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

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.

Background/Purpose APS ACTION Registry was created to study the outcomes of patients with persistently positive antiphospholipid antibodies (aPL) with or without other systemic autoimmune disease (SAIDx). Given that immunosuppression (IS) is used for certain aPL manifestations, for example, thrombocytopenia (TP), our primary objective was to describe the indications for IS in aPL-positive patients without other SAIDx. Secondly, we report the type of IS used in patients with selected microvascular or non-thrombotic aPL manifestations. Methods An online database is used to collect clinical data. The inclusion criteria are positive aPL based on the laboratory section of the APS Classification Criteria, tested at least twice within one year prior to enrollment. Patients are followed every 12 ± 3 months. For this descriptive retrospective and prospective analysis, we included aPL-positive patients without other SAIDx and excluded those with new SAIDx classification during follow-up. For each patient, we retrieved clinical data at baseline and follow-up including selected aPL manifestations (diffuse alveolar hemorrhage [DAH], antiphospholipid-nephropathy [aPL-N], livedoid vasculopathy [LV]-related skin ulcers, TP, autoimmune hemolytic anemia [AIHA], cardiac valve disease [VD]), and IS medications. Results Of 899 patients enrolled, 537 were included in this analysis (mean age 45 ± 13 years, female 377 [70%], APS Classification in 438 [82%], and at least one selected microvascular or non-thrombotic aPL manifestation in 141 (26%)). Of 537 patients, 76 (14%) were reported to use IS (ever), and 41/76 (54%) received IS primarily for selected aPL manifestation. In six of 8 (75%) DAH patients, 6/19 (32%) aPL-N, 4/28 (14%) LV, 25/88 (28%) TP, 6/11 (55%) AIHA, and 1/43 (2%) VD, the IS (excluding corticosteroids/hydroxychloroquine) indication was specific for selected aPL manifestation. Conclusion In our international cohort, 14% of aPL-positive patients without other SAIDx were reported to receive IS; the indication was at least one of the selected microvascular and/or non-thrombotic aPL-related manifestations in half. Thrombocytopenia was the most frequent among those selected aPL-related manifestations; however, approximately one-third received IS specifically for that indication. Diffuse alveolar hemorrhage was frequently treated with IS followed by AIHA and aPL-N. Systematic controlled studies are urgently needed to better define the role of IS in APS.

Introduction Serological markers such as anti-double stranded (ds)DNA antibodies and complement fractions C3/C4, are integral components of disease activity assessment in patients with systemic lupus erythematosus (SLE). However, it remains uncertain whether treatment should aim at restoration of serological abnormalities. Objectives To analyze and critically appraise the literature on the prognostic impact of active lupus serology despite clinical disease quiescence. Methods A systematic literature review was performed in PubMed and EMBASE using the PICOT(S) (population, index, comparator, outcome(s), timing, setting) system to identify studies evaluating the association of serum anti-dsDNA, C3 and C4 levels assessed at the time of clinical remission or during the disease course, against the risk for impending flares and organ damage. Risk of bias was determined by the Quality in Prognosis Studies and ROB2 tools for observational and randomized controlled studies, respectively. Results Fifty-three studies were eligible, the majority having moderate (70.6%) or high (11.8%) risk of bias and not adequately controlling for possible confounders. C3 hypocomplementemia during stable/inactive disease was associated with increased risk (2.0 to 3.8-fold) for subsequent flare in three out of seven relevant studies. Three out of four studies reported a significant effect of C4 hypocomplementemia on flare risk, including one study in lupus nephritis (likelihood ratio-positive 12.0). An increased incidence of flares (2.0 to 2.8-fold) was reported in 11 out of 16 studies assessing the prognostic effect of high anti-dsDNA, and similarly, the majority of studies yielded significant relationships with renal flares. Six studies examined the effect of combined (rather than individual) serological activity, confirming the increased risk (2.0 to 2.7-fold) for relapses. No consistent association was found with organ damage. Conclusion Notwithstanding the heterogeneity and risk of bias, existing evidence indicates a modest association between abnormal serology and risk for flare in patients with stable/inactive SLE. These findings provide limited support for inclusion of serology in the treat-to-target approach but rationalize to further investigate their prognostic implications especially in lupus nephritis.

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.

During the last decades, there has been an increased interest in the discovery and validation of biomarkers that reliably reflect specific aspects of lupus. Although many biomarkers have been developed, few of them have been validated and used in clinical practice, but with unsatisfactory performances. Thus, there is still a need to rigorously validate many of these novel promising biomarkers in large-scale longitudinal studies and also identify better biomarkers not only for lupus diagnosis but also for monitoring and predicting upcoming flares and response to treatment. Besides serological biomarkers, urinary and cerebrospinal fluid biomarkers have emerged for assessing both renal and central nervous system involvement in systemic lupus erythematosus, respectively. Also, novel omics techniques help us to understand the molecular basis of the disease and also allow the identification of novel biomarkers which may be potentially useful for guiding new therapeutic targets.

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 LupusQoL Results: 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.

Objective The aim of this study was to determine whether remission and low disease activity state protect systemic lupus erythematosus patients from being hospitalized. Materials and methods Patients from the Almenara Lupus Cohort were included. Visits were performed every 6 months. Variables were measured at each visit. Hospitalizations were evaluated in the interval between two visits. Remission was defined as: a SLEDAI-2 K of 0, prednisone ≤5 mg/day and immunosuppressants on maintenance dose; low disease activity state as: a SLEDAI-2 K of ≤4, prednisone ≤7.5 mg/day and immunosuppressants on maintenance dose. Univariable and multivariable interval-censored survival regression models were used. In multivariable analysis, possible confounders were gender, age at diagnosis, socioeconomic status, educational level, disease duration, antimalarial use, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SDI) and Charlson comorbidity index. Confounders were determined in the same visit as disease activity state. Results Of the 308 patients, 92.5% of them ( n = 285) were women, had a mean age at diagnosis of 34.8 (13.4) years and a disease duration of 7.7 (6.5) years. At baseline the mean SDI was 1.13 (1.34). A total of 163 of the patients were hospitalized. In the multivariable analysis remission (hazard ratio 0.445 (0.274–0.725), P = 0.001) and low disease activity state (relative risk 0.504 (0.336–0.757), P = 0.001) at baseline were found to decrease the risk of hospitalization in systemic lupus erythematosus patients. A total of 158 hospitalizations presented a discernible cause. Disease activity was the most common cause of hospitalization, with 84 admissions (53.16%), the majority, 38, was due to active kidney disease (45.23%). Conclusion Remission and low disease activity state decreased the risk of hospitalizations in these systemic lupus erythematosus patients. Disease activity, particularly renal, was the most frequent cause of hospitalization.

Aim The aim of this study was to identify factors predictive of serious infections over time in patients with systemic lupus erythematosus (SLE). Methods A multi-ethnic, multi-national Latin American SLE cohort was studied. Serious infection was defined as one that required hospitalization, occurred during a hospitalization or led to death. Potential predictors included were sociodemographic factors, clinical manifestations (per organ involved, lymphopenia and leukopenia, independently) and previous infections at baseline. Disease activity (SLEDAI), damage (SLICC/ACR Damage Index), non-serious infections, glucocorticoids, antimalarials (users and non-users), and immunosuppressive drugs use; the last six variables were examined as time-dependent covariates. Cox regression models were used to evaluate the predictors of serious infections using a backward elimination procedure. Univariable and multivariable analyses were performed. Results Of the 1243 patients included, 1116 (89.8%) were female. The median (interquartile range) age at diagnosis and follow-up time were 27 (20–37) years and 47.8 (17.9–68.6) months, respectively. The incidence rate of serious infections was 3.8 cases per 100 person-years. Antimalarial use (hazard ratio: 0.69; 95% confidence interval (CI): 0.48–0.99; p = 0.0440) was protective, while doses of prednisone >15 and ≤60 mg/day (hazard ratio: 4.18; 95 %CI: 1.69–10.31; p = 0.0019) and >60 mg/day (hazard ratio: 4.71; 95% CI: 1.35–16.49; p = 0.0153), use of methylprednisolone pulses (hazard ratio: 1.53; 95% CI: 1.10–2.13; p = 0.0124), increase in disease activity (hazard ratio: 1.03; 95% CI: 1.01–1.04; p = 0.0016) and damage accrual (hazard ratio: 1.22; 95% CI: 1.11–1.34; p Conclusions Over time, prednisone doses higher than 15 mg/day, use of methylprednisolone pulses, increase in disease activity and damage accrual were predictive of infections, whereas antimalarial use was protective against them in SLE patients.

Background/Objective Information available on the internet about Systemic Lupus Erythematosus (SLE) can influence the doctor-patient relationship. Therefore, the aim of this study was to identify the terms used for SLE on the internet. Methods We analyzed the data downloaded from Google Trends, considering the term “Lupus” in a six-year web-based research. The frequency of the terms for each Pan-American country was obtained automatically from Google Trends, which reports relative search volumes or RSV (on a scale from 0 to 100) across regions. Results We obtained a total of 67 registered terms in 18 countries. The terms were distributed into five categories. The categories with interest in all countries were “definition” and “symptoms”. Conclusions Google Trends allows us to find useful information about SLE on the internet; once the accuracy of this information is validated, it can be used by patients, health institutions, rheumatologists and other health professionals.

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.

In 1950, Hench, Kendall and Reichstein were awarded with the Nobel Prize in Physiology and Medicine for the isolation and first therapeutic use of glucocorticoids. Since then, they have become one of the main agents in the treatment of systemic lupus erythematosus (SLE). The use of high-dose oral glucocorticoids (usually 1 mg/kg/day of prednisone equivalent) have become the rule for treating moderate to severe lupus activity. In addition, tapering schemes have not been well defined, all this leading to prolonged exposures to potentially damaging amounts of glucocorticoids. Several studies have shown that glucocorticoids are a major cause of toxicity in SLE in a dose-dependent manner, with prolonged doses greater than 7.5 mg/day being associated with damage accrual. Thus, there is an urgent need for different therapeutic schedules that can achieve a rapid and durable control of lupus activity while reducing the many unwanted effects of glucocorticoids. Recent data show that pulses of methyl-prednisolone are an effective first-line therapy to treat lupus flares (not only severe ones) without major short or long-term toxicity and allowing a reduction in oral prednisone doses. Universal use of hydroxychloroquine – always recommended, infrequently accomplished – and early therapy with immunosuppressive drugs also help control SLE and reduce prednisone load. Results from observational studies confirm the more rapid achievement of remission and the reduction of long-term damage using these combination schedules with reduced prednisone doses. Seventy years after their first therapeutic use, we are learning to use glucocorticoids in a more efficient and safe manner.

Objective In this paper, we aim to define factors associated with health-related quality of life (HRQoL) in Mestizo patients with systemic lupus erythematosus (SLE). Methods We evaluated patients with SLE from Peru's two largest hospitals between October 2012 and July 2015 to ascertain HRQoL. Using a standard protocol, we incorporated demographic characteristics, clinical manifestations and treatment in our analysis. HRQoL was measured with the LupusQoL, disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and damage was appraised with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) damage index (SDI). The associations between the LupusQoL and these variables were examined using linear regression models. Model selection was based on backward elimination. Results A total of 277 patients fit the inclusion criterion. Of these, 254 (91.7%) were female, the median (interquartile range, IQR) age at diagnosis was 41.5 (33.8-51.8) years, disease duration was 6.5 (2.7-11.3) years. The HRQoL domains most affected were the following: burden to others, fatigue, and intimate relationships. Through multivariate analysis, we determined that older age at diagnosis, higher disease activity, damage, and immunosuppressive drug use were negatively associated with HRQoL. Further, we found that higher socioeconomic status, disease duration, and antimalarial use were positively associated with HRQoL. Conclusion Age at diagnosis, disease activity, damage, and use of immunosuppressive drugs were negatively associated with HRQoL; high socioeconomic status, disease duration, and use of antimalarials were positively associated with HRQoL.

Objective The objective of this report is to analyse retinal changes over a five-year period, assessed by spectral domain-optical coherence tomography (SD-OCT), in patients from the Lupus-Cruces cohort treated with hydroxychloroquine (HCQ). Methods SD-OCT screening was performed annually between 2012 and 2017. Average macular thickness (AMT), ganglion cell layer thickness (GCLT) and qualitative data of retinal pigment epithelium (RPE) and external retina (ExtR) were collected prospectively. We compared data from 2012 (first) and 2017 (second) SD-OCT. Results We studied 110 patients and 195 eyes. No cases of HCQ toxicity were detected. At the time of the second SD-OCT, 99% patients had taken a daily dose of HCQ ≤5 mg/kg/day. The median time on HCQ was 133 months. The mean AMT and GCLT were significantly lower in both eyes at the second SD-OCT; however, all the differences were clinically insignificant at less than 1%. Qualitative analysis of RPE and ExtR showed no significant changes. Similar results were found among patients with risk factors for retinopathy. The comparison of patients with and without risk factors showed no differences. Conclusions This study shows clinically irrelevant retinal changes in an SLE cohort on HCQ treatment over a five-year follow-up. Our findings support the safety of long-term HCQ at doses ≤5 mg/kg/day.

Objective The objective of this study was to determine the association of disease expression patterns with demographic and clinical characteristics in SLE. Methods Patients from a multi-ethnic SLE cohort were included. Disease expression patterns were defined as acute SLE and insidious SLE; this group was divided into those who accrued three ACR criteria and then accrued the fourth (insidious pattern A) and those who have one or two and then accrued four criteria (insidious pattern B). Disease activity was ascertained with the SLAM-R and disease damage with SLICC/ACR damage index. Variables were compared using analysis of variance for numeric variables and χ2 for categorical variables. Multivariable analyses adjusting for possible confounders were performed. Results Six hundred and forty patients were included; the most frequent pattern was the insidious pattern B, with 415 (64.8%) patients, followed by the acute SLE group with 115 (18.0%) and the insidious pattern A with 110 (17.2%) patients. Patients from the insidious pattern A were older at diagnosis (pattern A: 39.8 vs pattern B: 36.7 vs acute: 32.4 years; p Conclusions More insidious onset is associated with older age, Caucasian ethnicity, higher level of education, and lower disease activity than those with acute onset. However, after multivariable analyses, disease activity was not associated with any disease expression pattern.

Aim The aim of this study was to evaluate the clinical response to combined therapy with hydroxychloroquine and mepacrine in patients with systemic lupus erythematosus and refractory joint and/or skin disease. Methods Mepacrine was added to 46 systemic lupus erythematosus patients unresponsive to treatment with the following drug combinations: hydroxychloroquine + prednisone + immunosuppressive drugs ( n = 24), hydroxychloroquine + prednisone ( n = 16), hydroxychloroquine + prednisone + retinoids ( n = 2), hydroxychloroquine alone ( n = 1), hydroxychloroquine + one immunosuppressive drug ( n = 1), hydroxychloroquine + prednisone + one immunosuppressive drug + belimumab ( n = 1) or hydroxychloroquine + prednisone + belimumab ( n = 1). The outcome variable was the clinical response, either complete or partial, based on clinical judgement. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score were additionally used. Results A total of 91% patients showed complete/partial response, with similar rates among those with joint or skin disease. In patients with cutaneous activity, a statistically significant decrease in the CLASI was seen. There also was a statistically significant decrease in the SLEDAI. The mean daily dose of prednisone decreased from 5.8 to 3.4 mg/d ( p = 0.001). Prednisone could be discontinued in 20% of patients. No serious adverse events were seen. Smoking was the only predictor of complete response. Conclusion In the setting of refractory skin and/or joint disease, the addition of mepacrine to previous therapy including hydroxychloroquine was safe and effective in reducing disease activity and decreasing prednisone doses. The fact that smokers responded better opens the door to further studying the combination of mepacrine–hydroxychloroquine as a first-line therapy in such patients.

Objective To determine whether circulating CD4+CD28null and extra-thymic CD4+CD8+ double positive (DP) T cells are independently associated with damage accrual in systemic lupus erythematosus (SLE) patients. Methods This cross-sectional study was conducted between September 2013 and April 2014 in consecutive SLE patients from our Rheumatology Department. CD4+CD28null and CD4+CD8+ DP T-cell frequencies were analyzed by flow-cytometry. The association of damage (SLICC/ACR Damage Index, SDI) and CD4+CD28null and CD4+CD8+ DP T cells was examined by univariable and multivariable Poisson regression models, adjusting for possible confounders. All analyses were performed using SPSS 21.0. Results Patients’ ( n = 133) mean (SD) age at diagnosis was 35.5 (16.8) years, 124 (93.2%) were female; all were mestizo (mixed Caucasian and Amerindian ancestry). Disease duration was 7.4 (6.8) years. The SLE Disease Activity Index was 5.5 (4.2), and the SDI 0.9 (1.2). The percentages of CD4+CD28null and CD4+CD8+ DP T cells were 17.1 (14.4) and 0.4 (1.4), respectively. The percentage of CD4+CD28null and CD4+CD8+ DP T cells were positively associated with a higher SDI in both univariable (rate ratio (RR) 1.02, 95% confidence interval (CI): 1.01–1.03 and 1.17, 95% CI: 1.07–1.27, respectively; p Conclusions In SLE patients, CD4+CD28null and CD4+CD8+ DP T cells are independently associated with disease damage. Longitudinal studies are warranted to determine the predictive value of these associations.

Objective: to determine whether prolactin levels are independently associated with disease damage in systemic lupus erythematosus (SLE) patients. Methods: these cross-sectional analyses were conducted in SLE patient members of the Almenara Lupus Cohort who were seen between January 2012 and June 2013. Disease damage was ascertained with the System Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index (SDI). Prolactin was measured in ng/ml. The association between prolactin levels and the SDI (total and its domains) was evaluated using Spearman's correlation. Subsequently, adjusted Poisson regression models were performed to evaluate these associations. Results: 160 patients were included. 147 (91.9%) were female; their median age at diagnosis was 33.4 (interquartile range (IQR): 26.0–44.3) years; their disease duration was 5.5 (IQR: 2.6–9.7) years. The median prolactin value was 16.8 (IQR: 11.8–24.5) ng/ml. After adjusting for confounders in the Poisson regression model the estimated rate ratios (RR) and 95% confidence interval (CI) for each 10 ng/ml increment of prolactin were 1.13 (95% CI 1.60–1.20, p

Both acute pancreatitis and diffuse alveolar haemorrhage are rare conditions associated with systemic lupus erythematosus (SLE). In this case report, a 23-year-old female with SLE was diagnosed with lupus-associated pancreatitis and, within a few days and despite initial therapy with pulse methyl-prednisolone, subsequently suffered an acute respiratory failure due to a diffuse alveolar haemorrhage. The patient was admitted to the intensive care unit and treatment was intensified with cyclophosphamide and rituximab, which shortly induced the complete remission of SLE with resolution of both clinical conditions. She completed treatment with six pulses of cyclophosphamide followed by azathioprine, hydroxychloroquine and prednisone at initial doses of 20 mg/d with rapid tapering to 5 mg/d, without relapse of the disease during the following year. This case can illustrate that, even in severe, life-threatening SLE flares, it is possible to avoid high-dose prednisone, which has been associated with severe side effects, including infections. Acute pancreatitis and diffuse alveolar haemorrhage are rare conditions caused by SLE. DAH can be a life-threatening complication, with an early mortality of at least 50%. When facing such severe SLE activity, there is a general tendency to use high doses of prednisone as the initial therapy, maintaining such high doses for long periods of time, even after the clinical situation has subsided. We report a case of a young woman with SLE, suffering from acute pancreatitis and diffuse alveolar haemorrhage, who was successfully treated with pulse methyl-prednisolone, hydroxychloroquine, cyclophosphamide and rituximab, combined with medium doses of prednisone.

Objectives The objective of this study was to determine whether prolactin levels are associated with a pro-inflammatory body mass distribution in women with systemic lupus erythematosus (SLE). Methods This cross-sectional study was conducted in consecutive female SLE patients seen in our rheumatology department from January 2012 to July 2015. Prolactin was measured in ng/ml. Body mass distribution was measured by dual energy x-ray absorptiometry and it was divided into subtotal (whole body excluding the head), subtotal bone mineral content, lean mass index (appendicular lean mass/height2), subtotal trunk and leg fat percentages and trunk-to-leg fat ratio. The association between prolactin levels and body mass distribution components was evaluated by univariable and multivariable linear regression models adjusting for possible confounders. Results One hundred and eighty-five patients were evaluated; their mean (SD) age at diagnosis was 34.8 (13.8) years; nearly all patients were Mestizo. Patients included in this study were comparable to the rest of the cohort in terms of age, disease duration, SLEDAI, SDI and body mass index. Disease duration was 7.3 (6.6) years. The SLEDAI was 5.2 (4.3) and the SDI 0.9 (1.3). Prolactin levels were 18.9 (16.7) ng/ml. In univariable analyses, prolactin was negatively associated with bone mineral density, bone mineral content, leg fat percentage and lean mass index, and positively associated with trunk-to-leg fat ratio. In the multivariable analyses, prolactin was negatively associated with bone mineral content and positively associated with trunk-to-leg fat ratio. Conclusions Higher prolactin levels are associated with a pro-inflammatory body mass distribution in SLE patients.