The highest tertile of homocysteine levels predicted new damage accrual in the univariable and multivariable models [HR 1.78 (95% CI, 1.042-3.039); p = 0.035 and HR 2.045 (95% CI, 1.077-3.883); p = 0.029, respectively]. Increased levels (> 15 μmol/L) were found in 12 (8.3%) patients; 75 (51.7%) patients increased ≥ 1 SDI point., Conclusion: In SLE patients, homocysteine levels predicted damage accrual independently of other well-known risk factors for such occurrence.

Objectives: To determine whether the CD4+CD28null T-cells subpopulation predicts the occurrence of damage in SLE., Methods: This longitudinal study was conducted in consecutive SLE patients seen every six months in our Rheumatology Department since 2012. Patients in whom CD4+CD28null T-cells had been measured and who had at least one subsequent visit were included in the study. Survival analyses (univariable and multivariable Cox-regression models) were performed to determine the risk of overall and domain damage (as per the SLICC Damage Index - SDI) as a function of the frequency of this T-cell subpopulation. The multivariable model was adjusted for pertinent confounders. All analyses were performed using SPSS 21.0., Results: One hundred and nineteen patients were evaluated; their mean (SD) age was 43.5 (11.9) years, 113 (95.0%) were female. Disease duration was 7.8 (7.0) years, the SLEDAI 5.3 (4.1) and the SDI 1.0 (1.4). The percentage of CD4+CD28null T-cells was 17.4 (14.0). The mean follow-up was 2.1 (0.8) years, and the mean number of visits per patient 3.5 (1.1). Forty-six (38.7%) patients increase at least one SDI point. In the univariable and multivariable analyses, the percentage of CD4+CD28null predicted the occurrence of lung damage [HR: 1.042 (CI95%: 1.001-1.085); p=0.047 and HR: 1.099 (CI95%1.020-1.184); p=0.013, respectively] but neither the total SDI score nor all other SDI domain scores were predicted by the percentage of CD4+CD28null cells., Conclusions: In SLE patients, CD4+CD28null T-cells predict the occurrence of new lung damage, independently of other risk factors but not of overall damage or damage on other domains.