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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.

Introduction Factors related to presentation of neuropsychiatric (NP) SLE manifestations, early in the course of the disease, and during follow up have not been clearly established. Purpose To identify disease and non-disease related factors associated with NP manifestations in early SLE. Methods We included 1193 patients from the GLADEL inception cohort free of NP involvement at cohort entry. We evaluated the association of demographic, clinical and laboratory data with NP involvement during follow-up. Statistical methods Independent factors associated with NP involvement were identified using a multivariable Cox regression model. Results Factors independently associated with NP manifestations were: mestizo ethnicity (HR 1.701, 95% CI 1.282–2.258, p = 0.0002), myalgias/myositis (HR 1.832, 95% CI 1.335–2.515, p = 0.0002), pneumonitis (HR 2.476, 95% CI 1.085–5.648, p = 0.0312), shrinking lung (HR 2.428, 95% CI 1.074–5.493, p = 0.0331) and hemolytic anemia (HR 1.629, 95% CI 1.130–2.347, p = 0.0089). Longer disease duration at cohort entry (13 to 24 months) was associated with a lower risk of developing NP manifestations (HR 0.642, 95% CI 0.441–0.934, p = 0.0206). Conclusions Patients with myalgias/myositis, pneumonitis, shrinking lung and hemolytic anemia are at higher risk of NP involvement, whereas longer disease duration at cohort entry is associated with a lower risk of developing NP involvement.

Objective.To determine the predictors of remission and low disease activity state (LDAS) in patients with systemic lupus erythematosus (SLE).Methods.Three disease activity states were defined: Remission = SLE Disease Activity Index (SLEDAI) = 0 and prednisone ≤ 5 mg/day and/or immunosuppressants (maintenance dose); LDAS = SLEDAI ≤ 4, prednisone ≤ 7.5 mg/day and/or immunosuppressants (maintenance dose); and non-optimally controlled state = SLEDAI > 4 and/or prednisone > 7.5 mg/day and/or immunosuppressants (induction dose). Antimalarials were allowed in all groups. Patients with at least 2 SLEDAI reported and not optimally controlled at entry were included in these analyses. Outcomes were remission and LDAS. Multivariable Cox regression models (stepwise selection procedure) were performed for remission and for LDAS.Results.Of 1480 patients, 902 were non-optimally controlled at entry; among them, 196 patients achieved remission (21.7%) and 314 achieved LDAS (34.8%). Variables predictive of a higher probability of remission were the absence of mucocutaneous manifestations (HR 1.571, 95% CI 1.064–2.320), absence of renal involvement (HR 1.487, 95% CI 1.067–2.073), and absence of hematologic involvement (HR 1.354, 95% CI 1.005–1.825); the use of immunosuppressive drugs before the baseline visit (HR 1.468, 95% CI 1.025–2.105); and a lower SLEDAI score at entry (HR 1.028, 95% CI 1.006–1.051 per 1-unit decrease). These variables were predictive of LDAS: older age at entry, per 5-year increase (HR 1.050, 95% CI 1.004–1.098); absence of mucocutaneous manifestations (HR 1.401, 95% CI 1.016–1.930) and renal involvement (HR 1.344, 95% CI 1.049–1.721); and lower SLEDAI score at entry (HR 1.025, 95% CI 1.009–1.042).Conclusion.Absence of mucocutaneous, renal, and hematologic involvement, use of immunosuppressive drugs, and lower disease activity early in the course of the disease were predictive of remission in patients with SLE; older age was predictive of LDAS.

Objectives This study aimed to compare the clinical features, damage accrual, and survival of patients with familial and sporadic systemic lupus erythematosus (SLE). Methods A multi-ethnic, multinational Latin American SLE cohort was studied. Familial lupus was defined as patients with a first-degree SLE relative; these relatives were interviewed in person or by telephone. Clinical variables, disease activity, damage, and mortality were compared. Odds ratios (OR) and 95% confidence intervals (CI) were estimated. Hazard ratios (HR) were calculated using Cox proportional hazard adjusted for potential confounders for time to damage and mortality. Results A total of 66 (5.6%) patients had familial lupus, and 1110 (94.4%) had sporadic lupus. Both groups were predominantly female, of comparable age, and of similar ethnic distribution. Discoid lupus (OR = 1.97; 95% CI 1.08–3.60) and neurologic disorder (OR = 1.65; 95% CI 1.00–2.73) were significantly associated with familial SLE; pericarditis was negatively associated (OR = 0.35; 95% CI 0.14–0.87). The SLE Disease Activity Index and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) were similar in both groups, although the neuropsychiatric (45.4% vs. 33.5%; p = 0.04) and musculoskeletal (6.1% vs. 1.9%; p = 0.02) domains of the SDI were more frequent in familial lupus. They were not retained in the Cox models (by domains). Familial lupus was not significantly associated with damage accrual (HR = 0.69; 95% CI 0.30–1.55) or mortality (HR = 1.23; 95% CI 0.26–4.81). Conclusion Familial SLE is not characterized by a more severe form of disease than sporadic lupus. We also observed that familial SLE has a higher frequency of discoid lupus and neurologic manifestations and a lower frequency of pericarditis.

To identify baseline predictors of remission and low disease activity (LDA) in early rheumatoid arthritis (RA) from the GLADAR (Grupo Latino Americano De estudio de la Artritis Reumatoide) cohort.Patients with 1- and 2-year follow-up visits were included. Remission and LDA were defined by DAS28-ESR ( 2.6 and ≤ 3.2, respectively). Baseline predictors examined were gender, ethnicity, age at diagnosis, socioeconomic status, symptoms' duration, DMARDs, RF, thrombocytosis, anemia, morning stiffness, DAS28-ESR (and its components), HAQ-DI, DMARDs and corticosteroid use, and Sharp-VDH score. Multivariable binary logistic regression models (excluding DAS28-ESR components to avoid over adjustment) were derived using a backward selection method (α-level set at 0.05).Four hundred ninety-eight patients were included. Remission and LDA/remission were met by 19.3% and 32.5% at the 1-year visit, respectively. For the 280 patients followed for 2 years, these outcomes were met by 24.3% and 38.9%, respectively. Predictors of remission at 1 year were a lower DAS28-ESR (OR 1.17; CI 1.07-1.27; p = 0.001) and HAQ-DI (OR 1.48; CI 1.04-2.10; p = 0.028). At 2 years, only DAS28-ESR (OR 1.40; CI 1.17-1.6; p 0.001) was a predictor. Predictors of LDA/remission at 1 year were DAS28-ESR (OR 1.42; CI 1.26-1.61; p 0.001), non-use of corticosteroid (OR 1.74; CI 1.11-2.44; p = 0.008), and male gender (OR 1.77; CI 1.2-2.63; p = 0.036). A lower baseline DAS28-ESR (OR 1.45; CI 1.23-1.70; p 0.001) was the only predictor of LDA/remission at 2 years.A lower disease activity consistently predicted remission and LDA/remission at 1 and 2 years of follow-up in early RA patients from the GLADAR cohort. Key Points • In patients with early RA, a lower disease activity at first visit is a strong clinical predictor of achieving remission and LDA subsequently. • Other clinical predictors of remission and LDA to keep in mind in these patients are male gender, non-use of corticosteroids and low disability at baseline. • Not using corticosteroids at first visit is associated with a lower disease activity and predicts LDA/remission at 1 year in these patients.

Background We have previously shown that Latin American SLE patients treated with Antimalarials (AMs) have a 25% lower risk of damage accrual than patients not receiving them. The present study was conducted to assess the effects of AMs over the 12 items of the SLICC Damage Index, (SDI). Methods Patients with a recent SLE diagnosis (≤2 years) from the GLADEL cohort were studied. End-point: Increase in the 12 items SDI since cohort entry. Independent (socio-demographic, clinical laboratory and treatment) variables were included. The effect of AMs as a time dependent variable on items of the SDI (adjusting for potential confounders) was examined with a multivariable Cox regression model. Multivariate models were developed for the most common SDI items. Results Of the 1466 patients included in this analysis 1049 (72%) received AMs during follow-up (as defined); median exposure time: 30 months (Q1-Q3: 11–57 months). Total damage accrual occurred in 665 (45%) patients during a median follow up time of 24 months (Q1-Q3: 8–55) months. Within the 12 items of the SDI there were 301 integument, 208 renal, 149 neuropsychiatric, 98 musculoskeletal, 88 cardiovascular, 65 ocular, 43 pulmonary, 42 peripheral vascular, 33 gastrointestinal, 22 premature gonadal failure, 16 diabetes and 9 malignancy. After adjusting for potential confounders, at any time during follow-up a patient on AMs had a 35% and 30% lower risk of renal and neuropsychiatric damage accrual respectively than a patient not on AMs (adjusted HR 0.65, 95% CI 0.47 to 0.90 and HR 0.70, 95% CI 0.48 to 1.02). Such protective effect was not evident for integument, musculoskeletal and cardiovascular damage. Table 1. Conclusions After adjustment for possible confounding factors related to AMs use and damage accrual, AMs were independently associated with a reduced risk of renal and neuropsychiatric damage accrual in this cohort. Acknowledgements On behalf of the Grupo Latinoamericano de Estudio del Lupus (GLADEL).

The original version of this article, unfortunately, contained an error. The first and family name of Loreto Massardo was interchanged and is now presented correctly in this article.

Background and aims Remission and LLDAS prevent the occurrence of damage accrual in SLE patients. The aim of this study was to evaluate the predictors of remission and LLDAS in SLE patients. Methods Three disease activity statuses were defined: Remission= SLEDAI=0 and a prednisone dose ≤5 mg/d and/or immunosuppressive drugs in maintenance dose; LLDAS=SLEDAI≤4, a prednisone dose ≤7.5 mg/d and/or immunosuppressive drugs in maintenance dose; and non-optimally controlled status= SLEDAI >4 and/or prednisone dose >7.5 mg/d and/or IS drugs in induction dose. Antimalarials were allowed in all groups. Patients with at least two SLEDAI reported and not optimally controlled at cohort entry were included in this analysis. Predefined outcomes were remission and remission/LLDAS. Potential predictors were gender, age at diagnosis, ethnicity, socioeconomic status, residence, health insurance, disease duration at cohort entry, organs/systems affected at or before cohort entry, treatment at or before cohort entry and SLEDAI at cohort entry. Univariable and multivariable Cox regression models with a stepwise selection procedure were performed for remission alone and for remission/LLDAS. Results One-thousand one-hundred and forty patients were non-optimally controlled at cohort entry. One hundred and ninety-six patients achieved remission (17.2%) and 314 achieved remission/LLDAS (27.5%). Predictors of remission and remission/LLDAS in the multivariable models are depicted in Tables 1 and 2. Conclusions Mucocutaneous manifestations, renal involvement and higher disease activity early in the course of SLE were associated with a reduced risk of remission and remission/LLDAS; lower socioeconomic status was associated with a reduced risk of remission. A medium prednisone dose was associate with an increased risk of remission/LLDAS.

>> Objective: The objective of this paper is to examine the role of place of residency in the expression and outcomes of systemic lupus erythematosus (SLE) in a multi-ethnic Latin American cohort. Patients and methods: SLE patients (

We have previously developed and validated a self-administered questionnaire, modelled after the Systemic Lupus International Collaborating Clinics Damage Index (SDI), the Lupus Damage Index Questionnaire (LDIQ), which may allow the ascertainment of this construct in systemic lupus erythematosus (SLE) patients followed in the community and thus expand observations made about damage. We have now translated, back-translated and adapted the LDIQ to Spanish, Portuguese and French and applied it to patients followed at academic and non-academic centres in North and South America, Portugal and Spain while their physicians scored the SDI. A total of 887 patients (659 Spanish-speaking, 140 Portuguese-speaking and 80 French-speaking patients) and 40 physicians participated. Overall, patients scored all LDIQ versions higher than their physicians (total score and all domains). Infrequent manifestations had less optimal clinimetric properties but overall agreement was more than 95% for the majority of items. Higher correlations were observed among the Spanish-speaking patients than the Portuguese-speaking and French-speaking patients; further adjustments may be needed before the Portuguese and French versions of the LDIQ are applied in community-based studies. The relationship between the LDIQ and other outcome parameters is currently being investigated in a different patient sample.

Objective To examine hematological manifestations' correlates and their impact on damage accrual and mortality in SLE patients from the multiethnic, Latin American, GLADEL cohort. Methods In patients with recent SLE diagnosis (≤2 years), the association between follow-up hematological manifestations (per ACR criteria) and socio-demographic and clinical variables was examined by univariable and multivariable logistic regressions; their impact on damage accrual and mortality was examined by Poisson and Cox proportional-hazards regression analyses, respectively. Results Of 1437 patients, 948 (66.0%) developed ≥1 hematological manifestation [5.5% hemolytic anemia (AHA), 16.3% thrombocytopenia, and 56.4% lymphopenia] over 4.3 (3.3) follow-up years. Younger age, Mestizo ethnicity, hematologic disorder (at/or before SLE diagnosis), and first damage recorded were associated with hematological manifestations while antimalarials were negatively associated. AHA (at/or before SLE diagnosis), anti-Sm, and anti-RNP antibodies were associated with subsequent AHA occurrence while musculoskeletal involvement was negatively associated. Thrombocytopenia (at/or before SLE diagnosis), AHA, anti-phospholipid antibodies (aPLs), anti-SSA/Ro, anti-SSB/La antibodies, and first damage recorded were associated with later thrombocytopenia occurrence. Lymphopenia (at/or before SLE diagnosis), younger age at diagnosis, Mestizo ethnicity, having medical insurance, and first damage recorded were associated with subsequent lymphopenia occurrence while antimalarials and azathioprine treatment were negatively associated. AHA was associated with damage accrual and mortality after adjusting for variables known to affect these outcomes. Conclusions Mestizo ethnicity and early hematological manifestations are risk factors for their subsequent occurrence while antimalarials have a protective effect. The associations between AHA and aPLs and thrombocytopenia were corroborated. AHA contributes independently to damage accrual and diminished survival.

PurposeTo determine the association between the number of flares systemic lupus erythematosus (SLE) patients experience and damage accrual, independently of other known risk factors.MethodsSLE patients (34 centres, nine Latin American countries) with a recent diagnosis (≤2 years) and ≥3 evaluations were studied. Disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and damage with the SLICC/ACR Damage Index (SDI). Flare was defined as an increase ≥4 points in the SLEDAI between two study visits. An ambidirectional case- crossover design was used to determine the association between the number of flares and damage accrual.Results901 patients were eligible for the study; 500 of them (55.5%) experienced at least one flare, being the mean number of flares 0.9 (SD: 1.0). 574 intervals from 251 patients were included in the case-crossover design since they have case and control intervals, whereas, the remaining patients did not. Their mean age at diagnosis was 27.9 years (SD: 11.1), 213 (84.9%) were women. The mean baseline SDI and SLEDAI were 1.3 (1.3) and 13.6 (8.1), respectively. Other features were comparable to those of the entire sample. After adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (SDI) OR 2.05, 95% CI 1.43 to 2.94, pConclusionsThe number of flares patients experience, regardless of their severity, increases the risk of damage accrual, independently of other known risk factors.

Objective. To examine the role of ethnicity and the use of anti-malarials (protective) on lupus renal disease. Methods. A nested casecontrol study (1:2 proportion, n = 265 and 530) within GLADEL’s (Grupo Latino Americano De Estudio de Lupus) longitudinal inception cohort was carried out. The end-point was ACR renal criterion development after diagnosis. Cases and controls were matched for follow-up time (end-point or a comparable time, respectively). Renal disease predictors were examined by univariable and multivariable analyses. Additional analyses were done to determine if the protective effect of anti-malarials persisted after adjusting for intake-associated confounders. Results. Of the cases, 233 (87.9%) were women; their mean (S.D.) age at diagnosis was 28.0 (11.9) years and their median (Q3Q1 interquartile range) follow-up time for cases and controls was 8.3 months (Q3Q1: 23.5); 56.6% of the cases and 74.3% of the controls were anti-malarial users. Mestizo ethnicity [odds ratio (OR) 1.72, 95% CI 1.19, 2.48] and hypertension (OR 2.26, 95% CI 1.38, 3.70) were independently associated with a higher risk of renal disease, whereas anti-malarial use (OR 0.39, 95% CI 0.26, 0.58), older age at disease onset (OR 0.98, 95% CI 0.96, 0.99) and female gender (OR 0.56, 95% CI 0.32, 0.99) were negatively associated with such occurrence. After adjusting for variables associated with their intake, the protective effect of anti-malarials on renal disease occurrence persisted (OR 0.38, 95% CI 0.25, 0.58). Conclusion. Mestizo patients are at increased risk of developing renal disease, whereas anti-malarial use protects patients from such an occurrence.

Objective To determine the influence of socioeconomic factors on disease activity in a Latin American (LA) early rheumatoid arthritis (RA) multinational inception cohort at baseline. Methods Clinical evaluation, ethnicity, socioeconomic status (SES), 4-variable Disease Activity Score in 28 joints using the erythrocyte sedimentation rate (DAS28-ESR), Health Assessment Questionnaire (HAQ) disability index (DI), and erosions were recorded in 1,093 patients with early RA (